1203: DIFFUSE ALVEOLAR HEMORRHAGE

Abstract

Cryoglobulins are immunoglobulins that reversibly precipitate at low temperature. Mixed cryoglobulinema (polyclonal) is seen in patients with chronic hepatitis C. Vasculitis of small and medium sized vessels is due to deposition of immune complexes in the vessel walls leading to palpable purpura, arthralgias, weakness, glomerulonephritis, peripheral neuropathy, skin ulcers and very rarely diffuse alveolar hemorrhage (DAH).Case: A 52-year- old woman with a history of chronic untreated hepatitis C, type II mixed cryoglobulinemia and glomerulonephritis was evaluated for abdominal pain and lower gastrointestinal bleeding. Upper endoscopy, sigmoidoscopy, capsule enteroscopy were unrevealing. Biopsy from the distal jejunum showed ischemic features. Patient developed chest pain, dyspnea, severe hypoxemia and hemoptysis necessitating mechanical ventilation. CT chest showed diffuse groundglass opacities in both lungs. Bronchoalveolar lavage demonstrated bloody return with 46% hemosiderin-laden macrophages. DAH was diagnosed. Compliment levels were low and cryoglobulin levels were positive. High dose intravenous steroids were initiated. Patient received 7 sessions of plasmapheresis and two doses of rituximab. She progressively improved and was discharged.

DAH and the ischemic changes in the intestine were the result of multisystem involvement by cryoglobulinemic vasculitis. DAH from cryoglobulinemia is an unusual entity with only a handful of cases described in the literature. Majority of the patients died immediately or due to a recurrence. There should be a high index of suspicion for this entity in patients with hepatitis C presenting with above clinical features. Treatment options include high dose steroids, plasmapheresis and more recently rituximab. Plasmapheresis removes the cryoglobulins. Rituximab depletes the B-cell clones that produce the cryoglobulins. Rituximab has an important role in patients with life-threatening vasculitis in patients who are unresponsive to conventional therapy or who cannot receive antiviral therapy. Our patient is stable on immunosuppression without recurrence. Hepatitis C could not be treated due to her psychiatric illness. Cryocrit does not correlate with disease activity. Aggressive treatment which includes Rituximab should be considered as the prognosis is poor due to high mortality at presentation.