The online version of this article (doi:10.1186/1471-2172-15-20) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing or financial interests.
TH – Data analysis, primary manuscript preparer, guarantor of the paper. AH and AI - Data collection. ML– Radiology analysis. CG – Data and statistical analysis. NGM – Data and manuscript reviewer. SJO – Direction of research, data and manuscript reviewer as senior author. All authors read and approved the final manuscript.
Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may present with pulmonary involvement ranging from mild to life-threatening disease such as diffuse alveolar hemorrhage. There is a paucity of information regarding morbidity outcomes for AAV subjects presenting with lung involvement. This study determines the relationship between disease activity and damage in these subjects using the Birmingham Vasculitis Activity Score v 3 (BVAS 3) and Vasculitis Damage Index (VDI) respectively.
151 patients with AAV were included with 59 presenting initially with pulmonary involvement. The initial BVAS scores recorded at time of diagnosis were positively correlated with the final VDI scores at 24 months (p < 0.0001, rs = 0.5871). No differences between BVAS and VDI scores were seen for both groups, however in the lung-involvement group only, BVAS scores were significantly higher at 6, 12 and 24 months whilst the VDI scores were significantly higher at 12 and 24 months. Subjects presenting with pulmonary involvement had an increased likelihood for cardiovascular (OR 1.31, 95% CI 0.89, 1.54; p = 0.032) and renal (OR 1.32, 95% CI 1.22, 1.39; p = 0.005) involvement. Subjects presenting with lung involvement with granulomatosis with polyangiitis and microscopic polyangiitis had 24-month VDI scores that were significantly higher (p = 0.027, p = 0.045), and more likely to develop pulmonary fibrosis (OR 1.79, 95% CI 1.48, 2.12; p < 0.001).
AAV subjects with lung involvement at presentation had a higher disease activity and damage scores at 6, 12 and 24 months follow-up representing a considerable burden of disease despite improvement in overall survival due to the introduction of immunosuppressive therapy.
Additional file 1: Figure S1: BVAS 3 scores at initial presentation. Data were compared by non-parametric t- test (Mann Whitney U) with a significance p-value cut-off of 0.05 (*p < 0.05, **p < 0.01, ***p < 0.001). (PPTX 46 KB)
Little MA, Nightingale P, Verburgh CA, Hauser T, De Groot K, Savage C, Jayne D, Harper L, European Vasculitis Study (EUVAS) Group: Early mortality in systemic vasculitis: relative contribution of adverse events and active vasculitis. Ann Rheum Dis. 2010, 69: 1036-1043. 10.1136/ard.2009.109389. PubMedCrossRef
Gordon M, Luqmani RA, Adu D, Greaves I, Richards N, Michael J, Emery P, Howie AJ, Bacon PA: Relapses in patients with a systemic vasculitis. Q J Med. 1993, 86: 779-789. PubMed
Mahr A, Katsahian S, Varet H, Guillevin L, Hagen EC, Höglund P, Merkel PA, Pagnous C, Rasmussen N, Westman K, Jayne D, French Vasculitis Study Group (FVSG) and the European Vasculitis Society (EUVAS): Revisiting the classification of clinical phenotypes of antineutrophil cytoplasmic antibody-associated vasculitis: a cluster analysis. Ann Rheum Dis. 2013, 72: 1003-1010. 10.1136/annrheumdis-2012-201750. PubMedCrossRef
Hogan SL, Nachman PH, Wilkman AS, Jennette JC, Falk RJ: Prognostic markers in patients with anti-neutrophil cytoplasmic autoantibody associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol. 1996, 7: 23-32. PubMed
Reinhold-Keller E, Beuge N, Latza U, de Groot K, Rudert H, Nölle B, Heller M, Gross WL: An interdisplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 2000, 43: 1021-1032. 10.1002/1529-0131(200005)43:5<1021::AID-ANR10>3.0.CO;2-J. PubMedCrossRef
Zycinska K, Wardyn KA, Tyszko P, Otto M: Analysis of early death based on the prediction model in Wegener’s granulomatosis with pulmonary and renal involvement. J Physiol Pharmacol. 2007, 58 (Suppl 5): 829-837. PubMed
Kostianovsky A, Hauser T, Pagnoux C, Cohen P, Daugas E, Mouthon L, Miossec P, Cordier JP, Gueillevin L, French Vasculitis Study Group (FVSG): Alveolar haemorrhage in ANCA associated vasculitides: 80 patients’ features and prognostic factors. Clin Exp Rheumatol. 2012, 30 (70): S77-S82. PubMed
Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P: The Five Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group cohort. Medicine (Baltimore). 2011, 90: 19-27. 10.1097/MD.0b013e318205a4c6. CrossRef
Luqmani RA, Flossmann O: Outcome in small-vessel systemic vasculitis. J Rheumatol. 2006, 33: 1224-1227. PubMed
Pavone L, Grasselli C, Chierici E, Maggiore U, Garini G, Ronda N, Manganelli P, Pesci A, Rioda WT, Tumiati B, Pavesi G, VAglio A, Buzio C, Secondary and Primer Vasculitides Study Group: Outcome and prognostic factors during the course of primary small-vessel vasculitdes. J Rheumatol. 2006, 33: 1299-1306. PubMed
Hogan SL, Falk RJ, Chin H, Cai J, Jennette CE, Jennette JC, Falk RJ, Guillevin L, Nachman PH: Predictors of relapse and treatment resistant in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. Ann Intern Med. 2005, 143: 621-631. 10.7326/0003-4819-143-9-200511010-00005. PubMedCrossRef
Anderson G, Coles ET, Crane M, Douglas AC, Gibbs AR, Geddes DM, Peel ET, Wood JB: Wegener’s granuloma. A series of 265 British cases seen between 1975 and 1985. A report by a sub-committee of the British Thoracic Society Research Committee. Q J Med. 1992, 83: 427-438. PubMed
Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, Flossmann O, Hall C, Hollywood J, Jayne D, Jones R, Lanyon P, Muir A, Scott D, Young L, Luqmani RA: Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009, 68: 1827-1832. 10.1136/ard.2008.101279. PubMedCrossRef
Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P, Jarrousse B, French Vasculitis Study Group: Long-term follow-up of polyarteritis nodosa, microsocopic polyangiitis and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001, 44: 666-675. 10.1002/1529-0131(200103)44:3<666::AID-ANR116>3.0.CO;2-A. PubMedCrossRef
Koldingsnes W, Nossent JC: Basline features and intial treatment as predictors of remission and relapse in Wegener’s granulomatosis. J Rheumatol. 2003, 30: 80-88. PubMed
Basu N, Jones GT, Fluck N, MacDonald AG, Pang D, Dospinescu P, Reid DM, Macfarlene GJ: Fatigue: a principle contributor to impaired quality of life in ANCA-associated vasculitis. Rheumatology (Oxford). 2010, 49: 1383-1390. 10.1093/rheumatology/keq098. CrossRef
- Lung involvement at presentation predicts disease activity and permanent organ damage at 6, 12 and 24 months follow - up in ANCA - associated vasculitis
Tidi M Hassan
Astrid S Hassan
Noel G McElvaney
Shane J O’Neill
- BioMed Central
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