Isolated Jejunal Vasculitis in a Young Male

Vasculitis is the inflammation of blood vessel walls leading to organ ischemia. The gastrointestinal (GI) tract is a frequent site for systemic small and medium vessel vasculitis with isolated involvement being rare. We present a case of a young male with isolated jejunal vasculitis. A 21-year-old healthy white male had multiple ER visits in a 5 week period for intermittent sharp periumbilical pain with nausea and diarrhea. Proximal jejunal thickening was seen on CT scan. He was treated with antibiotics and opioids but developed non-bloody emesis and a tense abdomen with voluntary guarding and rebound. Labs were notable for WBCs of 15K and CRP 4.014 mg/dl (0.000-0.500 mg/dl). CT scan suggested findings consistent with jejunal intussusception. An urgent exploratory laparotomy resulted in resection of 35 cm of thickened jejunum. Biopsy confirmed small-to-medium vessel vasculitis with secondary ischemic changes and prominent eosinophils. Rheumatologic workup to include rheumatoid factor (RF), cryoglobulins, hepatitis B and C, ANA, lupus, and ANCA panels were negative. HIV was negative and urinalysis unremarkable. He had an elevated IgE level without peripheral eosinophilia. An angiogram confirmed no other areas of vasculitis. He was treated with high dose prednisone with a 4 week taper resulting in resolution of pain and normalization of CRP. However, shortly after cessation, abdominal pain and elevation in CRP returned with CT scan showing jejunal thickening proximal to the anastomotic site. Push enteroscopy demonstrated non-specific inflammation and was otherwise normal apart from the incidental discovery of eosinophilic esophagitis. Steroid taper attempts were unsuccessful and methotrexate and adalimumab were started. He is currently doing well on methotrexate and adalimumab 10 months after initial presentation with no evidence of systemic vasculitis. The GI tract is involved in up to 25% of systemic vasculitis syndromes but rarely occurs in isolation. It affects both genders equally and at a mean age of 50. Patients with isolated GI disease are more likely to present with an acute abdomen than those with systemic disease. Symptoms may remain in the isolated organ though some will go on to develop systemic disease with a positive ANA or RF seen in the latter. As in our patient, observational data suggests immunosuppressive therapy can successfully treat isolated small bowel vasculitis. Early diagnosis is difficult given the nonspecific presenting symptoms.