Filipa Santos Cardoso
1* 
, David Barrote Navarro Dias
1 , Marco Sandro Oliveira Mendes
1 , Mário Rui Ventura Góis
1 , Helena Sousa Viana
1 , Fernando Eduardo Barbosa Nolasco
1 1 Department of Nephrology Hospital Curry Cabral, Lisboa, Portugal
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.