CSS may evolve in phases. Asthma and other allergic symptoms usually precede other manifestations. In time, pulmonary infiltrates (Fig. 8) and peripheral hypereosinophilia may dominate the clinical picture, followed by the involvement of multiple organ systems. Convincing pictures of CSS may evolve several months to years after the onset of asthma. Occasionally, asthma can occur concurrently with other CSS features (30).
The most common sites of CSS involvement are the lungs, ENT organs, and nervous system (Table 2). Pulmonary manifestations include asthma, alveolar hemorrhage, infiltrates, and pleural effusions. Asthma is one of the major symptoms and is considered necessary for the diagnosis. It usually starts during adult age and may be associated with other allergic symptoms (e.g., rhinitis). The majority of patients have nervous system involvement, with peripheral neuropathy (PN) and CNS involvement occurring in 50% to 80% and 8%, respectively. Mononeuritis multiplex is the most common form of PN. CNS symptoms include stroke, meningeal symptoms, intracerebral hemorrhage, convulsions, and cognitive deficits.
Skin involvement is common, with purpura being the most frequent manifestation. Other skin lesions include papules, vesicles, ischemia, necrosis, livedo reticularis, and
Raynaud's phenomenon. Arthralgias and myalgias are frequent, but frank arthritis and myositis are uncommon. Gastrointestinal (GI) manifestations most frequently present as abdominal pain. The entire GI tract can be involved. Biopsies of affected sites may reveal vasculitis, granuloma formation, and/or inflammatory eosinophilic infiltrates. Cardiac disease may be severe and is the most frequent cause of death (30), occurring in approximately 35% (15-85%) of patients. Cardiac involvement most frequently includes heart failure, pericarditis, cardiomyopathy, and ischemic cardiomyopathy. Renal involvement is not as frequent in CSS as it is in WG and MPA. When present, renal disease is characterized by crescentic GN with scant or no immune-complex deposits.
ENT features are common in CSS. Sinusitis is one of the major manifestations and may precede the diagnosis in 60% of patients. Allergic rhinitis and nasal or sinus polyps are frequent (70%) (Fig. 9) (31). Although ENT features may be very similar to those of WG, they are usually milder and associated with less morbidity. It would be extremely unusual for CSS to cause a saddle nose deformity or SGS. Eye involvement in CSS includes episcleritis, scleritis, uveitis, retinal vasculitis, and conjunctivitis (32). Orbital pseudotumor would be very rare, and its presence should suggest WG.
Mild forms of CSS have been described, usually localized to one or few organ systems (32,33). Asthma may be absent, but allergic symptoms are frequent. Since the disease is rare, limited manifestations may not lead to suspicion of CSS.
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