Systemic Manifestations of CSS and Diagnosis

Mean age at the time of CSS diagnosis is 48 years, with a sex ratio of approximately 1 1. Most patients have general symptoms, like fever or weight loss, but pulmonary manifestations are core disease features, particularly asthma and patchy pulmonary infiltrates (Fig. 1) (about 100% and 70% patients, respectively) (2, 16,21-25). Airways involvement, including ear, nose, and 

Neurological involvement is noted in 50% to 78% of patients (26,27). Mononeuritis multiplex is the most frequent ( 70% patients with peripheral neuropathy) at CSS onset whereas symmetrical polyneuropathy can be seen in up to 30% of patients (26,27). Central nervous system (CNS) involvement (cranial nerves palsies, cerebral hemorrhage or infarction, convulsions, coma, and psychiatric features) is rarely seen (16). 

Heart involvement develops in up to 60% of CSS patients (1). In the French Vasculitis Study Group (FVSG) experience, heart disease was found in 39 of 112 (35%) patients, including 28 patients with pericarditis and 27 with cardiomyopathy (3). Heart disease can involve the myocardium, pericardium, and at lesser degree, endocardium (28) and, in earlier studies, represented the major cause of death, accounting for about 48% of deaths (28,29) and morbidity. Myocarditis (caused by different or perhaps associated mechanisms, including eosinophilia, coronary arteritis, and fibrosis) can lead to restrictive (29), congestive (30), or dilated cardiomyopathy (30,31). Pericardial effusion occurs in up to 22% of CSS patients. CS usually control pericarditis but relapses may occur. 

Endomyocardial involvement was also reported (29,30). Conduction disorders and supraventricular arrhythmias, which can be fatal, can respond to CS. New imaging methods to detect heart disease are under development, including cardiac magnetic resonance and gated single photon-emission computed tomography (SPECT) that provide functional information (32,33). 

Skin lesions develop in 40% to 75% of patients. Palpable (often necrotic) purpura, urticaria, and cutaneous nodules or papules on the limbs or fingers may be observed (34). 

---