Skip to main content
Erschienen in: Lung 5/2020

11.08.2020 | STATE OF THE ART REVIEW

Connective Tissue Disease-Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment

verfasst von: Niranjan Jeganathan, Matheni Sathananthan

Erschienen in: Lung | Ausgabe 5/2020

Einloggen, um Zugang zu erhalten

Abstract

Interstitial lung disease is a common pulmonary manifestation of connective tissue diseases and results in significant morbidity and mortality. Although all connective tissue diseases are linked by underlying autoimmunity, there is significant variability in the presentation and clinical course of interstitial lung disease associated with the different types of connective tissue diseases. In this paper, we review the prevalence, patterns, predictors, and prognosis of interstitial lung disease secondary to the most common forms of connective tissue diseases. We have also highlighted the available evidence regarding treatment options for interstitial lung disease due to different connective tissue diseases.
Literatur
1.
Zurück zum Zitat Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, Lee JS, Leslie KO, Lynch DA, Matteson EL, Mosca M, Noth I, Richeldi L, Strek ME, Swigris JJ, Wells AU, West SG, Collard HR, Cottin V, CTD-ILD EATFoUFo (2015) An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J 46(4):976–987. https://doi.org/10.1183/13993003.00150-2015CrossRefPubMed Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, Lee JS, Leslie KO, Lynch DA, Matteson EL, Mosca M, Noth I, Richeldi L, Strek ME, Swigris JJ, Wells AU, West SG, Collard HR, Cottin V, CTD-ILD EATFoUFo (2015) An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J 46(4):976–987. https://​doi.​org/​10.​1183/​13993003.​00150-2015CrossRefPubMed
6.
Zurück zum Zitat McNearney TA, Reveille JD, Fischbach M, Friedman AW, Lisse JR, Goel N, Tan FK, Zhou X, Ahn C, Feghali-Bostwick CA, Fritzler M, Arnett FC, Mayes MD (2007) Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum 57(2):318–326. https://doi.org/10.1002/art.22532CrossRefPubMed McNearney TA, Reveille JD, Fischbach M, Friedman AW, Lisse JR, Goel N, Tan FK, Zhou X, Ahn C, Feghali-Bostwick CA, Fritzler M, Arnett FC, Mayes MD (2007) Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum 57(2):318–326. https://​doi.​org/​10.​1002/​art.​22532CrossRefPubMed
7.
Zurück zum Zitat Steen VD, Conte C, Owens GR, Medsger TA Jr (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37(9):1283–1289CrossRef Steen VD, Conte C, Owens GR, Medsger TA Jr (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37(9):1283–1289CrossRef
8.
Zurück zum Zitat Steen VD, Owens GR, Fino GJ, Rodnan GP, Medsger TA Jr (1985) Pulmonary involvement in systemic sclerosis (scleroderma). Arthritis Rheum 28(7):759–767CrossRef Steen VD, Owens GR, Fino GJ, Rodnan GP, Medsger TA Jr (1985) Pulmonary involvement in systemic sclerosis (scleroderma). Arthritis Rheum 28(7):759–767CrossRef
11.
Zurück zum Zitat Suliman YA, Dobrota R, Huscher D, Nguyen-Kim TD, Maurer B, Jordan S, Speich R, Frauenfelder T, Distler O (2015) Brief report: pulmonary function tests: high rate of false-negative results in the early detection and screening of scleroderma-related interstitial lung disease. Arthritis Rheumatol 67(12):3256–3261. https://doi.org/10.1002/art.39405CrossRefPubMed Suliman YA, Dobrota R, Huscher D, Nguyen-Kim TD, Maurer B, Jordan S, Speich R, Frauenfelder T, Distler O (2015) Brief report: pulmonary function tests: high rate of false-negative results in the early detection and screening of scleroderma-related interstitial lung disease. Arthritis Rheumatol 67(12):3256–3261. https://​doi.​org/​10.​1002/​art.​39405CrossRefPubMed
12.
Zurück zum Zitat Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P, Haslam PL, Vassilakis DA, Black CM, du Bois RM (2002) Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 165(12):1581–1586. https://doi.org/10.1164/rccm.2106012CrossRefPubMed Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P, Haslam PL, Vassilakis DA, Black CM, du Bois RM (2002) Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 165(12):1581–1586. https://​doi.​org/​10.​1164/​rccm.​2106012CrossRefPubMed
13.
Zurück zum Zitat Kim DS, Yoo B, Lee JS, Kim EK, Lim CM, Lee SD, Koh Y, Kim WS, Kim WD, Colby TV, Kitiaichi M (2002) The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 19(2):121–127PubMed Kim DS, Yoo B, Lee JS, Kim EK, Lim CM, Lee SD, Koh Y, Kim WS, Kim WD, Colby TV, Kitiaichi M (2002) The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 19(2):121–127PubMed
14.
Zurück zum Zitat Goldin JG, Lynch DA, Strollo DC, Suh RD, Schraufnagel DE, Clements PJ, Elashoff RM, Furst DE, Vasunilashorn S, McNitt-Gray MF, Brown MS, Roth MD, Tashkin DP, Scleroderma Lung Study Research G (2008) High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest 134(2):358–367. https://doi.org/10.1378/chest.07-2444CrossRefPubMed Goldin JG, Lynch DA, Strollo DC, Suh RD, Schraufnagel DE, Clements PJ, Elashoff RM, Furst DE, Vasunilashorn S, McNitt-Gray MF, Brown MS, Roth MD, Tashkin DP, Scleroderma Lung Study Research G (2008) High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest 134(2):358–367. https://​doi.​org/​10.​1378/​chest.​07-2444CrossRefPubMed
17.
Zurück zum Zitat Okano Y, Steen VD, Medsger TA Jr (1993) Autoantibody reactive with RNA polymerase III in systemic sclerosis. Ann Intern Med 119(10):1005–1013CrossRef Okano Y, Steen VD, Medsger TA Jr (1993) Autoantibody reactive with RNA polymerase III in systemic sclerosis. Ann Intern Med 119(10):1005–1013CrossRef
18.
Zurück zum Zitat Sacks DG, Okano Y, Steen VD, Curtiss E, Shapiro LS, Medsger TA Jr (1996) Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3RNP antibody. J Rheumatol 23(4):639–642PubMed Sacks DG, Okano Y, Steen VD, Curtiss E, Shapiro LS, Medsger TA Jr (1996) Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3RNP antibody. J Rheumatol 23(4):639–642PubMed
21.
Zurück zum Zitat Gladman DD, Kung TN, Siannis F, Pellett F, Farewell VT, Lee P (2005) HLA markers for susceptibility and expression in scleroderma. J Rheumatol 32(8):1481–1487PubMed Gladman DD, Kung TN, Siannis F, Pellett F, Farewell VT, Lee P (2005) HLA markers for susceptibility and expression in scleroderma. J Rheumatol 32(8):1481–1487PubMed
24.
25.
Zurück zum Zitat Goh NS, Hoyles RK, Denton CP, Hansell DM, Renzoni EA, Maher TM, Nicholson AG, Wells AU (2017) Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol 69(8):1670–1678. https://doi.org/10.1002/art.40130CrossRefPubMed Goh NS, Hoyles RK, Denton CP, Hansell DM, Renzoni EA, Maher TM, Nicholson AG, Wells AU (2017) Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol 69(8):1670–1678. https://​doi.​org/​10.​1002/​art.​40130CrossRefPubMed
29.
30.
