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Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India

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Abstract

Objectives

To report data on Idiopathic inflammatory myopathies (IIM) from eastern India.

Methods

All IIM patients diagnosed over the last 5 y (2011–2016) were included through a retrospective review of records from the hospital and specialty clinic at Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata.

Results

Out of the 11 IIM patients, 9 had Juvenile dermatomyositis (JDM) and 2 had overlap myositis (OM) [with systemic lupus erythematosus (SLE) and scleroderma]. The overall sex ratio was M: F = 1: 2.6. The mean age at diagnosis was 6.94 y for JDM and 7 y for OM. The mean interval from onset to diagnosis was 5.2 mo. All patients had heliotrope rash and proximal myopathy (n = 11,100%). Other findings included Gottron papule (n = 7; 64%), arthritis (n = 6; 54%), malar rash (n = 5; 45%), dysphagia (n = 4; 36%), nasal intonation (n = 3; 27%), subcutaneous nodule (n = 2; 18%), cutaneous sinus (n = 1; 9%), calcinosis universalis (n = 1; 9%), GI bleed (n = 1; 9%). All patients had raised erythrocyte sedimentation rate (ESR), aspartate aminotransferase (AST), lactate dehydrogenase (LDH) while 10 had raised creatine kinase (CK). Three were anti-nuclear-antibody (ANA) positive. Electromyography (EMG) showed proximal myopathy in most cases (n = 9; 82%). All patients received corticosteroid. Intravenous immunoglobulin (IVIG) was given to 2 patients. Two received hydroxychloroquine. Cyclophosphamide and azathioprine were given in one each.

Conclusions

This study, first reported profile of IIM from eastern India, showed JDM as the commonest form of IIM with a female preponderance. Five children had complete and 2 had partial remission. Two patients of JDM and 1 of OM died. Increased awareness, early referral, prompt diagnosis and treatment might improve the outcome and survival.

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References

  1. Dimachkie MM, Barohn RJ, Amato AA. Idiopathic inflammatory myopathies. Neurol Clin. 2014;32:595–628.

    Article  Google Scholar 

  2. Lazarou IN, Guerne PA. Classification, diagnosis, and management of idiopathic inflammatory myopathies. J Rheumatol. 2013;40:550–64.

    Article  CAS  Google Scholar 

  3. Lorenzoni PJ, Scola RH, Kay CS, Prevedello PG, Espíndola G, Werneck LC. Idiopathic inflammatory myopathies in childhood: a brief review of 27 cases. Pediatr Neurol. 2011;45:17–22.

    Article  Google Scholar 

  4. Rider LG, Lindsley CB, Miller FW. Juvenile dermatomyositis. In: Petty RE, Laxer RM, Lindsley CB, Lucy Wedderburn L, editors. Textbook of Pediatric Rheumatology. 7th ed. Philadelphia: Saunders; 2016. p. 351–83.

    Chapter  Google Scholar 

  5. Singh S, Kumar L, Shankar KR. Juvenile dermatomyositis in North India. Indian Pediatr. 1997;34:193–8.

    CAS  PubMed  Google Scholar 

  6. Singh S, Bansal A. Twelve years experience of juvenile dermatomyositis in North India. Rheumatol Int. 2006;26:510–5.

    Article  Google Scholar 

  7. Singh S, Suri D, Aulakh R, Gupta A, Rawat A, Kumar RM. Mortality in children with juvenile dermatomyositis: two decades of experience from a single tertiary care centre in North India. Clin Rheumatol. 2014;33:1675–9.

    Article  Google Scholar 

  8. Khubchandani RP, Mankad D, Chickermane PR. Disease patterns in juvenile dermatomyositis patients from Mumbai, India. Pediatr Rheumatol Online J. 2011;9:P53.

  9. Chickermane PR, Mankad D, Khubchandani RP. Disease patterns of juvenile dermatomyositis from western India. Indian Pediatr. 2013;50:961–3.

    Article  Google Scholar 

  10. Chowdhary V, Wakhlu A, Aggarwal A, Misra R. Outcome in juvenile dermatomyositis. Indian Pediatr. 2002;39:931–5.

    PubMed  Google Scholar 

  11. Prasad S, Misra R, Agarwal V, Lawrence A, Aggarwal A. Juvenile dermatomyositis at a tertiary care hospital: is there any change in the last decade? Int J Rheum Dis. 2013;16:556–60.

    Article  Google Scholar 

  12. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292:403–7.

    Article  CAS  Google Scholar 

  13. McCann LJ, Juggins AD, Maillard SM, et al. The juvenile dermatomyositis National Registry and repository (UK and Ireland)- clinical characteristics of children recruited within the first 5 yr. Rheumatology. 2006;45:1255–60.

    Article  CAS  Google Scholar 

  14. Mathiesen PR, Zak M, Herlin T, Nielsen SM. Clinical features and outcome in a Danish cohort of juvenile dermatomyositis patients. Clin Exp Rheumatol. 2010;28:782–9.

