Immunoglobulin a Vasculitis in a Middle-Aged Male

Abstract

Introduction: Immunoglobulin A vasculitis (IgAV), formerly Henoch—Schönlein purpura vasculitis, is a vasculitis commonly seen in children but seldomly seen in young adults, and rarely described in older patients. Classic presentation is a palpable cutaneous purpura on the lower extremities and buttocks with concomitant arthralgia. Studies have shown half of patients will present with gastrointestinal symptoms typically abdominal pain from ileus, intussusception and ischemia. Ileitis has only rarely been described as a presentation of IgAV in adults and the majority of such cases were seen in young adults. Case: 56-year-old Caucasian male with a history of hypertension and diabetes mellitus developed worsening abdominal pain for one month. Prior to his symptoms he was having daily bowel movements with no associated pain or gastrointestinal bleeding. He developed symptoms of constipation with abdominal pain and was started on polyethylene glycol and initially improved. Subsequently he developed a bilateral lower extremity purpuric rash which was diagnosed as a leukocytoclastic vasculitis and treated with oral sulfamethoxazole /trimethoprim. His rash improved, however due to worsening abdominal pain he presented to the hospital for evaluation. Upon initial examination his vital signs were unremarkable. His physical exam revealed a soft non-tender abdomen with bowel sounds present, and a resolving purpuric rash on the posterior bilateral lower extremities. Initial blood-work revealed leukocytosis of 14.5 K/cmm and acute kidney injury (AKI) with a creatinine of 2.15 mg/dL. Computed tomography with intravenous contrast showed findings suggestive of ileitis. Evaluation for multiple myeloma, cryoglobulinemia, and ANCA vasculitis was negative. Initially his kidney function improved with rehydration and his abdominal pain resolved with aggressive bowel regimen. Colonoscopy showed terminal ileitis with biopsies compatible with a vasculitic process. Given these findings and recurrent AKI renal biopsy was performed which showed IgA glomerulonephritis. He was started on high dose steroids and subsequently his kidney function improved with complete resolution of abdominal pain. Discussion: Although, classically associated with children and young adults, IgAV can present in older patients. Common symptoms of rash and arthralgia may be absent initially, however, this does not exclude IgAV. Findings of terminal ileitis, while rare, could be a sign of a vasculitis.