A gut feeling about butyrate

Abstract

LEARNING OBJECTIVE 1: Recognize common signs, symptoms, and presentation of Henoch-Schonlein Purpura LEARNING OBJECTIVE 2: Diagnose Henoch-Schonlein Purpura with gastrointestinal involvement CASE: A 62 year old woman presented with 5 days of severe abdominal pain, joint pain, and rash. Abdominal pain was band-like, radiated across her upper abdomen and back, and associated with bilious emesis, but no hematemesis, melena or hematochezia. She has started no new medications except for Ibuprofen for her new joint pains. Her epigastrium was mildly tender, but without any peritoneal signs. She had a scattered, flat, erythematous, non-blanchable, macular rash involving her bilateral lower extremities, concentrated on the shins and plantar aspects of her feet. Additionally, she had tender swelling of her bilateral 2nd, 3rd and 4th metacarpophalangeal joints and wrists. Initial lab work revealed normal platelet, and white blood cell count, creatinine, urinalysis, lipase and transaminases. C Reactive Protein was mildly elevated at 8 mg/dL, Erythrocyte Sedimentation Rate was 5 mm/hr. Abdominal CT revealed diffuse wall thickening and mucosal enhancements of the second and third portions of the duodenum. Esophagogastroduodenoscopy, demonstrated severe duodenitis with evidence of severe bowel edema, ulcerations, and submucosal hemorrhage in the second and third part of the duodenum, with biopsy showing severe neutrophilic inflammation with erosion and hemorrhage. Within 48 h of presentation, her rash advanced to a raised, purpuric quality. Skin biopsy revealed neutrophilic inflammation involving adjacent blood vessels. Direct immunofluorescence revealed IgA and fibrinogen deposition in the walls of superficial dermal vessels, consistent with Henoch-Schonlein Purpura (HSP). Initiation of high dose corticosteroids led to gradual improvement of her rash, arthralgias and abdominal pain. She was discharged with a steroid taper and follow-up 3 months off steroids revealed resolution of all symptoms and normal kidney function. DISCUSSION: The combination of abdominal pain, joint pain, and rash are commonly seen in general internal medicine. Vasculitis is an uncommon cause of the above symptoms, and HSP, while primarily a disease of children, is often more severe in adults, particularly with renal involvement. HSP vasculitis is an inflammatory process involving IgAmediated immune complex deposition into vessel walls, which activates the complement system and promotes neutrophilic infiltration into blood vessels. Diagnosis, as it did in this case, is often times confirmed by tissue biopsy. This patient had a common presentation of the disease; however, it was striking that she first developed vasculitis in her bowel-a harbinger for later findings on her skin. Treatment regarding HSP can be controversial, however, it is widely held that immunosuppression with corticosteroids is key, especially in adults with evidence of renal compromise. This case illustrates a common presentation of adult HSP, but underscores the severity of possible gastrointestinal involvement, as well as the importance of early recognition to minimize complications and initiated early treatment.