Rheumatology International

, Volume 39, Issue 3, pp 577–581 | Cite as

Juvenile dermatomyositis with IgA nephropathy: case-based review

  • Mohsin Raj Mantoo
  • Saroj Kumar Tripathy
  • Ravi Hari Phulware
  • Narendra Kumar BagriEmail author
  • Pankaj Hari
  • Adarsh Barwad
Cases with a Message


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.


Juvenile dermatomyositis Acute kidney injury IgA nephropathy 



We acknowledge Dr Ayush for giving critical inputs in the preparation of manuscript.

Author contributions

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.

Ethical approval

This article does not contain any studies on human participants or animals performed by any authors.

Informed Consent

Informed consent of parents of patient concerned was taken in an appropriate format.


  1. 1.
    Shah M, Mamyrova G, Targoff IN, Huber AM, Malley JD, Rice MM et al (2013) The clinical phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore) 92:25–41CrossRefGoogle Scholar
  2. 2.
    Peloro TM, Miller OF, Hahn TF, Newman ED (2001) Juvenile dermatomyositis: a retrospective review of a 30-year experience. J Am Acad Dermatol 45(1):28–34CrossRefGoogle Scholar
  3. 3.
    Rider LG, Lindsley CB, Cassidy JT (2011) Juvenile dermatomyositis. In: Cassidy JT, Petty RE, Laxer RM et al (eds) Textbook of pediatric rheumatology, 6th edn. Saunders, Philadelphia, p 410 (Table 24–20) Google Scholar
  4. 4.
    Bohan A, Peter JB (1975) Polymyositis and dermatomyositis. N Engl J Med 292:344–347 (403–407) CrossRefGoogle Scholar
  5. 5.
    Yen T-H, Lai P-C, Chen C-C, Hsueh S, Huang J-Y (2005) Renal involvement in patients with polymyositis and dermatomyositis. Int J Clin Pract 59(2):188–193CrossRefGoogle Scholar
  6. 6.
    Cucchiari D, Angelini C (2017) Renal involvement in idiopathic inflammatory myopathies. Clin Rev Allergy Immunol 52(1):99–107CrossRefGoogle Scholar
  7. 7.
    Nickavar A, Mehrazma M (2012) Nephrotic syndrome and juvenile dermatomyositis. Rheumatol Int 32(9):2933–2935CrossRefGoogle Scholar
  8. 8.
    Civilibal M, SelcukDuru N, Ozagari A, Durali K, Elevli M (2009) Immunoglobulin A nephropathy associated with juvenile dermatomyositis. Pediatr Nephrol 24(10):2073–2075CrossRefGoogle Scholar
  9. 9.
    Ronco P, Debiec H (2009) Pathophysiological lessons from rare associations of immunological disorders. Pediatr Nephrol Berl Ger 24(1):3–8CrossRefGoogle Scholar
  10. 10.
    Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB et al (2011) The pathophysiology of IgA nephropathy. J Am Soc Nephrol JASN 22(10):1795–1803CrossRefGoogle Scholar
  11. 11.
    Saha MK, Julian BA, Novak J, Rizk DV (2018) Secondary IgA nephropathy. Kidney Int 94(4):674–681CrossRefGoogle Scholar
  12. 12.
    Suzuki K, Honda K, Tanabe K, Toma H, Nihei H, Yamaguchi Y (2003) Incidence of latent mesangial IgA deposition in renal allograft donors in Japan. Kidney Int 63(6):2286–2294CrossRefGoogle Scholar
  13. 13.
    Wyatt RJ, Julian BA (2013) IgA nephropathy. N Engl J Med 368(25):2402–2414CrossRefGoogle Scholar
  14. 14.
    Barros TBM, Souza FHC de, Malheiros DMAC, Levy Neto M, Shinjo SK (2014) Nefropatiapor IgA e polimiosite: umararaassociação. Rev Bras Reumatol 54(3):231–233CrossRefGoogle Scholar
  15. 15.
    Oh Y-J, Park ES, Jang M, Kang EW, Kie J-H, Lee S-W et al (2017) A case of polymyositis associated with immunoglobulin A nephropathy. J Rheum Dis 24(4):241CrossRefGoogle Scholar
  16. 16.
    Machado NP, Camargo CZ, Oliveira ACD, Buosi ALP, Pucinelli MLC, Souza AWS (2010) Association of anti-glomerular basement membrane antibody disease with dermatomyositis and psoriasis: case report. Sao Paulo Med J 128(5):306–308CrossRefGoogle Scholar
  17. 17.
    Yuste C, Rapalai M, Pritchard BA, Jones TJ, Amoasii C, Al-Ansari A et al (2014) Overlap between dermatomyositis and ANCA vasculitides. Clin Kidney J 7(1):59–61CrossRefGoogle Scholar
  18. 18.
    Xie Q, Liu Y, Liu G, Yang N, Yin G (2010) Diffuse proliferative glomerulonephritis associated with dermatomyositis with nephrotic syndrome. Rheumatol Int 30(6):821–825CrossRefGoogle Scholar
  19. 19.
    Couvrat-Desvergnes G, Masseau A, Benveniste O, Bruel A, Hervier B, Mussini J-M et al (2014) The spectrum of renal involvement in patients with inflammatory myopathies. Medicine (Baltimore) 93(1):33–41CrossRefGoogle Scholar
  20. 20.
    Akashi Y, Inoh M, Gamo N, Kinashi M, Ohbayashi S, Miyake H et al (2002) Dermatomyositis associated with membranous nephropathy in a 43-year-old female. Am J Nephrol 22(4):385–388CrossRefGoogle Scholar
  21. 21.
    Makino H, Hirata K, Matsuda M, Amano T, Ota Z (1994) Membranous nephropathy developing during the course of dermatomyositis. J Rheumatol 21(7):1377–1378Google Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PediatricsAll India Institute of Medical SciencesNew DelhiIndia
  2. 2.Department of PathologyAll India Institute of Medical SciencesNew DelhiIndia
  3. 3.Division of Rheumatology, Department of PediatricsAll India Institute of Medical Sciences (AIIMS)New DelhiIndia
  4. 4.Division of Nephrology, Department of PediatricsAll India Institute of Medical SciencesNew DelhiIndia

Personalised recommendations