Mixed Cryoglobulinemia Associated With Pulmonary Embolism and Small-Medium Vessel Vasculitis

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease SESSION TYPE: Case Report Slide PRESENTED ON: Saturday, April 16, 2016 at 09:45 AM - 11:15 AM INTRODUCTION: Cryoglobulinemia is an immune complex vasculitis that involves small to medium-sized blood vessels, leading to manifestations like purpura, arthralgia, fatigue, neuropathy, liver and renal involvement. We present a case of pulmonary embolism (PE) occurring in a patient with Cryoglobulinemia and other typical features of vasculitis. CASE PRESENTATION: A 77 year old male presented with gangrene, ulceration of his left foot and shortness of breath. A Chest CT angiogram which revealed bilateral PE’s with large clot burden. Physical examination showed multiple necrotic wounds in both the upper and lower extremities along with palpable purpura affecting all extremities. Labs showed a -ve ANCA but +ve ANA, high rheumatoid factor titer, low C3 and C4 levels and positive results for cryoglobulins with trace Cryocrit. Screen for both Hep B DNA and Hep C antibody are negative. Patient had a 15 year of history of rheumatoid arthritis and his previous medications including azathioprine, hydroxychloroquine, prednisone and aspirin. Patient was started on ACE- inhibitors due to proteinuria. An IVC filter was also placed due to a subdural hematoma a year before. Patient underwent 3 rounds of plasmapheresis along with the steroids, hydroxychloroquine and rituximab infusions. This lead to a marked improvement in his overall condition with improved dyspnea, oxygenation, skin ulcers, renal function, cryoglobulin levels and hematocrit. He was ultimately discharged after 23 days in the hospital with a near total recovery. DISCUSSION: Cryoglobinemia is typically associated with medium to small vessel vasculitis and PE is a rare occurrence in this disorder. PE likely occurred as a result of the procoagulant state and systemic inflammation from the underlying rheumatoid arthritis and associated cryoglobinemia. Treatment of the underlying condition (RA in our case) typically resolves cryoglobinemia related features. CONCLUSIONS: The systemic pro-inflammatory state occurring in the setting of type II and type III cryoglobinemia can rarely result in PE. Prompt treatment of the underlying cause is very important in the management of this condition. Our patient showed marked improvement after plasmapheresis and intravenous rituximab therapy. Reference #1: Roithinger, F. X., et al., A lethal course of chronic hepatitis C, glomerulonephritis, and pulmonary vasculitis unresponsive to interferon treatment. Am J Gastroenterol, 1995. 90(6): p. 1006-8. Reference #2: Ferraccioli, G. F., et al., Autoimmune connective tissue disease, chronic polyarthritides and B cell expansion: risks and perspectives with immunosuppressive drugs. Clin Exp Rheumatol, 1996. 14(14): p. S71-80. Reference #3: Ramos-Casals, M., et al., Mixed cryoglobulinemia: new concepts. Lupus, 2000. 9(2): p. 83-91. DISCLOSURE: The following authors have nothing to disclose: Bibek Pannu, Evan Nadler, Kumar Sarvottam, Vivek Iyer No Product/Research Disclosure Information