Zurück zum Zitat Strange C, Bolster MB, Roth MD, Silver RM, Theodore A, Goldin J, Clements P, Chung J, Elashoff RM, Suh R, Smith EA, Furst DE, Tashkin DP, Scleroderma Lung Study Research G (2008) Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med 177(1):91–98. https://doi.org/10.1164/rccm.200705-655OCCrossRefPubMed Strange C, Bolster MB, Roth MD, Silver RM, Theodore A, Goldin J, Clements P, Chung J, Elashoff RM, Suh R, Smith EA, Furst DE, Tashkin DP, Scleroderma Lung Study Research G (2008) Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med 177(1):91–98. https://​doi.​org/​10.​1164/​rccm.​200705-655OCCrossRefPubMed
31.
Zurück zum Zitat Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Arriola E, Silver R, Strange C, Bolster M, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel DE, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M, Scleroderma Lung Study Research G (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354(25):2655–2666. https://doi.org/10.1056/NEJMoa055120CrossRefPubMed Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Arriola E, Silver R, Strange C, Bolster M, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel DE, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M, Scleroderma Lung Study Research G (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354(25):2655–2666. https://​doi.​org/​10.​1056/​NEJMoa055120CrossRefPubMed
32.
Zurück zum Zitat Goh NS, Veeraraghavan S, Desai SR, Cramer D, Hansell DM, Denton CP, Black CM, du Bois RM, Wells AU (2007) Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum 56(6):2005–2012. https://doi.org/10.1002/art.22696CrossRefPubMed Goh NS, Veeraraghavan S, Desai SR, Cramer D, Hansell DM, Denton CP, Black CM, du Bois RM, Wells AU (2007) Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum 56(6):2005–2012. https://​doi.​org/​10.​1002/​art.​22696CrossRefPubMed
33.
Zurück zum Zitat Launay D, Remy-Jardin M, Michon-Pasturel U, Mastora I, Hachulla E, Lambert M, Delannoy V, Queyrel V, Duhamel A, Matran R, De Groote P, Hatron PY (2006) High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. J Rheumatol 33(9):1789–1801PubMed Launay D, Remy-Jardin M, Michon-Pasturel U, Mastora I, Hachulla E, Lambert M, Delannoy V, Queyrel V, Duhamel A, Matran R, De Groote P, Hatron PY (2006) High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. J Rheumatol 33(9):1789–1801PubMed
39.
Zurück zum Zitat Khanna D, Tseng CH, Farmani N, Steen V, Furst DE, Clements PJ, Roth MD, Goldin J, Elashoff R, Seibold JR, Saggar R, Tashkin DP (2011) Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung Study Placebo Group. Arthritis Rheum 63(10):3078–3085. https://doi.org/10.1002/art.30467CrossRefPubMedPubMedCentral Khanna D, Tseng CH, Farmani N, Steen V, Furst DE, Clements PJ, Roth MD, Goldin J, Elashoff R, Seibold JR, Saggar R, Tashkin DP (2011) Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung Study Placebo Group. Arthritis Rheum 63(10):3078–3085. https://​doi.​org/​10.​1002/​art.​30467CrossRefPubMedPubMedCentral
42.
Zurück zum Zitat Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Muller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumanovics G, Coleiro B, Feierl E, Szucs G, Von Muhlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kotter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69(10):1809–1815. https://doi.org/10.1136/ard.2009.114264CrossRefPubMed Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Muller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumanovics G, Coleiro B, Feierl E, Szucs G, Von Muhlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kotter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69(10):1809–1815. https://​doi.​org/​10.​1136/​ard.​2009.​114264CrossRefPubMed
43.
Zurück zum Zitat Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM, Goldin J, Arriola E, Strange C, Bolster MB, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel D, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M, Khanna D, Li N, Li G, Scleroderma Lung Study Research G (2007) Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med 176(10):1026–1034. https://doi.org/10.1164/rccm.200702-326OCCrossRefPubMedPubMedCentral Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM, Goldin J, Arriola E, Strange C, Bolster MB, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel D, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M, Khanna D, Li N, Li G, Scleroderma Lung Study Research G (2007) Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med 176(10):1026–1034. https://​doi.​org/​10.​1164/​rccm.​200702-326OCCrossRefPubMedPubMedCentral
44.
Zurück zum Zitat Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC, Goldin J, Arriola E, Volkmann ER, Kafaja S, Silver R, Steen V, Strange C, Wise R, Wigley F, Mayes M, Riley DJ, Hussain S, Assassi S, Hsu VM, Patel B, Phillips K, Martinez F, Golden J, Connolly MK, Varga J, Dematte J, Hinchcliff ME, Fischer A, Swigris J, Meehan R, Theodore A, Simms R, Volkov S, Schraufnagel DE, Scholand MB, Frech T, Molitor JA, Highland K, Read CA, Fritzler MJ, Kim GHJ, Tseng CH, Elashoff RM, Sclerodema Lung Study III (2016) Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med 4(9):708–719. https://doi.org/10.1016/S2213-2600(16)30152-7CrossRefPubMedPubMedCentral Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC, Goldin J, Arriola E, Volkmann ER, Kafaja S, Silver R, Steen V, Strange C, Wise R, Wigley F, Mayes M, Riley DJ, Hussain S, Assassi S, Hsu VM, Patel B, Phillips K, Martinez F, Golden J, Connolly MK, Varga J, Dematte J, Hinchcliff ME, Fischer A, Swigris J, Meehan R, Theodore A, Simms R, Volkov S, Schraufnagel DE, Scholand MB, Frech T, Molitor JA, Highland K, Read CA, Fritzler MJ, Kim GHJ, Tseng CH, Elashoff RM, Sclerodema Lung Study III (2016) Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med 4(9):708–719. https://​doi.​org/​10.​1016/​S2213-2600(16)30152-7CrossRefPubMedPubMedCentral
45.
Zurück zum Zitat Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM, Investigators ST (2019) Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 380(26):2518–2528. https://doi.org/10.1056/NEJMoa1903076CrossRefPubMed Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM, Investigators ST (2019) Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 380(26):2518–2528. https://​doi.​org/​10.​1056/​NEJMoa1903076CrossRefPubMed
46.
Zurück zum Zitat Khanna D, Albera C, Fischer A, Khalidi N, Raghu G, Chung L, Chen D, Schiopu E, Tagliaferri M, Seibold JR, Gorina E (2016) An open-label, Phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial. J Rheumatol 43(9):1672–1679. https://doi.org/10.3899/jrheum.151322CrossRefPubMed Khanna D, Albera C, Fischer A, Khalidi N, Raghu G, Chung L, Chen D, Schiopu E, Tagliaferri M, Seibold JR, Gorina E (2016) An open-label, Phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial. J Rheumatol 43(9):1672–1679. https://​doi.​org/​10.​3899/​jrheum.​151322CrossRefPubMed
47.
Zurück zum Zitat Khanna D, Denton CP, Jahreis A, van Laar JM, Frech TM, Anderson ME, Baron M, Chung L, Fierlbeck G, Lakshminarayanan S, Allanore Y, Pope JE, Riemekasten G, Steen V, Muller-Ladner U, Lafyatis R, Stifano G, Spotswood H, Chen-Harris H, Dziadek S, Morimoto A, Sornasse T, Siegel J, Furst DE (2016) Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet 387(10038):2630–2640. https://doi.org/10.1016/S0140-6736(16)00232-4CrossRefPubMed Khanna D, Denton CP, Jahreis A, van Laar JM, Frech TM, Anderson ME, Baron M, Chung L, Fierlbeck G, Lakshminarayanan S, Allanore Y, Pope JE, Riemekasten G, Steen V, Muller-Ladner U, Lafyatis R, Stifano G, Spotswood H, Chen-Harris H, Dziadek S, Morimoto A, Sornasse T, Siegel J, Furst DE (2016) Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet 387(10038):2630–2640. https://​doi.​org/​10.​1016/​S0140-6736(16)00232-4CrossRefPubMed
48.
