The Churg—Strauss Syndrome

Abstract

The Churg—Strauss Syndrome (CSS) is a form of necrotizing vasculitis characterized by eosinophilic infiltration of small- and medium-sized blood vessels. The initial disease manifestation is usually the development of asthma in an individual who did not suffer previously from reactive airway disease. Allergic rhinitis is another manifestation of atopy, often identified in retrospect as the first sign of CSS. The extra-pulmonary organs commonly involved in the CSS are the peripheral nerves, skin, joints, and heart. The brain, eyes, gastrointestinal tract, and kidneys are involved less often. Vasculitic neuropathy occurs in 80% of patients with CSS and can lead to devastating complications through paralysis of the distal extremities. Nerve infarctions generally lead to an asymmetric, axonal sensorimotor neuropathy. The clinical features of CSS overlap in many ways on those of two other forms of necrotizing vasculitis, Wegener's granulomatosis, and microscopic poly-angiitis. However, patients with CSS are less likely to have antineutrophil cytoplasmic antibodies (ANCA) than those with other conditions. When patients with CSS are ANCA-positive, the antigen specificity is directed more often against myeloperoxidase than against serine proteinase-3. Anti-myeloperoxidase ANCA produces a perinuclear (“P-ANCA”) pattern of staining in immunofluorescence studies of serum. CSS must be distinguished from a group of non-vasc-ulitic conditions associated with hypereosinophilia. Among these are the hypereosinophil syndrome, eosinophilic leukemia, eosinophilic fasciitis, and parasitic infections.