ABSTRACT
INTRODUCTION: Churg-Strauss syndrome (CSS) is characterized by asthma, hypereosinophilia, and vasculitis involving at least two extrapulmonary organs. CASE: We report a case of a patient with antineutrophilic cytoplasmic antibody-negative CSS who developed pulmonary interstitial fibrosis (PIF). DISCUSSION: The possible relations between CSS and PIF are discussed. Because this case report is the first to describe features of pulmonary fibrosis in a patient with CSS, we cannot know whether this association is causal or fortuitous.
Subject(s)
Churg-Strauss Syndrome/complications , Pulmonary Fibrosis/complications , Aged , C-Reactive Protein/analysis , Colon/diagnostic imaging , Female , Humans , Immunoglobulin E/blood , Lung/diagnostic imaging , Neutrophils/metabolism , RadiographyABSTRACT
BACKGROUND: Since testing for antineutrophil cytoplasmic antibodies (ANCA) became available for routine evaluation, no large homogeneous cohort of patients with the Churg-Strauss syndrome has been studied. OBJECTIVE: To define the clinical and biological characteristics of newly diagnosed Churg-Strauss syndrome, according to the presence or absence of ANCA. DESIGN: Cross-sectional analysis of manifestations of participants who were enrolled in treatment trials between December 1995 and December 2002. SETTING: Multicenter study in 63 clinical centers in France, Belgium, Latvia, and the United Kingdom, coordinated by the French Vasculitis Study Group. PARTICIPANTS: 112 patients with Churg-Strauss syndrome that was recently diagnosed on the basis of current classifications. MEASUREMENTS: The authors compared principal demographic, clinical, and laboratory features according to ANCA status at diagnosis. RESULTS: The authors detected ANCA in 43 (38%) patients. Positive ANCA status at diagnosis was associated with renal involvement, peripheral neuropathy, and biopsy-proven vasculitis, whereas negative ANCA status was associated with heart disease and fever. LIMITATIONS: The authors assessed ANCA by immunofluorescence, but they did not assess ANCA centrally or systematically retest if ANCA was undetected at diagnosis. CONCLUSIONS: Phenotypically, ANCA-positive and ANCA-negative Churg-Strauss syndrome might differ. The association of ANCA positivity with clinical symptoms that indicate inflammation and necrosis of small vessels might characterize a predominantly vasculitic pattern of the Churg-Strauss syndrome.