Zurück zum Zitat Khanna D, Denton CP, Lin CJF, van Laar JM, Frech TM, Anderson ME, Baron M, Chung L, Fierlbeck G, Lakshminarayanan S, Allanore Y, Pope JE, Riemekasten G, Steen V, Muller-Ladner U, Spotswood H, Burke L, Siegel J, Jahreis A, Furst DE (2018) Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). Ann Rheum Dis 77(2):212–220. https://doi.org/10.1136/annrheumdis-2017-211682CrossRefPubMed Khanna D, Denton CP, Lin CJF, van Laar JM, Frech TM, Anderson ME, Baron M, Chung L, Fierlbeck G, Lakshminarayanan S, Allanore Y, Pope JE, Riemekasten G, Steen V, Muller-Ladner U, Spotswood H, Burke L, Siegel J, Jahreis A, Furst DE (2018) Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). Ann Rheum Dis 77(2):212–220. https://​doi.​org/​10.​1136/​annrheumdis-2017-211682CrossRefPubMed
49.
Zurück zum Zitat van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J, Schuerwegh AJ, Marijt EW, Vonk MC, Schattenberg AV, Matucci-Cerinic M, Voskuyl AE, van de Loosdrecht AA, Daikeler T, Kotter I, Schmalzing M, Martin T, Lioure B, Weiner SM, Kreuter A, Deligny C, Durand JM, Emery P, Machold KP, Sarrot-Reynauld F, Warnatz K, Adoue DF, Constans J, Tony HP, Del Papa N, Fassas A, Himsel A, Launay D, Lo Monaco A, Philippe P, Quere I, Rich E, Westhovens R, Griffiths B, Saccardi R, van den Hoogen FH, Fibbe WE, Socie G, Gratwohl A, Tyndall A, Group EESS (2014) Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 311(24):2490–2498. https://doi.org/10.1001/jama.2014.6368CrossRefPubMed van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J, Schuerwegh AJ, Marijt EW, Vonk MC, Schattenberg AV, Matucci-Cerinic M, Voskuyl AE, van de Loosdrecht AA, Daikeler T, Kotter I, Schmalzing M, Martin T, Lioure B, Weiner SM, Kreuter A, Deligny C, Durand JM, Emery P, Machold KP, Sarrot-Reynauld F, Warnatz K, Adoue DF, Constans J, Tony HP, Del Papa N, Fassas A, Himsel A, Launay D, Lo Monaco A, Philippe P, Quere I, Rich E, Westhovens R, Griffiths B, Saccardi R, van den Hoogen FH, Fibbe WE, Socie G, Gratwohl A, Tyndall A, Group EESS (2014) Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 311(24):2490–2498. https://​doi.​org/​10.​1001/​jama.​2014.​6368CrossRefPubMed
50.
Zurück zum Zitat Sullivan KM, Goldmuntz EA, Keyes-Elstein L, McSweeney PA, Pinckney A, Welch B, Mayes MD, Nash RA, Crofford LJ, Eggleston B, Castina S, Griffith LM, Goldstein JS, Wallace D, Craciunescu O, Khanna D, Folz RJ, Goldin J, St Clair EW, Seibold JR, Phillips K, Mineishi S, Simms RW, Ballen K, Wener MH, Georges GE, Heimfeld S, Hosing C, Forman S, Kafaja S, Silver RM, Griffing L, Storek J, LeClercq S, Brasington R, Csuka ME, Bredeson C, Keever-Taylor C, Domsic RT, Kahaleh MB, Medsger T, Furst DE, Investigators SS (2018) Myeloablative autologous stem-cell transplantation for severe scleroderma. N Engl J Med 378(1):35–47CrossRef Sullivan KM, Goldmuntz EA, Keyes-Elstein L, McSweeney PA, Pinckney A, Welch B, Mayes MD, Nash RA, Crofford LJ, Eggleston B, Castina S, Griffith LM, Goldstein JS, Wallace D, Craciunescu O, Khanna D, Folz RJ, Goldin J, St Clair EW, Seibold JR, Phillips K, Mineishi S, Simms RW, Ballen K, Wener MH, Georges GE, Heimfeld S, Hosing C, Forman S, Kafaja S, Silver RM, Griffing L, Storek J, LeClercq S, Brasington R, Csuka ME, Bredeson C, Keever-Taylor C, Domsic RT, Kahaleh MB, Medsger T, Furst DE, Investigators SS (2018) Myeloablative autologous stem-cell transplantation for severe scleroderma. N Engl J Med 378(1):35–47CrossRef
56.
Zurück zum Zitat Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, Roberts C, Desai S, Herrick AL, McHugh NJ, Foley NM, Pearson SB, Emery P, Veale DJ, Denton CP, Wells AU, Black CM, du Bois RM (2006) A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 54(12):3962–3970. https://doi.org/10.1002/art.22204CrossRefPubMed Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, Roberts C, Desai S, Herrick AL, McHugh NJ, Foley NM, Pearson SB, Emery P, Veale DJ, Denton CP, Wells AU, Black CM, du Bois RM (2006) A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 54(12):3962–3970. https://​doi.​org/​10.​1002/​art.​22204CrossRefPubMed
57.
Zurück zum Zitat Miele CH, Schwab K, Saggar R, Duffy E, Elashoff D, Tseng CH, Weigt S, Charan D, Abtin F, Johannes J, Derhovanessian A, Conklin J, Ghassemi K, Khanna D, Siddiqui O, Ardehali A, Hunter C, Kwon M, Biniwale R, Lo M, Volkmann E, Torres Barba D, Belperio JA, Sayah D, Mahrer T, Furst DE, Kafaja S, Clements P, Shino M, Gregson A, Kubak B, Lynch JP, 3rd, Ross D, Saggar R (2016) Lung transplant outcomes in systemic sclerosis with significant esophageal dysfunction. A comprehensive single-center experience. Ann Am Thorac Soc 13(6):793–802. Miele CH, Schwab K, Saggar R, Duffy E, Elashoff D, Tseng CH, Weigt S, Charan D, Abtin F, Johannes J, Derhovanessian A, Conklin J, Ghassemi K, Khanna D, Siddiqui O, Ardehali A, Hunter C, Kwon M, Biniwale R, Lo M, Volkmann E, Torres Barba D, Belperio JA, Sayah D, Mahrer T, Furst DE, Kafaja S, Clements P, Shino M, Gregson A, Kubak B, Lynch JP, 3rd, Ross D, Saggar R (2016) Lung transplant outcomes in systemic sclerosis with significant esophageal dysfunction. A comprehensive single-center experience. Ann Am Thorac Soc 13(6):793–802.
58.
Zurück zum Zitat Pradere P, Tudorache I, Magnusson J, Savale L, Brugiere O, Douvry B, Reynaud-Gaubert M, Claustre J, Borgne AL, Holm AM, Schultz HH, Knoop C, Godinas L, Fisher AJ, Hirschi S, Gottlieb J, Le Pavec J, Working Group on Heart/Lung Transplantation in Systemic S (2018) Lung transplantation for scleroderma lung disease: An international, multicenter, observational cohort study. J Heart Lung Transpl 37(7):903–911. https://doi.org/10.1016/j.healun.2018.03.003CrossRef Pradere P, Tudorache I, Magnusson J, Savale L, Brugiere O, Douvry B, Reynaud-Gaubert M, Claustre J, Borgne AL, Holm AM, Schultz HH, Knoop C, Godinas L, Fisher AJ, Hirschi S, Gottlieb J, Le Pavec J, Working Group on Heart/Lung Transplantation in Systemic S (2018) Lung transplantation for scleroderma lung disease: An international, multicenter, observational cohort study. J Heart Lung Transpl 37(7):903–911. https://​doi.​org/​10.​1016/​j.​healun.​2018.​03.​003CrossRef
60.
