Síndrome de Churg-Strauss: casuística

Abstract

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.

Churg-Strauss syndrome that presented with mediastinal lymphadenopathy and calculous cholecystitis

“…These criteria include asthma, greater than 10% peripheral eosinophilia, mono- or polyneuropathy, migratory pulmonary infiltrates, sinus anomalies, and extravascular eosinophils in the biopsy. The presence of four of these criteria in a patient with biopsy-proven vasculitis has a sensitivity of 85% and a specificity of 99.7% for CSS [ 1 ]. Involvement of the lung, skin, gastrointestinal tract, and nervous system are common in CSS, but involvement of the mediastinal lymph node (LN) and gallbladder (GB) containing stones are less common.…”

“…High dose steroids are the mainstay of treatment and generally begin with a dose of 1 mg/kg/day (max 60 mg/day) of prednisolone, maintained until symptoms are controlled, and then slowly tapered for 1 year. In patients with factors which have a poor prognosis (e.g., serum creatinine > 140 mmol/L, proteinuria > 1 g/day, severe gastrointestinal tract involvement, cardiomyopathy, and/or CNS involvement) [ 5 ], the combination of steroids and cyclophosphamide produced better control and sustained remission rates, compared with steroids alone [ 1 ]. This case reported herein was without poor prognosis factors and hence initially had good response to steroid treatment alone, but then rapidly showed newly developed cholecystitis while steroids were tapered off.…”