Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia and asthma. This is a rare syndrome of unknown etiology, affecting both genders and all age groups. CSS patients usually respond well to steroid treatment, although relapses are common after it ends. Timely diagnosis and treatment generally lead to a good prognosis with a 90% survival rate at one year. A brief review of CSS is presented, with particular attention to diagnosis, therapy and recent developments in this area. The authors then report and discuss the clinical, laboratory and imaging characteristics of four patients admitted to an Internal Medicine Department with this diagnosis. The treatment, response and follow-up of the cases are also described.
A 45 year old white man presented to the emergency department with progressively worsening weakness and chest discomfort on moderate intensity physical activity over the past two months. He also reported paleness and "yellowish eyes" in the past month. He had a history of anxiety, treated with alprazolam as needed.At presentation, his vital signs were normal. On physical examination he had mild generalised pallor, scleral icterus, and tender non-painful hepatomegaly.He was admitted to the internal medicine ward for further evaluation.Laboratory studies showed haemoglobin 70 g/L (reference range 13-17), mean corpuscular volume 111.5 fL (83-101), haematocrit 19.4% (40-50%), leucocyte count 3. Antibodies against parietal cells were positive; Coombs test and anti-intrinsic factor antibodies were negative.Upper gastrointestinal endoscopy showed atrophic gastritis of the body and fundus. Staining for Helicobacter pylori was negative (fig 1). Biopsy confirmed the presence of type A atrophic gastritis with moderate intestinal metaplasia.
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