Zurück zum Zitat Tanoue LT (1998) Pulmonary manifestations of rheumatoid arthritis. Clin Chest Med 19(4):667–685CrossRef Tanoue LT (1998) Pulmonary manifestations of rheumatoid arthritis. Clin Chest Med 19(4):667–685CrossRef
65.
Zurück zum Zitat Suzuki A, Ohosone Y, Obana M, Mita S, Matsuoka Y, Irimajiri S, Fukuda J (1994) Cause of death in 81 autopsied patients with rheumatoid arthritis. J Rheumatol 21(1):33–36PubMed Suzuki A, Ohosone Y, Obana M, Mita S, Matsuoka Y, Irimajiri S, Fukuda J (1994) Cause of death in 81 autopsied patients with rheumatoid arthritis. J Rheumatol 21(1):33–36PubMed
68.
Zurück zum Zitat Kelly CA, Saravanan V, Nisar M, Arthanari S, Woodhead FA, Price-Forbes AN, Dawson J, Sathi N, Ahmad Y, Koduri G, Young A, British Rheumatoid Interstitial Lung N (2014) Rheumatoid arthritis-related interstitial lung disease: associations, prognostic factors and physiological and radiological characteristics–a large multicentre UK study. Rheumatology (Oxford) 53(9):1676–1682. https://doi.org/10.1093/rheumatology/keu165CrossRef Kelly CA, Saravanan V, Nisar M, Arthanari S, Woodhead FA, Price-Forbes AN, Dawson J, Sathi N, Ahmad Y, Koduri G, Young A, British Rheumatoid Interstitial Lung N (2014) Rheumatoid arthritis-related interstitial lung disease: associations, prognostic factors and physiological and radiological characteristics–a large multicentre UK study. Rheumatology (Oxford) 53(9):1676–1682. https://​doi.​org/​10.​1093/​rheumatology/​keu165CrossRef
72.
73.
Zurück zum Zitat Chen J, Doyle TJ, Liu Y, Aggarwal R, Wang X, Shi Y, Ge SX, Huang H, Lin Q, Liu W, Cai Y, Koontz D, Fuhrman CR, Golzarri MF, Liu Y, Hatabu H, Nishino M, Araki T, Dellaripa PF, Oddis CV, Rosas IO, Ascherman DP (2015) Biomarkers of rheumatoid arthritis-associated interstitial lung disease. Arthritis Rheumatol 67(1):28–38. https://doi.org/10.1002/art.38904CrossRefPubMedPubMedCentral Chen J, Doyle TJ, Liu Y, Aggarwal R, Wang X, Shi Y, Ge SX, Huang H, Lin Q, Liu W, Cai Y, Koontz D, Fuhrman CR, Golzarri MF, Liu Y, Hatabu H, Nishino M, Araki T, Dellaripa PF, Oddis CV, Rosas IO, Ascherman DP (2015) Biomarkers of rheumatoid arthritis-associated interstitial lung disease. Arthritis Rheumatol 67(1):28–38. https://​doi.​org/​10.​1002/​art.​38904CrossRefPubMedPubMedCentral
74.
Zurück zum Zitat Maniwa K, Ogushi F, Tani K, Ohmoto Y, Muraguchi M, Sone S (2000) Increased incidence of autoantibodies to interleukin-1a in rheumatoid arthritis with interstitial lung disease. Respirology 5(4):315–320PubMed Maniwa K, Ogushi F, Tani K, Ohmoto Y, Muraguchi M, Sone S (2000) Increased incidence of autoantibodies to interleukin-1a in rheumatoid arthritis with interstitial lung disease. Respirology 5(4):315–320PubMed
75.
Zurück zum Zitat Nakajima H, Harigai M, Hara M, Hakoda M, Tokuda H, Sakai F, Kamatani N, Kashiwazaki S (2000) KL-6 as a novel serum marker for interstitial pneumonia associated with collagen diseases. J Rheumatol 27(5):1164–1170PubMed Nakajima H, Harigai M, Hara M, Hakoda M, Tokuda H, Sakai F, Kamatani N, Kashiwazaki S (2000) KL-6 as a novel serum marker for interstitial pneumonia associated with collagen diseases. J Rheumatol 27(5):1164–1170PubMed
77.
Zurück zum Zitat Juge PA, Lee JS, Ebstein E, Furukawa H, Dobrinskikh E, Gazal S, Kannengiesser C, Ottaviani S, Oka S, Tohma S, Tsuchiya N, Rojas-Serrano J, Gonzalez-Perez MI, Mejia M, Buendia-Roldan I, Falfan-Valencia R, Ambrocio-Ortiz E, Manali E, Papiris SA, Karageorgas T, Boumpas D, Antoniou K, van Moorsel CHM, van der Vis J, de Man YA, Grutters JC, Wang Y, Borie R, Wemeau-Stervinou L, Wallaert B, Flipo RM, Nunes H, Valeyre D, Saidenberg-Kermanac'h N, Boissier MC, Marchand-Adam S, Frazier A, Richette P, Allanore Y, Sibilia J, Dromer C, Richez C, Schaeverbeke T, Liote H, Thabut G, Nathan N, Amselem S, Soubrier M, Cottin V, Clement A, Deane K, Walts AD, Fingerlin T, Fischer A, Ryu JH, Matteson EL, Niewold TB, Assayag D, Gross A, Wolters P, Schwarz MI, Holers M, Solomon JJ, Doyle T, Rosas IO, Blauwendraat C, Nalls MA, Debray MP, Boileau C, Crestani B, Schwartz DA, Dieude P (2018) MUC5B promoter variant and rheumatoid arthritis with interstitial lung disease. N Engl J Med 379(23):2209–2219. https://doi.org/10.1056/NEJMoa1801562CrossRefPubMedPubMedCentral Juge PA, Lee JS, Ebstein E, Furukawa H, Dobrinskikh E, Gazal S, Kannengiesser C, Ottaviani S, Oka S, Tohma S, Tsuchiya N, Rojas-Serrano J, Gonzalez-Perez MI, Mejia M, Buendia-Roldan I, Falfan-Valencia R, Ambrocio-Ortiz E, Manali E, Papiris SA, Karageorgas T, Boumpas D, Antoniou K, van Moorsel CHM, van der Vis J, de Man YA, Grutters JC, Wang Y, Borie R, Wemeau-Stervinou L, Wallaert B, Flipo RM, Nunes H, Valeyre D, Saidenberg-Kermanac'h N, Boissier MC, Marchand-Adam S, Frazier A, Richette P, Allanore Y, Sibilia J, Dromer C, Richez C, Schaeverbeke T, Liote H, Thabut G, Nathan N, Amselem S, Soubrier M, Cottin V, Clement A, Deane K, Walts AD, Fingerlin T, Fischer A, Ryu JH, Matteson EL, Niewold TB, Assayag D, Gross A, Wolters P, Schwarz MI, Holers M, Solomon JJ, Doyle T, Rosas IO, Blauwendraat C, Nalls MA, Debray MP, Boileau C, Crestani B, Schwartz DA, Dieude P (2018) MUC5B promoter variant and rheumatoid arthritis with interstitial lung disease. N Engl J Med 379(23):2209–2219. https://​doi.​org/​10.​1056/​NEJMoa1801562CrossRefPubMedPubMedCentral
80.
Zurück zum Zitat Song JW, Lee HK, Lee CK, Chae EJ, Jang SJ, Colby TV, Kim DS (2013) Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 30(2):103–112PubMed Song JW, Lee HK, Lee CK, Chae EJ, Jang SJ, Colby TV, Kim DS (2013) Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 30(2):103–112PubMed
81.
