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.
Highlights
Henoch-Schönlein purpura (HSP) is an immunecomplex mediated small vessels vasculitis with dominant immunoglobulin A (IgA) deposits [1]
We describe a rare presentation of HSP in an adult male with a fatal outcome
It usually presents with the classic symptoms of purpura, arthritis/arthralgia, abdominal pain and renal disease [2]
Summary
Henoch-Schönlein purpura (HSP) is an immunecomplex mediated small vessels vasculitis with dominant immunoglobulin A (IgA) deposits [1] It usually presents with the classic symptoms of purpura, arthritis/arthralgia, abdominal pain and renal disease [2]. The laboratory tests revealed an acute kidney injury (serum creatinine [sCr] level of 3.26 mg/dL) and hematoproteinuria (proteinuria of 50 mg/dL on dipstick and 417 red blood cells [RBCs] per high power field on automated urinalysis) He underwent workup for possible vasculitis and glomerulonephritis, including antinuclear antibody, perinuclear anti-neutrophil cytoplasmic antibodies, cytoplasmic anti neutrophil cytoplasmic antibodies, antiglomerular basement membrane serologies, complement levels, cryoglobulins, human immunodeficiency virus and hepatitis panel serologies, IgA levels, and serum protein electrophoresis, all of which were unremarkable. The patient went into shock and died, despite aggressive RBC transfusion
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