Juvenile dermatomyositis with IgA nephropathy: case-based review

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 in...

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Bibliographic Details
Published in:Rheumatology international 2019-03, Vol.39 (3), p.577-581
Main Authors: Mantoo, Mohsin Raj, Tripathy, Saroj Kumar, Phulware, Ravi Hari, Bagri, Narendra Kumar, Hari, Pankaj, Barwad, Adarsh
Format: Article
Language:English
Subjects:
Antirheumatic Agents - therapeutic use
Biopsy
Cases with a Message
Child
Dermatomyositis - complications
Dermatomyositis - drug therapy
Disease
Fever
Glomerulonephritis, IGA - etiology
Glomerulonephritis, IGA - pathology
Glucocorticoids - therapeutic use
Hematuria
Humans
Inflammatory diseases
Kidneys
Magnetic resonance imaging
Male
Medicine
Medicine & Public Health
Methotrexate - therapeutic use
Musculoskeletal diseases
Patients
Pediatrics
Prednisolone - therapeutic use
Proteins
Rheumatology
Treatment Outcome
Urine
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Summary: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.
ISSN:0172-8172
1437-160X
DOI:10.1007/s00296-018-4229-4