Juvenile dermatomyositis with IgA nephropathy: case-based review
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Juvenile dermatomyositis (JDM) is the most common childhood idiopathic inflammatory myopathy (IIM). It is characterized by the classic skin rash in the form of Gottron papules and heliotrope rash, and symmetric proximal muscle weakness. Renal involvement in JDM is rare which includes acute kidney injury and glomerulonephritis. We report a 10-year-old boy with juvenile dermatomyositis and IgA nephropathy. Child responded dramatically to the conventional therapy with steroids and methotrexate for the primary disease, and did not require any additional treatment for his renal disease. Child’s primary disease is in remission and has normal urinalysis with normal renal function at 6-month follow-up. We reviewed the literature and found 11 cases of IIMs with renal involvement. Four patients (one JDM, two polymyositis, and one dermatomyositis) had IgA nephropathy out of which three patients responded to the conventional therapy of primary disease and only one patient with polymyositis needed hiking immunosuppression targeted for renal condition. Therapy targeting the underlying disorder is usually sufficient in patients with JDM and secondary IgA nephropathy.
KeywordsJuvenile dermatomyositis Acute kidney injury IgA nephropathy
We acknowledge Dr Ayush for giving critical inputs in the preparation of manuscript.
MRM: prepared the manuscript. SKT: collected data of patient and reviewed the literature. RHP: pathological part of manuscript was prepared along with review of current and old cases. NKB: edited the manuscript and gave critical inputs for preparation of manuscript. PH: reviewed the draft and final revision of the manuscript. AB: analysis and interpretation of pathological findings of cases and inclusion in the main draft. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.
Compliance with ethical standards
Conflict of interest
All authors declare that there are no conflicts of interest.
This article does not contain any studies on human participants or animals performed by any authors.
Informed consent of parents of patient concerned was taken in an appropriate format.
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