    CAS  PubMed  Google Scholar 

  15. Sallum AM, Kiss MH, Sachetti S, et al. Juvenile dermatomyositis: clinical, laboratorial, histological, therapeutical and evolutive parameters of 35 patients. Arq Neuropsiquiatr. 2002;60:889–99.

    Article  Google Scholar 

  16. Pachman LM, Hayford JR, Chung A, et al. Juvenile dermatomyositis at diagnosis: clinical characteristics of 79 children. J Rheumatol. 1998;25:1198–204.

    CAS  PubMed  Google Scholar 

  17. Mondal R, Sarkar S, Nandi M, et al. Overlap syndrome: a child cohort. Indian J Pediatr. 2013;80:434–5.

    Article  Google Scholar 

  18. Jayanthi MR, Basher RK, Bhadada SK, Bhattacharya A, Mittal BR. A case of adult dermatomyositis with calcinosis universalis. Indian J Nucl Med. 2014;29:278–9.

    Article  Google Scholar 

  19. Terroso G, Bernardes M, Aleixo A, et al. Therapy of calcinosis universalis complicating adult dermatomyositis. Acta Reumatol Port. 2013;38:44–8.

    PubMed  Google Scholar 

  20. Touimy M, Janani S, Rachidi W, Etaouil N, Mkinsi O. Calcinosis universalis complicating juvenile dermatomyositis: improvement after intravenous immunoglobulin therapy. Joint Bone Spine. 2013;80:108–9.

    Article  Google Scholar 

  21. Jazayeri SB, Mehdizadeh M, Shahlaee A. Sketched x-rays: calcinosis universalis. Eur J Pediatr. 2012;171:1577–8.

    Article  Google Scholar 

  22. Yun SJ, Lee JB, Kim SJ, Lee SC, Won YH, Kang HC. Calcinosis cutis universalis with joint contractures complicating juvenile dermatomyositis. Dermatology. 2006;212:401–3.

    Article  Google Scholar 

  23. Boelch SP, Barthel T, Goebel S, Rudert M, Plumhoff P. Calcinosis universalis of the elbow: a rare case with classical presentation. Case Rep Orthop. 2015;2015:505420. doi:10.1155/2015/505420.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Malik A, Hayat G, Kalia JS, Guzman MA. Idiopathic inflammatory myopathies: clinical approach and management. Front Neurol. 2016;7:64.

    Article  Google Scholar 

  25. Malattia C, Damasio MB, Madeo A, et al. Whole-body MRI in the assessment of disease activity in juvenile dermatomyositis. Ann Rheum Dis. 2014;73:1083–90.

    Article  Google Scholar 

  26. Enders FB, Bader-Meunier B, Baildam E, et al. Consensus-based recommendations for the management of juvenile dermatomyositis. Ann Rheum Dis. 2016. doi:10.1136/annrheumdis-2016-209247.

    Article  Google Scholar 

  27. Huber AM. Idiopathic inflammatory myopathies in childhood: current concepts. Pediatr Clin N Am. 2012;59:365–80.

    Article  Google Scholar 

  28. Huber AM, Giannini EH, Bowyer SL, et al. Protocols for the initial treatment of moderately severe juvenile dermatomyositis: results of a Children's arthritis and rheumatology research alliance consensus conference. Arthritis Care Res (Hoboken). 2010;62:219–25.

    Article  CAS  Google Scholar 

  29. Huber AM, Mamyrova G, Lachenbruch PA, et al. Childhood myositis heterogeneity collaborative study group. Early illness features associated with mortality in the juvenile idiopathic inflammatory myopathies. Arthritis Care Res. 2014;66:732–40.

    Article  Google Scholar 

  30. Ravelli A, Trail L, Ferrari C, et al. Long-term outcome and prognostic factors of juvenile dermatomyositis: a multinational, multicenter study of 490 patients. Arthritis Care Res. 2010;62:63–72.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pediatrics, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India

    Sumantra Sarkar, Arpan Saha & Supratim Datta

  2. Department of Community Medicine, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India

    Tanushree Mondal

  3. Kediabagan Jonepur, Kanchrapara, North 24 PGS, West Bengal, 743145, India

    Arpan Saha

  4. Department of Pediatrics, Medical College, Kolkata, India

    Rakesh Mondal

Authors
  1. Sumantra Sarkar
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  2. Tanushree Mondal
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  4. Rakesh Mondal
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  5. Supratim Datta
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Corresponding author

Correspondence to Arpan Saha.

Ethics declarations

SD, RM, SS, AS were involved in management of cases. TM and AS collected the data, drafted the manuscript, did the analysis of cases. AS, SS, TM searched the literature. SD,SS, TM did the critical review. All the authors approved the final version of the manuscript. SD remains the guarantor.

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Sarkar, S., Mondal, T., Saha, A. et al. Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India. Indian J Pediatr 84, 299–306 (2017). https://doi.org/10.1007/s12098-017-2302-8

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  • DOI: https://doi.org/10.1007/s12098-017-2302-8

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