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2017 ; 9
(3
): e1088
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English Wikipedia
Henoch-Schönlein Purpura Presenting as Severe Gastrointestinal and Renal
Involvement with Mixed Outcomes in an Adult Patient
#MMPMID28405538
Shah R
; Ramakrishnan M
; Vollmar A
; Harrell A
; Van Trump R
; Masoud A
Cureus
2017[Mar]; 9
(3
): e1088
PMID28405538
show ga
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.