Zurück zum Zitat Dawson JK, Fewins HE, Desmond J, Lynch MP, Graham DR (2001) Fibrosing alveolitis in patients with rheumatoid arthritis as assessed by high resolution computed tomography, chest radiography, and pulmonary function tests. Thorax 56(8):622–627CrossRef Dawson JK, Fewins HE, Desmond J, Lynch MP, Graham DR (2001) Fibrosing alveolitis in patients with rheumatoid arthritis as assessed by high resolution computed tomography, chest radiography, and pulmonary function tests. Thorax 56(8):622–627CrossRef
82.
Zurück zum Zitat Solomon JJ, Chung JH, Cosgrove GP, Demoruelle MK, Fernandez-Perez ER, Fischer A, Frankel SK, Hobbs SB, Huie TJ, Ketzer J, Mannina A, Olson AL, Russell G, Tsuchiya Y, Yunt ZX, Zelarney PT, Brown KK, Swigris JJ (2016) Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J 47(2):588–596. https://doi.org/10.1183/13993003.00357-2015CrossRefPubMed Solomon JJ, Chung JH, Cosgrove GP, Demoruelle MK, Fernandez-Perez ER, Fischer A, Frankel SK, Hobbs SB, Huie TJ, Ketzer J, Mannina A, Olson AL, Russell G, Tsuchiya Y, Yunt ZX, Zelarney PT, Brown KK, Swigris JJ (2016) Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J 47(2):588–596. https://​doi.​org/​10.​1183/​13993003.​00357-2015CrossRefPubMed
88.
90.
Zurück zum Zitat Fernandez-Diaz C, Loricera J, Castaneda S, Lopez-Mejias R, Ojeda-Garcia C, Olive A, Rodriguez-Muguruza S, Carreira PE, Perez-Sandoval T, Retuerto M, Cervantes-Perez EC, Flores-Robles BJ, Hernandez-Cruz B, Urruticoechea A, Maiz-Alonso O, Arboleya L, Bonilla G, Hernandez-Rodriguez I, Palma D, Delgado C, Exposito-Molinero R, Ruibal-Escribano A, Alvarez-Rodriguez B, Blanco-Madrigal J, Bernal JA, Vela-Casasempere P, Rodriguez-Gomez M, Fito C, Ortiz-Sanjuan F, Narvaez J, Moreno M, Lopez-Corbeto M, Mena-Vazquez N, Aguilera-Cros C, Romero-Yuste S, Ordonez S, Villa-Blanco I, Gonzelez-Vela MC, Mora-Cuesta V, Palmou-Fontana N, Hernandez JL, Gonzalez-Gay MA, Blanco R (2018) Abatacept in patients with rheumatoid arthritis and interstitial lung disease: a national multicenter study of 63 patients. Semin Arthritis Rheum 48(1):22–27. https://doi.org/10.1016/j.semarthrit.2017.12.012CrossRefPubMed Fernandez-Diaz C, Loricera J, Castaneda S, Lopez-Mejias R, Ojeda-Garcia C, Olive A, Rodriguez-Muguruza S, Carreira PE, Perez-Sandoval T, Retuerto M, Cervantes-Perez EC, Flores-Robles BJ, Hernandez-Cruz B, Urruticoechea A, Maiz-Alonso O, Arboleya L, Bonilla G, Hernandez-Rodriguez I, Palma D, Delgado C, Exposito-Molinero R, Ruibal-Escribano A, Alvarez-Rodriguez B, Blanco-Madrigal J, Bernal JA, Vela-Casasempere P, Rodriguez-Gomez M, Fito C, Ortiz-Sanjuan F, Narvaez J, Moreno M, Lopez-Corbeto M, Mena-Vazquez N, Aguilera-Cros C, Romero-Yuste S, Ordonez S, Villa-Blanco I, Gonzelez-Vela MC, Mora-Cuesta V, Palmou-Fontana N, Hernandez JL, Gonzalez-Gay MA, Blanco R (2018) Abatacept in patients with rheumatoid arthritis and interstitial lung disease: a national multicenter study of 63 patients. Semin Arthritis Rheum 48(1):22–27. https://​doi.​org/​10.​1016/​j.​semarthrit.​2017.​12.​012CrossRefPubMed
91.
Zurück zum Zitat Manfredi A, Cassone G, Furini F, Gremese E, Venerito V, Atzeni F, Arrigoni E, Della Casa G, Cerri S, Govoni M, Petricca L, Iannone F, Salvarani C, Sebastiani M (2019) Tocilizumab therapy in rheumatoid arthritis with interstitial lung disease: a multicenter retrospective study. Intern Med J. https://doi.org/10.1111/imj.14670CrossRef Manfredi A, Cassone G, Furini F, Gremese E, Venerito V, Atzeni F, Arrigoni E, Della Casa G, Cerri S, Govoni M, Petricca L, Iannone F, Salvarani C, Sebastiani M (2019) Tocilizumab therapy in rheumatoid arthritis with interstitial lung disease: a multicenter retrospective study. Intern Med J. https://​doi.​org/​10.​1111/​imj.​14670CrossRef
94.
Zurück zum Zitat Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, Richeldi L, Kolb M, Tetzlaff K, Stowasser S, Coeck C, Clerisme-Beaty E, Rosenstock B, Quaresma M, Haeufel T, Goeldner RG, Schlenker-Herceg R, Brown KK, Investigators IT (2019) Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 381(18):1718–1727. https://doi.org/10.1056/NEJMoa1908681CrossRefPubMed Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, Richeldi L, Kolb M, Tetzlaff K, Stowasser S, Coeck C, Clerisme-Beaty E, Rosenstock B, Quaresma M, Haeufel T, Goeldner RG, Schlenker-Herceg R, Brown KK, Investigators IT (2019) Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 381(18):1718–1727. https://​doi.​org/​10.​1056/​NEJMoa1908681CrossRefPubMed
99.
Zurück zum Zitat Ikeda S, Arita M, Misaki K, Mishima S, Takaiwa T, Nishiyama A, Ito A, Furuta K, Yokoyama T, Tokioka F, Noyama M, Yoshioka H, Ishida T (2015) Incidence and impact of interstitial lung disease and malignancy in patients with polymyositis, dermatomyositis, and clinically amyopathic dermatomyositis: a retrospective cohort study. Springerplus 4:240. https://doi.org/10.1186/s40064-015-1013-8CrossRefPubMedPubMedCentral Ikeda S, Arita M, Misaki K, Mishima S, Takaiwa T, Nishiyama A, Ito A, Furuta K, Yokoyama T, Tokioka F, Noyama M, Yoshioka H, Ishida T (2015) Incidence and impact of interstitial lung disease and malignancy in patients with polymyositis, dermatomyositis, and clinically amyopathic dermatomyositis: a retrospective cohort study. Springerplus 4:240. https://​doi.​org/​10.​1186/​s40064-015-1013-8CrossRefPubMedPubMedCentral
105.
