title: Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Pediatrics, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India.
authors.forename: Sumantra
authors.initials: S
authors.lastname: Sarkar
authors.affiliation: Department of Community Medicine, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India.
authors.forename: Tanushree
authors.initials: T
authors.lastname: Mondal
authors.affiliation: Department of Pediatrics, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India. dr.arpan.saha@gmail.com.|Kediabagan Jonepur, Kanchrapara, North 24 PGS, West Bengal, 743145, India. dr.arpan.saha@gmail.com.
authors.forename: Arpan
authors.initials: A
authors.lastname: Saha
authors.affiliation: Department of Pediatrics, Medical College, Kolkata, India.
authors.forename: Rakesh
authors.initials: R
authors.lastname: Mondal
authors.affiliation: Department of Pediatrics, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India.
authors.forename: Supratim
authors.initials: S
authors.lastname: Datta
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007194:India; D008297:Male; D009220:Myositis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D012189:Retrospective Studies; D012737:Sex Factors; D062606:Tertiary Care Centers
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous
keywords: Dermatomyositis; Eastern India; Inflammatory; Juvenile; Myopathy; Profile