J Nephropathol. 2019;8(3): e31.
doi: 10.15171/jnp.2019.31
  Abstract View: 299
  PDF Download: 156

Case Report

A fatal case of severe gastrointestinal and renal involvement in Henoch–Schönlein purpura

Abstract

Henoch-Schönlein purpura (HSP) is an immune-complex mediated vasculitis affecting small vessels with dominant IgA deposits. It is seen mostly in children, with a self-limiting disease, but can present with more severe clinical features in older patients, such as gastrointestinal (GI) involvement, with a propensity for rapid progression. In this report, we describe our experience with a male HSP patient who presented with pneumonia, palpable purpuric rash, severe GI involvement with hemodynamic compromise and acute kidney injury. Even though we escalated therapy over time given the lack of response with each previous strategy, with corticosteroids and cyclophosphamide, he developed massive lower gastrointestinal hemorrhage that was not responsive to any supportive measure and died as a result of hemorrhagic shock. There was no established protocol that guided this treatment due to lack of rigorous data, which emphasizes the need for more studies on adult HSP in order to establish the optimal management for HSP patients with severe gastrointestinal manifestations.

Implication for health policy/practice/research/medical education:

HSP is a small vessel vasculitis which rarely presents in adults. As a consequence, there is a paucity of clinical studies and no treatment guidelines exist. Additional research is vital to define management strategies for HSP in adults. We describe a rare presentation of HSP in an adult male with a fatal outcome.

Please cite this paper as: Cardoso FS, Navarro Dias DB, Oliveira Mendes MS, Ventura Góis MR, Viana HS, Barbosa Nolasco FE. A fatal case of severe gastrointestinal and renal involvement in Henoch–Schönlein purpura. J Nephropathol. 2019;8(3):e31. DOI: 10.15171/ jnp.2019.31.

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Submitted: 11 Dec 2018
Accepted: 20 Mar 2019
First published online: 14 Apr 2019
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