Zurück zum Zitat Cavagna L, Trallero-Araguas E, Meloni F, Cavazzana I, Rojas-Serrano J, Feist E, Zanframundo G, Morandi V, Meyer A, Pereira da Silva JA, Matos Costa CJ, Molberg O, Andersson H, Codullo V, Mosca M, Barsotti S, Neri R, Scire C, Govoni M, Furini F, Lopez-Longo FJ, Martinez-Barrio J, Schneider U, Lorenz HM, Doria A, Ghirardello A, Ortego-Centeno N, Confalonieri M, Tomietto P, Pipitone N, Rodriguez Cambron AB, Blazquez Canamero MA, Voll RE, Wendel S, Scarpato S, Maurier F, Limonta M, Colombelli P, Giannini M, Geny B, Arrigoni E, Bravi E, Migliorini P, Mathieu A, Piga M, Drott U, Delbrueck C, Bauhammer J, Cagnotto G, Vancheri C, Sambataro G, De Langhe E, Sainaghi PP, Monti C, Gigli Berzolari F, Romano M, Bonella F, Specker C, Schwarting A, Villa Blanco I, Selmi C, Ceribelli A, Nuno L, Mera-Varela A, Perez Gomez N, Fusaro E, Parisi S, Sinigaglia L, Del Papa N, Benucci M, Cimmino MA, Riccieri V, Conti F, Sebastiani GD, Iuliano A, Emmi G, Cammelli D, Sebastiani M, Manfredi A, Bachiller-Corral J, Sifuentes Giraldo WA, Paolazzi G, Saketkoo LA, Giorgi R, Salaffi F, Cifrian J, Caporali R, Locatelli F, Marchioni E, Pesci A, Dei G, Pozzi MR, Claudia L, Distler J, Knitza J, Schett G, Iannone F, Fornaro M, Franceschini F, Quartuccio L, Gerli R, Bartoloni E, Bellando Randone S, Zampogna G, Gonzalez Perez MI, Mejia M, Vicente E, Triantafyllias K, Lopez-Mejias R, Matucci-Cerinic M, Selva-O'Callaghan A, Castaneda S, Montecucco C, Gonzalez-Gay MA (2019) Influence of antisynthetase antibodies specificities on antisynthetase syndrome clinical spectrum time course. J Clin Med. https://doi.org/10.3390/jcm8112013CrossRefPubMedPubMedCentral Cavagna L, Trallero-Araguas E, Meloni F, Cavazzana I, Rojas-Serrano J, Feist E, Zanframundo G, Morandi V, Meyer A, Pereira da Silva JA, Matos Costa CJ, Molberg O, Andersson H, Codullo V, Mosca M, Barsotti S, Neri R, Scire C, Govoni M, Furini F, Lopez-Longo FJ, Martinez-Barrio J, Schneider U, Lorenz HM, Doria A, Ghirardello A, Ortego-Centeno N, Confalonieri M, Tomietto P, Pipitone N, Rodriguez Cambron AB, Blazquez Canamero MA, Voll RE, Wendel S, Scarpato S, Maurier F, Limonta M, Colombelli P, Giannini M, Geny B, Arrigoni E, Bravi E, Migliorini P, Mathieu A, Piga M, Drott U, Delbrueck C, Bauhammer J, Cagnotto G, Vancheri C, Sambataro G, De Langhe E, Sainaghi PP, Monti C, Gigli Berzolari F, Romano M, Bonella F, Specker C, Schwarting A, Villa Blanco I, Selmi C, Ceribelli A, Nuno L, Mera-Varela A, Perez Gomez N, Fusaro E, Parisi S, Sinigaglia L, Del Papa N, Benucci M, Cimmino MA, Riccieri V, Conti F, Sebastiani GD, Iuliano A, Emmi G, Cammelli D, Sebastiani M, Manfredi A, Bachiller-Corral J, Sifuentes Giraldo WA, Paolazzi G, Saketkoo LA, Giorgi R, Salaffi F, Cifrian J, Caporali R, Locatelli F, Marchioni E, Pesci A, Dei G, Pozzi MR, Claudia L, Distler J, Knitza J, Schett G, Iannone F, Fornaro M, Franceschini F, Quartuccio L, Gerli R, Bartoloni E, Bellando Randone S, Zampogna G, Gonzalez Perez MI, Mejia M, Vicente E, Triantafyllias K, Lopez-Mejias R, Matucci-Cerinic M, Selva-O'Callaghan A, Castaneda S, Montecucco C, Gonzalez-Gay MA (2019) Influence of antisynthetase antibodies specificities on antisynthetase syndrome clinical spectrum time course. J Clin Med. https://​doi.​org/​10.​3390/​jcm8112013CrossRefPubMedPubMedCentral
106.
Zurück zum Zitat Hall JC, Casciola-Rosen L, Samedy LA, Werner J, Owoyemi K, Danoff SK, Christopher-Stine L (2013) Anti-melanoma differentiation-associated protein 5-associated dermatomyositis: expanding the clinical spectrum. Arthritis Care Res (Hoboken) 65(8):1307–1315. https://doi.org/10.1002/acr.21992CrossRef Hall JC, Casciola-Rosen L, Samedy LA, Werner J, Owoyemi K, Danoff SK, Christopher-Stine L (2013) Anti-melanoma differentiation-associated protein 5-associated dermatomyositis: expanding the clinical spectrum. Arthritis Care Res (Hoboken) 65(8):1307–1315. https://​doi.​org/​10.​1002/​acr.​21992CrossRef
112.
Zurück zum Zitat Lopez-Mejias R, Remuzgo-Martinez S, Genre F, Pulito-Cueto V, Rozas SMF, Llorca J, Fernandez DI, Cuesta VMM, Ortego-Centeno N, Gomez NP, Mera-Varela A, Martinez-Barrio J, Lopez-Longo FJ, Mijares V, Lera-Gomez L, Usetti MP, Laporta R, Perez V, Gafas AP, Gonzalez MAA, Calvo-Alen J, Romero-Bueno F, Sanchez-Pernaute O, Nuno L, Bonilla G, Balsa A, Hernandez-Gonzalez F, Grafia I, Prieto-Gonzalez S, Narvaez J, Trallero-Araguas E, Selva-O'Callaghan A, Gualillo O, Castaneda S, Cavagna L, Cifrian JM, Gonzalez-Gay MA (2020) Influence of MUC5B gene on antisynthetase syndrome. Sci Rep 10(1):1415. https://doi.org/10.1038/s41598-020-58400-0CrossRefPubMedPubMedCentral Lopez-Mejias R, Remuzgo-Martinez S, Genre F, Pulito-Cueto V, Rozas SMF, Llorca J, Fernandez DI, Cuesta VMM, Ortego-Centeno N, Gomez NP, Mera-Varela A, Martinez-Barrio J, Lopez-Longo FJ, Mijares V, Lera-Gomez L, Usetti MP, Laporta R, Perez V, Gafas AP, Gonzalez MAA, Calvo-Alen J, Romero-Bueno F, Sanchez-Pernaute O, Nuno L, Bonilla G, Balsa A, Hernandez-Gonzalez F, Grafia I, Prieto-Gonzalez S, Narvaez J, Trallero-Araguas E, Selva-O'Callaghan A, Gualillo O, Castaneda S, Cavagna L, Cifrian JM, Gonzalez-Gay MA (2020) Influence of MUC5B gene on antisynthetase syndrome. Sci Rep 10(1):1415. https://​doi.​org/​10.​1038/​s41598-020-58400-0CrossRefPubMedPubMedCentral
125.
Zurück zum Zitat Sambataro G, Ferro F, Orlandi M, Sambataro D, Torrisi SE, Quartuccio L, Vancheri C, Baldini C, Matucci Cerinic M (2020) Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjgren's syndrome: a systematic review from the Italian Society of Rheumatology. Autoimmun Rev 19(2):102447. https://doi.org/10.1016/j.autrev.2019.102447CrossRefPubMed Sambataro G, Ferro F, Orlandi M, Sambataro D, Torrisi SE, Quartuccio L, Vancheri C, Baldini C, Matucci Cerinic M (2020) Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjgren's syndrome: a systematic review from the Italian Society of Rheumatology. Autoimmun Rev 19(2):102447. https://​doi.​org/​10.​1016/​j.​autrev.​2019.​102447CrossRefPubMed
127.
Zurück zum Zitat Constantopoulos SH, Papadimitriou CS, Moutsopoulos HM (1985) Respiratory manifestations in primary Sjogren's syndrome. A clinical, functional, and histologic study. Chest 88(2):226–229CrossRef Constantopoulos SH, Papadimitriou CS, Moutsopoulos HM (1985) Respiratory manifestations in primary Sjogren's syndrome. A clinical, functional, and histologic study. Chest 88(2):226–229CrossRef
128.
