A Case of Cryoglobulinemic Vasculitis Causing Ischemic Enterocolitis

Abstract

30 year old female with systemic lupus erythematosus and Sjogren's syndrome overlap, leukocytoclastic vasculitis, marginal zone lymphoma and nephritis secondary to cryoglobulinemia treated with rituximab presents with severe bilateral lower quadrant abdominal pain, diarrhea, hematochezia, and hematemesis. On presentation she was tachycardic and her abdomen was tender to palpation without rebound or guarding. Initial labs were remarkable for leukocytosis, hemoconcentration and elevated ESR and CRP. CT abdomen pelvis showed diffuse bowel wall thickening most prominently in the duodenum but throughout the small bowel, ascending and transverse colon. She was given fluids, empiric antibiotics and steroids. Colonoscopy showed fresh blood in the terminal ileum but no sign of active bleeding. EGD showed duodenitis in D3/D4 with possible necrosis concerning for ischemic small bowel with likely microperforation. Laboratory evaluation revealed complement levels at baseline and dsDNA, anti-histone, myeloperoxidase antibody IgG, protease 3 IgG and beta-2 glycoprotein-1 antibodies were negative. Infectious work-up including HCV was negative. MRA did not show large vessel vasculitis. She was cryoglobulin positive with monoclonal IgM lambda and mixed Ig in the setting of high rheumatoid factor (>500). Pathology showed active duodenitis with erosion, fibrin thrombi and pseudomelanosis duodeni. These findings were suggestive of small bowel ischemia secondary to cryoglobulinemia vasculitis caused by underlying marginal zone lymphoma. Plasmapheresis was initiated with clinical improvement, and she was discharged home on steroids. Clinical manifestations from cryoglobulinemia occur due to hyperviscosity with vascular occlusion or formation and deposition of cryoglobulins, the latter of which is more common in mixed cryoglobulinemia. Vasculitis most commonly occurs in the skin, kidneys, joints and peripheral nervous system.1 Small bowel involvement is rarely reported.2 First-line therapy for severe or life threatening manifestations includes a combination of corticosteroids, immunosuppressants, plasmapheresis, and rituximab and treatment of any associated underlying infection.1 While small bowel vasculitis secondary to cryoglobulinemia is rare, recognizing this manifestation to allow early and appropriate treatment with steroids and immunosuppresants or plasmapheresis is essential.