Society of Diabetic Nephropathy Prevention Journal of Nephropathology 2251-8363 8 3 2019 07 01 A fatal case of severe gastrointestinal and renal involvement in Henoch–Schönlein purpura e31 e31 10.15171/jnp.2019.31 EN Filipa Santos Cardoso https://orcid.org/0000-0002-0114-6787 David Barrote Navarro Dias https://orcid.org/0000-0002-1977-794X Marco Sandro Oliveira Mendes https://orcid.org/0000-0002-5109-0697 Mário Rui Ventura Góis https://orcid.org/0000-0002-0937-8611 Helena Sousa Viana https://orcid.org/0000-0002-7644-2230 Fernando Eduardo Barbosa Nolasco https://orcid.org/0000-0002-7089-1975 Journal Article 10.15171/jnp.2019.31 2018 12 11 2019 03 20 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.