A Case of “Inflamed Vessels”

Abstract

Purpose: Ulcerative colitis (UC) is an inflammatory bowel disease (IBD), which can have many systemic complications. We present a case of UC with systemic vasculitis involvement of skin, lungs, and central nervous system. Methods: A case report of a patient seen in the outpatient Gastroenterology Clinic. Results: A 35 year old Caucasian man presented with non-blanching, non-pruritic, annular skin lesions, myalgia, and arthritis that preceded the onset of his intestinal illness. Complete blood cell count, electrolytes, and renal function tests were within normal limits. Inflammatory markers were elevated: C-reactive protein 65 mg/L and erythrocyte sedimentation rate 47 mm/hr. Rheumatoid factor, anti-DNA antibody, antinuclear antibody, immunoglobulins, peroxidase-antineutrophil cytoplasmic antibodies were negative. CT of the neck showed C4-C5 disc herniation. Electromyography studies revealed axonal injury consistent with brachial neuritis. Flexible sigmoidoscopy revealed diffuse hemorrhagic mucosa, with loss of vascular pattern, mild edema, and preservation of haustral architecture compatible with moderate UC. The patient was started on Salofalk 4 g/day with complete resolution of rectal bleeding, diarrhea, arthralgias and skin lesions within 48 hours. He then developed hemoptysis and dyspnea, hemoglobin 85 g/L, elevated peripheral eosinophil count 1.5 × 103/μl and a right middle lobe consolidation on chest radiograph. CT of the chest confirmed an intrapulmonary hemorrhage. The question of Salofalk-induced pulmonary and skin complications was raised; therefore, Salofalk was discontinued. A muscle biopsy revealed necrotizing small vessel vasculitis with eosinophilia. After more than one month off Salofalk, the skin lesions, myalgias and arthralgias recurred. The patient remained asymptomatic from intestinal symptoms. Treatment with oral prednisone 40 mg/day was commenced with resultant improvement of the arthritis, skin lesions and brachial neuritis. Over the next year, the patient tapered prednisone slowly, with recurrence of rectal bleeding and diarrhea at prednisone dose of 20 mg/day. Salofalk was slowly re-introduced; however, in view of corticosteroid dependence, Azathioprine was introduced. Conclusion: Vasculitis affecting multiple organ systems in a patient with UC suggests that IBD may be part of a larger inflammatory systemic illness as manifested by the extra-intestinal symptoms. The distinction between disease-related and drug-induced systemic symptoms in patients with IBD is a diagnostic challenge and complicates medical management. Early awareness of vasculitis as an extra-intestinal manifestation should be made, so that prompt treatment with a course of corticosteroid therapy may be initiated to prevent serious complications.