Zurück zum Zitat Papathanasiou MP, Constantopoulos SH, Tsampoulas C, Drosos AA, Moutsopoulos HM (1986) Reappraisal of respiratory abnormalities in primary and secondary Sjogren's syndrome. A controlled study. Chest 90(3):370–374CrossRef Papathanasiou MP, Constantopoulos SH, Tsampoulas C, Drosos AA, Moutsopoulos HM (1986) Reappraisal of respiratory abnormalities in primary and secondary Sjogren's syndrome. A controlled study. Chest 90(3):370–374CrossRef
129.
Zurück zum Zitat Vitali C, Tavoni A, Viegi G, Begliomini E, Agnesi A, Bombardieri S (1985) Lung involvement in Sjogren's syndrome: a comparison between patients with primary and with secondary syndrome. Ann Rheum Dis 44(7):455–461CrossRef Vitali C, Tavoni A, Viegi G, Begliomini E, Agnesi A, Bombardieri S (1985) Lung involvement in Sjogren's syndrome: a comparison between patients with primary and with secondary syndrome. Ann Rheum Dis 44(7):455–461CrossRef
130.
131.
Zurück zum Zitat Ramos-Casals M, Brito-Zeron P, Seror R, Bootsma H, Bowman SJ, Dorner T, Gottenberg JE, Mariette X, Theander E, Bombardieri S, De Vita S, Mandl T, Ng WF, Kruize A, Tzioufas A, Vitali C, Force ESST (2015) Characterization of systemic disease in primary Sjogren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology (Oxford) 54(12):2230–2238. https://doi.org/10.1093/rheumatology/kev200CrossRef Ramos-Casals M, Brito-Zeron P, Seror R, Bootsma H, Bowman SJ, Dorner T, Gottenberg JE, Mariette X, Theander E, Bombardieri S, De Vita S, Mandl T, Ng WF, Kruize A, Tzioufas A, Vitali C, Force ESST (2015) Characterization of systemic disease in primary Sjogren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology (Oxford) 54(12):2230–2238. https://​doi.​org/​10.​1093/​rheumatology/​kev200CrossRef
133.
Zurück zum Zitat Davidson BK, Kelly CA, Griffiths ID (2000) Ten year follow up of pulmonary function in patients with primary Sjogren's syndrome. Ann Rheum Dis 59(9):709–712CrossRef Davidson BK, Kelly CA, Griffiths ID (2000) Ten year follow up of pulmonary function in patients with primary Sjogren's syndrome. Ann Rheum Dis 59(9):709–712CrossRef
134.
Zurück zum Zitat Hatron PY, Wallaert B, Gosset D, Tonnel AB, Gosselin B, Voisin C, Devulder B (1987) Subclinical lung inflammation in primary Sjogren's syndrome: relationship between bronchoalveolar lavage cellular analysis findings and characteristics of the disease. Arthritis Rheum 30(11):1226–1231CrossRef Hatron PY, Wallaert B, Gosset D, Tonnel AB, Gosselin B, Voisin C, Devulder B (1987) Subclinical lung inflammation in primary Sjogren's syndrome: relationship between bronchoalveolar lavage cellular analysis findings and characteristics of the disease. Arthritis Rheum 30(11):1226–1231CrossRef
135.
Zurück zum Zitat Dalavanga YA, Constantopoulos SH, Galanopoulou V, Zerva L, Moutsopoulos HM (1991) Alveolitis correlates with clinical pulmonary involvement in primary Sjogren's syndrome. Chest 99(6):1394–1397CrossRef Dalavanga YA, Constantopoulos SH, Galanopoulou V, Zerva L, Moutsopoulos HM (1991) Alveolitis correlates with clinical pulmonary involvement in primary Sjogren's syndrome. Chest 99(6):1394–1397CrossRef
137.
Zurück zum Zitat Salaffi F, Manganelli P, Carotti M, Baldelli S, Blasetti P, Subiaco S, Binci MC, Bichi Secchi E, Amici F, Cervini C (1998) A longitudinal study of pulmonary involvement in primary Sjogren's syndrome: relationship between alveolitis and subsequent lung changes on high-resolution computed tomography. Br J Rheumatol 37(3):263–269CrossRef Salaffi F, Manganelli P, Carotti M, Baldelli S, Blasetti P, Subiaco S, Binci MC, Bichi Secchi E, Amici F, Cervini C (1998) A longitudinal study of pulmonary involvement in primary Sjogren's syndrome: relationship between alveolitis and subsequent lung changes on high-resolution computed tomography. Br J Rheumatol 37(3):263–269CrossRef
139.
Zurück zum Zitat Gottenberg JE, Cinquetti G, Larroche C, Combe B, Hachulla E, Meyer O, Pertuiset E, Kaplanski G, Chiche L, Berthelot JM, Gombert B, Goupille P, Marcelli C, Feuillet S, Leone J, Sibilia J, Zarnitsky C, Carli P, Rist S, Gaudin P, Salliot C, Piperno M, Deplas A, Breban M, Lequerre T, Richette P, Ghiringhelli C, Hamidou M, Ravaud P, Mariette X, Club Rhumatismes et I, The French Society of R (2013) Efficacy of rituximab in systemic manifestations of primary Sjogren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry. Ann Rheum Dis 72(6):1026–1031. https://doi.org/10.1136/annrheumdis-2012-202293CrossRefPubMed Gottenberg JE, Cinquetti G, Larroche C, Combe B, Hachulla E, Meyer O, Pertuiset E, Kaplanski G, Chiche L, Berthelot JM, Gombert B, Goupille P, Marcelli C, Feuillet S, Leone J, Sibilia J, Zarnitsky C, Carli P, Rist S, Gaudin P, Salliot C, Piperno M, Deplas A, Breban M, Lequerre T, Richette P, Ghiringhelli C, Hamidou M, Ravaud P, Mariette X, Club Rhumatismes et I, The French Society of R (2013) Efficacy of rituximab in systemic manifestations of primary Sjogren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry. Ann Rheum Dis 72(6):1026–1031. https://​doi.​org/​10.​1136/​annrheumdis-2012-202293CrossRefPubMed
143.
Zurück zum Zitat Nakano M, Hasegawa H, Takada T, Ito S, Muramatsu Y, Satoh M, Suzuki E, Gejyo F (2002) Pulmonary diffusion capacity in patients with systemic lupus erythematosus. Respirology 7(1):45–49CrossRef Nakano M, Hasegawa H, Takada T, Ito S, Muramatsu Y, Satoh M, Suzuki E, Gejyo F (2002) Pulmonary diffusion capacity in patients with systemic lupus erythematosus. Respirology 7(1):45–49CrossRef
145.
Zurück zum Zitat Jacobsen S, Petersen J, Ullman S, Junker P, Voss A, Rasmussen JM, Tarp U, Poulsen LH, van Overeem HG, Skaarup B, Hansen TM, Podenphant J, Halberg P (1998) A multicentre study of 513 Danish patients with systemic lupus erythematosus. I. Disease manifestations and analyses of clinical subsets. Clin Rheumatol 17(6):468–477CrossRef Jacobsen S, Petersen J, Ullman S, Junker P, Voss A, Rasmussen JM, Tarp U, Poulsen LH, van Overeem HG, Skaarup B, Hansen TM, Podenphant J, Halberg P (1998) A multicentre study of 513 Danish patients with systemic lupus erythematosus. I. Disease manifestations and analyses of clinical subsets. Clin Rheumatol 17(6):468–477CrossRef
148.
