ICNC Abstracts, ICNC 2018

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Clinicopathological profile and treatment outcome of Inflammatory myopathies in children: a retrospective cohort study
Prateek Kumar Panda, Vyshakh Anand, Prabhjoth Kaur, Prashant Jauhari, Biswaroop Chakrabarty, Atin Kumar, Manisha Jana, M C Sharma, Vaishali Suri, Chitra Sarkar, Sheffali Gulati

Last modified: 2018-09-09

Abstract


Introduction: Inflammatory myopathies are acquired myopathies in children of autoimmune, infectious or idiopathis etiology.

Methods: Clinical profile of children with inflammatory myopathies in between 2011 and 2018 in a tertiary care centre were retrospectively reviewed.

Result: In 30 children (17 girls, median age 7 years, IQR-5-9 years) with inflammatory myopathies, commonest clinical features were proximal muscle weakness (86%), muscle pain(80%), skin rash(60%), fever(50%) and bulbar muscle weakness(26%). 28/30 children had elevated serum CPK levels(median: 610, IQR-388-4213).

Apart from 5 children with viral myositis, muscle biopsy done in 25 children(83%) revealed Juvenile dermatomyositis (JDM) Juvenile Polymyositis (JPM) and granulomatous myositis in 20, 4 and 1 children respectively. Median duration from symptom onset to diagnosis in  JDM and JPM was 6 months (IQR 4-12 months).

Commonest muscle MRI (done in 13/24 cases) abnormality in JDM and JPM was T2 hyperintensity in gluteal and thigh muscles. None of them were positive for muscle specific antibodies. 1 child each were positive for ANA and Anti TTG.

All viral myositis cases recovered completely with analgesics and supportive therapy. 23/24 cases of JDM and JPM responded to initial methyl prednisolone pulse followed by oral steroid. 1 child with HIV associated JDM expired following systemic complications. Oral/subcutaneous Methotrexate(20/24) and Azathioprine(3/24) were used as long term immunomodulators . 5/24 cases relapsed within 6 months-2 years, required repeat steroid pulse, IVIg(3/24 cases) and Rituximab(1/24 case).

Conclusion: Early diagnosis and appropriate immunotherapy is empirical for good clinical outcome in JDM and JPM.


Keywords


inflammatory myopathy;children;clinicopathological profile

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