Rapid evolution of IGA nephropathy into Henoch-Schönlein Purpura nephritis in a pediatric patient: A case report

Cahyani Gita Ambarsari, Yoshua Martin Luther, Agustina Kadaristiana, Meilania Saraswati

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review

Abstract

We report a case of a child with Henoch-Schönlein purpura (HSP) who developed symptoms of hematuria, edema, proteinuria, and hypoalbuminemia 1 year before. Therefore, the initial diagnosis was acute post-streptococcal glomerulonephritis (APSGN) and nephrotic syndrome. Proteinuria had improved, but hematuria had persisted and deteriorated upon the development of an upper respiratory tract infection (URTI), which prompted a revised diagnosis of immunoglobulin A (IgA) nephropathy. About 6 months later, the patient suddenly developed renal dysfunction and a palpable rash as well as arthralgia; thus, a second diagnosis of HSP nephritis (HSPN) was made. The first renal biopsy demonstrated mesangial hypercellularity, focal segmental glomerulosclerosis, and formation of one crescent-shaped glomerulus. The second renal biopsy following the onset of acute kidney injury showed more endocapillary hypercellularity along with slightly more tubular atrophy and interstitial fibrosis. High-dose intravenous methylprednisolone improved the symptoms, particularly by reverting kidney function back to normal. HSP is an IgA-associated systemic disease that causes vasculitis. When in a mild state, HSP can spontaneously resolve; however, moderate to severe HSP usually requires systemic steroid administration. This case report suggests that HSPN may not present with the classic tetrad of nonthrombocytopenic palpable purpura, arthritis, or arthralgia, with gastrointestinal and renal involvement at the early onset of disease. In cases initially categorized as IgA nephropathy (IgAN), a close follow-up is required as the disease may evolve to HSPN over a short period of time, as in this case (within 6 months).

Original languageEnglish
Title of host publicationMedical Case Reports
PublisherNova Science Publishers, Inc.
Pages437-447
Number of pages11
ISBN (Electronic)9781536168853
ISBN (Print)9781536168846
Publication statusPublished - 14 Feb 2020

Keywords

  • Case report
  • Glomerulonephritis IgA
  • Hematuria
  • IgA
  • Proteinuria
  • Purpura
  • Schoenlein-Henoch

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