Zurück zum Zitat Stoll T, Seifert B, Isenberg DA (1996) SLICC/ACR Damage Index is valid, and renal and pulmonary organ scores are predictors of severe outcome in patients with systemic lupus erythematosus. Br J Rheumatol 35(3):248–254CrossRef Stoll T, Seifert B, Isenberg DA (1996) SLICC/ACR Damage Index is valid, and renal and pulmonary organ scores are predictors of severe outcome in patients with systemic lupus erythematosus. Br J Rheumatol 35(3):248–254CrossRef
149.
Zurück zum Zitat Weinrib L, Sharma OP, Quismorio FP Jr (1990) A long-term study of interstitial lung disease in systemic lupus erythematosus. Semin Arthritis Rheum 20(1):48–56CrossRef Weinrib L, Sharma OP, Quismorio FP Jr (1990) A long-term study of interstitial lung disease in systemic lupus erythematosus. Semin Arthritis Rheum 20(1):48–56CrossRef
151.
Zurück zum Zitat Gunnarsson R, Aalokken TM, Molberg O, Lund MB, Mynarek GK, Lexberg AS, Time K, Dhainaut AS, Bertelsen LT, Palm O, Irgens K, Becker-Merok A, Nordeide JL, Johnsen V, Pedersen S, Proven A, Garabet LS, Gran JT (2012) Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study. Ann Rheum Dis 71(12):1966–1972. https://doi.org/10.1136/annrheumdis-2011-201253CrossRefPubMed Gunnarsson R, Aalokken TM, Molberg O, Lund MB, Mynarek GK, Lexberg AS, Time K, Dhainaut AS, Bertelsen LT, Palm O, Irgens K, Becker-Merok A, Nordeide JL, Johnsen V, Pedersen S, Proven A, Garabet LS, Gran JT (2012) Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study. Ann Rheum Dis 71(12):1966–1972. https://​doi.​org/​10.​1136/​annrheumdis-2011-201253CrossRefPubMed
153.
Zurück zum Zitat Vegh J, Szilasi M, Soos G, Devenyi K, Dezso B, Soltesz P, Zeher M, Szegedi G, Bodolay E (2005) Interstitial lung disease in mixed connective tissue disease. Orv Hetil 146(48):2435–2443PubMed Vegh J, Szilasi M, Soos G, Devenyi K, Dezso B, Soltesz P, Zeher M, Szegedi G, Bodolay E (2005) Interstitial lung disease in mixed connective tissue disease. Orv Hetil 146(48):2435–2443PubMed
154.
Zurück zum Zitat Kawano-Dourado L, Baldi BG, Kay FU, Dias OM, Gripp TE, Gomes PS, Fuller R, Caleiro MT, Kairalla RA, Carvalho CR (2015) Pulmonary involvement in long-term mixed connective tissue disease: functional trends and image findings after 10 years. Clin Exp Rheumatol 33(2):234–240PubMed Kawano-Dourado L, Baldi BG, Kay FU, Dias OM, Gripp TE, Gomes PS, Fuller R, Caleiro MT, Kairalla RA, Carvalho CR (2015) Pulmonary involvement in long-term mixed connective tissue disease: functional trends and image findings after 10 years. Clin Exp Rheumatol 33(2):234–240PubMed
155.
Zurück zum Zitat Narula N, Narula T, Mira-Avendano I, Wang B, Abril A (2018) Interstitial lung disease in patients with mixed connective tissue disease: pilot study on predictors of lung involvement. Clin Exp Rheumatol 36(4):648–651PubMed Narula N, Narula T, Mira-Avendano I, Wang B, Abril A (2018) Interstitial lung disease in patients with mixed connective tissue disease: pilot study on predictors of lung involvement. Clin Exp Rheumatol 36(4):648–651PubMed
164.
Zurück zum Zitat Sambataro G, Vancheri A, Torrisi SE, Colaci M, Pavone M, Libra A, Martorana E, Rosso R, Pignataro F, Del Papa N, Malatino L, Palmucci S, Sambataro D, Vancheri C (2020) The morphological domain does not affect the rate of progression to defined autoimmune diseases in patients with interstitial pneumonia with autoimmune features. Chest 157(1):238–242. https://doi.org/10.1016/j.chest.2019.08.2175CrossRefPubMed Sambataro G, Vancheri A, Torrisi SE, Colaci M, Pavone M, Libra A, Martorana E, Rosso R, Pignataro F, Del Papa N, Malatino L, Palmucci S, Sambataro D, Vancheri C (2020) The morphological domain does not affect the rate of progression to defined autoimmune diseases in patients with interstitial pneumonia with autoimmune features. Chest 157(1):238–242. https://​doi.​org/​10.​1016/​j.​chest.​2019.​08.​2175CrossRefPubMed
166.
Zurück zum Zitat Moore OA, Goh N, Corte T, Rouse H, Hennessy O, Thakkar V, Byron J, Sahhar J, Roddy J, Gabbay E, Youssef P, Nash P, Zochling J, Proudman SM, Stevens W, Nikpour M (2013) Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford) 52(1):155–160. https://doi.org/10.1093/rheumatology/kes289CrossRef Moore OA, Goh N, Corte T, Rouse H, Hennessy O, Thakkar V, Byron J, Sahhar J, Roddy J, Gabbay E, Youssef P, Nash P, Zochling J, Proudman SM, Stevens W, Nikpour M (2013) Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford) 52(1):155–160. https://​doi.​org/​10.​1093/​rheumatology/​kes289CrossRef
167.
Zurück zum Zitat Christopher P, Denton CJFL, Goldin J, Kim G, Kuwana M, Allanore Y, Batalov A, Butrimiene I, Carreira P, Matucci-Cerinic M, Distler O, Kaliterna DM, Mihai C-M, Mogensen M, Olesińska M, Pope JE, Riemekasten G, Rodriguez-Reyne TS, Santos MJ, Van Laar J, Spotswood H, Siegel J, Jahreis A, Furst DE, Khanna D (2019) Lung function preservation in a phase 3 trial of tocilizumab (TCZ) in systemic sclerosis (SSc). Eur Respir J. https://doi.org/10.1183/13993003CrossRef Christopher P, Denton CJFL, Goldin J, Kim G, Kuwana M, Allanore Y, Batalov A, Butrimiene I, Carreira P, Matucci-Cerinic M, Distler O, Kaliterna DM, Mihai C-M, Mogensen M, Olesińska M, Pope JE, Riemekasten G, Rodriguez-Reyne TS, Santos MJ, Van Laar J, Spotswood H, Siegel J, Jahreis A, Furst DE, Khanna D (2019) Lung function preservation in a phase 3 trial of tocilizumab (TCZ) in systemic sclerosis (SSc). Eur Respir J. https://​doi.​org/​10.​1183/​13993003CrossRef
168.
Zurück zum Zitat Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, Axmann J, Kirchgaessler KU, Samara K, Gilberg F, Cottin V (2020) Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med 8(2):147–157. https://doi.org/10.1016/S2213-2600(19)30341-8CrossRefPubMed Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, Axmann J, Kirchgaessler KU, Samara K, Gilberg F, Cottin V (2020) Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med 8(2):147–157. https://​doi.​org/​10.​1016/​S2213-2600(19)30341-8CrossRefPubMed
Metadaten
Titel
Connective Tissue Disease-Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment
verfasst von
Niranjan Jeganathan
Matheni Sathananthan
Publikationsdatum
11.08.2020
Verlag
Springer US
Erschienen in
Lung / Ausgabe 5/2020
Print ISSN: 0341-2040
Elektronische ISSN: 1432-1750
DOI
https://doi.org/10.1007/s00408-020-00383-w

Weitere Artikel der Ausgabe 5/2020

Lung 5/2020 Zur Ausgabe

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.