Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue, and antineutrophil cytoplasmic antibodies (ANCA) against neutrophil granulocytes. Two pathological subsets of<unk>  are differentiated by the presence of antineutrophil cytoplasmic antibodies (ANCA), autoantibodies that mistakenly target and attack specific proteins found within the cytoplasm of neutrophils. The ANCA+ subtype is characterized by predominantly vasculitis-like manifestations, while the ANCA- subtype is more commonly associated with eosinophilic-related symptoms. Approximately 30-40% of<unk>  cases are ANCA+. While the pathogenic mechanisms are not fully understood, this finding suggests a role of B cells – the precursors of ANCA-producing plasma cells – in the pathogenesis of<unk> . The American College of Rheumatology 1990 criteria for diagnosis of Churg–Strauss syndrome lists these criteria: * Asthma * Eosinophilia, i.e. eosinophil blood count greater than 500/microliter, or hypereosinophilia, i.e. eosinophil blood count greater than 1,500/microliter * Presence of mononeuropathy or polyneuropathy * Unfixed pulmonary infiltrates * Presence of paranasal sinus abnormalities * Histological evidence of extravascular eosinophils For classification purposes, a patient shall be said to have<unk>  if at least four of these six criteria are positive. The presence of any four or more of the six criteria yields a sensitivity of 85% and a specificity of 99.7%. Risk stratification The French Vasculitis Study Group has developed a five-point system ("five-factor score") that predicts the risk of death in Churg–Strauss syndrome using clinical presentations. These factors are: * Reduced renal function (creatinine >1.58 mg/dl or 140 μmol/l) * Proteinuria (>1 g/24h) * Gastrointestinal hemorrhage, infarction, or pancreatitis * Involvement of the central nervous system * Cardiomyopathy Having none of these factors indicates milder case, with a five-year mortality rate of 11.9%. The presence of one factor indicates severe disease, with a five-year mortality rate of 26%, and three or more indicate very severe disease: 46% five-year mortality rate. Imaging On CT scan of the lungs, peripheral parenchymal opacification (pulmonary consolidation or ground-glass opacity) in the middle and lower zones is the predominant pattern. Interlobular septal thickening (septa separating the secondary pulmonary lobules can be due to pulmonary odema secondary to heart failure.
Eosinophilic granulomatosis with polyangiitis
Eosinophilic esophagitis
Granulomatosis with polyangiitis
Microscopic polyangiitis
Nephrogenic systemic fibrosis
Thromboangiitis obliterans
IgA vasculitis
Systemic vasculitis