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- Title
A fatal case of severe gastrointestinal and renal involvement in Henoch-Schönlein purpura.
- Authors
Santos Cardoso, Filipa; Navarro Dias, David Barrote; Oliveira Mendes, Marco Sandro; Ventura Góis, Mário Rui; Viana, Helena Sousa; Barbosa Nolasco, Fernando Eduardo
- 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.
- Subjects
SCHOENLEIN-Henoch purpura; GASTROINTESTINAL hemorrhage; ACUTE kidney failure; HEMORRHAGIC shock; OLDER patients
- Publication
Journal of Nephropathology, 2019, Vol 8, Issue 3, p1
- ISSN
2251-8363
- Publication type
Article
- DOI
10.15171/jnp.2019.31