PMID- 28251541 OWN - NLM STAT- MEDLINE DCOM- 20180322 LR - 20181113 IS - 0973-7693 (Electronic) IS - 0019-5456 (Linking) VI - 84 IP - 4 DP - 2017 Apr TI - Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India. PG - 299-306 LID - 10.1007/s12098-017-2302-8 [doi] AB - 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. FAU - Sarkar, Sumantra AU - Sarkar S AD - Department of Pediatrics, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India. FAU - Mondal, Tanushree AU - Mondal T AD - Department of Community Medicine, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India. FAU - Saha, Arpan AU - Saha A AD - Department of Pediatrics, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India. dr.arpan.saha@gmail.com. AD - Kediabagan Jonepur, Kanchrapara, North 24 PGS, West Bengal, 743145, India. dr.arpan.saha@gmail.com. FAU - Mondal, Rakesh AU - Mondal R AD - Department of Pediatrics, Medical College, Kolkata, India. FAU - Datta, Supratim AU - Datta S AD - Department of Pediatrics, Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata, India. LA - eng PT - Journal Article DEP - 20170302 PL - India TA - Indian J Pediatr JT - Indian journal of pediatrics JID - 0417442 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Immunoglobulins, Intravenous) SB - IM MH - Adrenal Cortex Hormones/therapeutic use MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Immunoglobulins, Intravenous/therapeutic use MH - India MH - Male MH - Myositis/complications/*diagnosis/drug therapy MH - Retrospective Studies MH - Sex Factors MH - Tertiary Care Centers OTO - NOTNLM OT - Dermatomyositis OT - Eastern India OT - Inflammatory OT - Juvenile OT - Myopathy OT - Profile EDAT- 2017/03/03 06:00 MHDA- 2018/03/23 06:00 CRDT- 2017/03/03 06:00 PHST- 2016/09/07 00:00 [received] PHST- 2017/01/30 00:00 [accepted] PHST- 2017/03/03 06:00 [pubmed] PHST- 2018/03/23 06:00 [medline] PHST- 2017/03/03 06:00 [entrez] AID - 10.1007/s12098-017-2302-8 [pii] AID - 10.1007/s12098-017-2302-8 [doi] PST - ppublish SO - Indian J Pediatr. 2017 Apr;84(4):299-306. doi: 10.1007/s12098-017-2302-8. Epub 2017 Mar 2.