Evans Syndrome and Granulomatosis With Polyangiitis: An Interesting Concomitant Occurrence

Abstract

INTRODUCTION: Evans Syndrome and Wegener’s granulomatosis are rare autoimmune conditions, both having annual incidence of less than 1 in 200,000(1). Evans syndrome is a combination of autoimmune hemolytic anemia and immune thrombocytopenia, while Wegener’s granulomatosis is characterized by granulomatous vasculitis of the upper and lower respiratory tracts together with glomerulonephritis. CASE PRESENTATION: A 42-year-old Caucasian female admitted to our hospital with acute febrile illness with thrombocytopenia. On further evaluation, she was diagnosed with Idiopathic thrombocytopenic purpura (ITP). Subsequently, patient was treated with steroids with favorable response. Six weeks later, she presented with nausea, vomiting and jaundice. Her hemoglobin was 5.2 g/dl with positive direct Coombs test. Elevated LDH, low haptoglobulin and splenomegaly were indicative of hemolysis. Evans Syndrome was diagnosed with the presence of autoimmune hemolytic anemia in the setting of ITP. Patient had partial response to steroids, IVIG, vincristine and splenectomy. Remission was achieved with the addition of cyclophosphamide following splenectomy. Thirteen years later, she presented with painless hematuria of four days duration. She also complained of persistent dry cough with symptoms of sinusitis for years. She was also being treated for urticarial vasculitis during the last three years. Further evaluation revealed proteinuria and acute onset renal failure. A chest radiograph revealed bilateral pleural effusion with bibasilar infiltrates. She was strongly positive for antimyeloperoxidase antibody and renal biopsy revealed focal necrotizing glomerulitis with epithelial crescent formation. At this point, a diagnosis of Wegener’s granulomatosis was made(2). She was started on a cyclophosphamide along with prednisone, with induction of remission as indicated by an improvement in her renal function. Her symptoms of cough and sinusitis also improved with treatment. DISCUSSION: Wegener’s Granulomatosis is an uncommon disease characterized by granulomatous vasculitis of the respiratory tract with glomerulonephritis. Evans syndrome is an even more rare diagnosis, presenting with autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura. An association of autoimmune hemolytic anemia with Wegener’s granulomatosis has been reported once(3). CONCLUSIONS: To the best of our knowledge this is the first reported case of Wegener’s granulomatosis concomitantly occurring with Evans syndrome.