Abstract
Henoch-Schönlein purpura (HSP) is typically seen as a self-limiting disease in children, but can present more severely in adults, especially when there is renal involvement. Management of HSP in adults also remains a controversial topic with very few studies evaluating available therapies. In this case, HSP presenting as a combination of severe gastrointestinal involvement and a rapid decline in renal function in an adult patient directed our therapy.The patient was a 48-year-old Caucasian male with no known past medical history, who presented with a combination of purpuric rash over the lower extremities, severe abdominal pain with upper gastrointestinal bleeding and a rapidly increasing serum creatinine, with hematuria. He initially underwent a skin biopsy, along with investigation for other possible causes, including autoimmune and infectious etiologies, which were negative. He was started on therapy for presumed HSP with intravenous methylprednisolone. The skin biopsy, however, was not conclusive, and the patient had no improvement in his clinical status. He then underwent a kidney biopsy that was consistent with HSP nephritis (immunoglobulin A (IgA) predominant glomerulonephritis with crescents), and esophagogastroduodenoscopy (EGD) that showed mucosal inflammation, ulcerations, and stigmata of bleeding—findings that were consistent with ischemia. Cyclophosphamide was added to the regimen at this time. However, he had worsening abdominal pain, continued gastrointestinal bleeding, now with hematochezia, and also worsening renal function that required dialysis. Plasmapheresis was then initiated on days alternating with dialysis. This resulted in the improvement of his gastrointestinal symptoms, but no recovery was seen of his renal function, and the patient required outpatient dialysis.This case report exhibits the unique presentation of severe gastrointestinal (GI) manifestations and rapid progression to renal failure in an adult patient with partial resolution of his severe manifestation after therapy was escalated as above. There was no established protocol that guided this therapy, which reflects the need for more studies on adult HSP.
Highlights
Henoch-Schönlein purpura (HSP), commonly called immunoglobulin A (IgA) vasculitis, is a small vessel vasculitis, that usually presents with the classic symptoms of purpura, arthritis/arthralgia, abdominal pain, and renal disease [1]
Very little is known about the management in adults, as there are very few studies evaluating the efficacy of the different treatment options, including steroids, immunosuppressive drugs, and plasmapheresis
We review a case of HSP presenting with severe gastrointestinal and renal involvement in a 48-year-old Caucasian male with no previous medical history, with mixed outcomes associated with the aggressive therapy we utilized
Summary
Henoch-Schönlein purpura (HSP), commonly called immunoglobulin A (IgA) vasculitis, is a small vessel vasculitis, that usually presents with the classic symptoms of purpura, arthritis/arthralgia, abdominal pain, and renal disease [1]. The patient underwent workup for other possible vasculitis and glomerulonephritis, with labs including antinuclear antibody (ANA), perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA), cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), anti-glomerular basement membrane (anti-GBM), complement levels, cryoglobulins, human immunodeficiency virus (HIV), hepatitis panel, IgA levels, and serum protein electrophoresis, all of which were within normal limits His hemoglobin, which was 15.5 g/dL on presentation, trended down to 11 g/dL over two days. With no improvement of gastrointestinal or renal manifestations and no definitive diagnosis yet, he was sent for a renal biopsy and was started on hemodialysis three times a week He underwent an esophagogastroduodenoscopy that showed Grade B esophagitis with > 5 mm long mucosal break in the lower esophagus and severe patchy inflammation, erythema, edema, 2017 Shah et al Cureus 9(3): e1088. His renal function showed no sign of recovery, and the patient was discharged after a total of eight sessions of plasmapheresis on an outpatient hemodialysis regimen
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