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05.07.2023 | Observational Research

Long-standing and poorly controlled disease in juvenile dermatomyositis is associated with calcinosis: a real-world experience from a low-middle income country

verfasst von: Pankti Mehta, Able Lawrence, Latika Gupta, Durga P. Misra, Vikas Agarwal, Ramnath Misra, Amita Aggarwal

Erschienen in: Rheumatology International

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Abstract

To study the prevalence and predictors of calcinosis in Juvenile Dermatomyositis (JDM). Medical records over 20 years at a tertiary care rheumatology center in Northern India were reviewed to identify patients with JDM and clinical details were recorded. The frequency of calcinosis, predictors, specific treatment, and its outcomes were studied. Data are expressed as median and interquartile range. In eighty-six patients (median age 10) of JDM, the frequency of calcinosis was 18.2% (8.5% at presentation). Younger age at presentation, longer follow-up, heliotrope rash [Odds Ratio (95% confidence interval), 11.4 (1.4–92.12)], chronic or polycyclic course [4.4 (1.2–15.5)] and cyclophosphamide use [8.2 (1.6–41.9)] were associated with calcinosis. Dysphagia [0.14 (0.02–1.2)] and elevated muscle enzymes [0.14 (0.04–0.5)] were negatively associated with calcinosis. Treatment with pamidronate had a good to moderate response to calcinosis in five of seven children. Calcinosis in JDM is associated with long-standing, poorly controlled disease, and the use of bisphosphonates like pamidronate offer promise in the future for its treatment.

Lundberg IE, Tjärnlund A, Bottai M et al (2017) 2017 European league against rheumatism/American college of rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Arthritis Rheumatol 69:2271–2282. https://doi.org/10.1002/art.40320CrossRefPubMedPubMedCentral

Ravelli A, Trail L, Ferrari C et al (2010) Long-term outcome and prognostic factors of juvenile dermatomyositis: a multinational, multicenter study of 490 patients. Arthritis Care Res 62:63–72. https://doi.org/10.1002/acr.20015CrossRef

Ramanan AV, Feldman BM (2002) Clinical features and outcomes of juvenile dermatomyositis and other childhood onset myositis syndromes. Rheum Dis Clin North Am 28:833–857. https://doi.org/10.1016/s0889-857x(02)00024-8CrossRefPubMed

Pachman LM (2015) Juvenile dermatomyositis and other inflammatory myopathies in children. In: Pachman Lauren M (ed) Neuromuscul disord of infancy, child, and adolesc: a clin approach. Elsevier, Amsterdam

Pachman LM, Khojah AM (2018) Advances in juvenile dermatomyositis: myositis specific antibodies aid in understanding disease heterogeneity. J Pediatr 195:16–27. https://doi.org/10.1016/j.jpeds.2017.12.053CrossRefPubMedPubMedCentral

Tansley SL (2016) Antibodies in juvenile-onset myositis. Curr Opin Rheumatol 28:645–650. https://doi.org/10.1097/BOR.0000000000000330CrossRefPubMed

Gunawardena H (2017) The clinical features of myositis-associated autoantibodies: a review. Clin Rev Allergy Immunol 52:45–57. https://doi.org/10.1007/s12016-015-8513-8CrossRefPubMed

Traineau H, Aggarwal R, Monfort J-B et al (2020) Treatment of calcinosis cutis in systemic sclerosis and dermatomyositis: a review of the literature. J Am Acad Dermatol 82:317–325. https://doi.org/10.1016/j.jaad.2019.07.006CrossRefPubMed

Saini I, Kalaivani M, Kabra SK (2016) Calcinosis in juvenile dermatomyositis: frequency, risk factors and outcome. Rheumatol Int 36:961–965. https://doi.org/10.1007/s00296-016-3467-6CrossRefPubMed

10.

Singh S, Bansal A (2006) Twelve years experience of juvenile dermatomyositis in North India. Rheumatol Int 26:510–515. https://doi.org/10.1007/s00296-005-0030-2CrossRefPubMed

11.

Prasad S, Misra R, Agarwal V et al (2013) Juvenile dermatomyositis at a tertiary care hospital: is there any change in the last decade? Int J Rheum Dis 16:556–560. https://doi.org/10.1111/1756-185X.12053CrossRefPubMed

12.

Hoeltzel MF, Oberle EJ, Robinson AB et al (2014) The presentation, assessment, pathogenesis, and treatment of calcinosis in juvenile dermatomyositis. Curr Rheumatol Rep 16:467. https://doi.org/10.1007/s11926-014-0467-yCrossRefPubMedPubMedCentral

13.

Stringer E, Singh-Grewal D, Feldman BM (2008) Predicting the course of juvenile dermatomyositis: significance of early clinical and laboratory features. Arthritis Rheum 58:3585–3592. https://doi.org/10.1002/art.23960CrossRefPubMed

14.

Sanner H, Gran J-T, Sjaastad I, Flatø B (2009) Cumulative organ damage and prognostic factors in juvenile dermatomyositis: a cross-sectional study median 16.8 years after symptom onset. Rheumatol Oxf Engl 48:1541–1547. https://doi.org/10.1093/rheumatology/kep302CrossRef

15.

Robinson AB, Reed AM (2011) Clinical features, pathogenesis and treatment of juvenile and adult dermatomyositis. Nat Rev Rheumatol 7:664–675. https://doi.org/10.1038/nrrheum.2011.139CrossRefPubMed

16.

Orandi AB, Baszis KW, Dharnidharka VR et al (2017) Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA). Pediatr Rheumatol Online J. https://doi.org/10.1186/s12969-017-0199-4CrossRefPubMedPubMedCentral

17.

Pachman LM, Abbott K, Sinacore JM et al (2006) Duration of illness is an important variable for untreated children with juvenile dermatomyositis. J Pediatr 148:247–253. https://doi.org/10.1016/j.jpeds.2005.10.032CrossRefPubMed

18.

McHugh NJ, Tansley SL (2018) Autoantibodies in myositis. Nat Rev Rheumatol 14:290–302. https://doi.org/10.1038/nrrheum.2018.56CrossRefPubMed

19.

Tsaltskan V, Aldous A, Serafi S et al (2020) Long-term outcomes in juvenile myositis patients. Semin Arthritis Rheum 50:149–155. https://doi.org/10.1016/j.semarthrit.2019.06.014CrossRefPubMed

20.

Patwardhan A, Rennebohm R, Dvorchik I, Spencer CH (2012) Is juvenile dermatomyositis a different disease in children up to three years of age at onset than in children above three years at onset? A retrospective review of 23 years of a single center’s experience. Pediatr Rheumatol Online J 10:34. https://doi.org/10.1186/1546-0096-10-34CrossRefPubMedPubMedCentral

Titel: Long-standing and poorly controlled disease in juvenile dermatomyositis is associated with calcinosis: a real-world experience from a low-middle income country
verfasst von: Pankti Mehta
Able Lawrence
Latika Gupta
Durga P. Misra
Vikas Agarwal
Ramnath Misra
Amita Aggarwal
Publikationsdatum: 05.07.2023
Verlag: Springer Berlin Heidelberg
Erschienen in: Rheumatology International
Print ISSN: 0172-8172
Elektronische ISSN: 1437-160X
DOI: https://doi.org/10.1007/s00296-023-05377-4

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