Pulmonary Manifestations of CSS

Asthma is quasi-constant (4) and can be severe and become CS dependent with continuous dyspnea, and severe attacks are common. In the vast majority of cases, asthma develops before systemic signs of vasculitis. The time between asthma onset and CSS may be very long (up to 30 years). For 96 of our patients (16), this delay was 8.86 10.86 years (range 0-61). The severity of asthma attacks usually increases before CSS onset. However, asthma appears after vasculitis (4) or simultaneously with it [about 20% of the patients described by 

Chumbley et al. (23)]. Vasculitis preceded asthma by 6 and 12 months, respectively, for two highly unusual patients (16). Only 1 of 23 CSS patients followed by Reid et al. (22) had no asthma, and this absence should always be interpreted as a possible argument against the diagnosis. In CSS, asthma may persist after treatment controls systemic vasculitis. About 75% of patients in long-term CSS remission have persistent asthma requiring low-dose prednisone and/or inhaled CS (16). 

This pattern, found in some patients (40,41), was described as diffusely scattered centrilobular nodules (5 mm) within ground glass opacities in eight of nine patients investigated by Choi et al (39). These lesions, located in centrilobular spaces, correspond to pulmonary vasculitis and perivascular infiltration. 

According to Lanham et al. (4) and Reid et al. (22), pleural effusions were present in 20% to 30% of their patients, but were less frequent in our patients (16). The pleural fluid is usually exudative and rich in eosinophils. Pleural biopsy might contain eosinophil infiltrates with necrotizing vasculitis (16) or 

In CSS, asthma, heart failure, pulmonary infiltrates, and alveolar hemorrhage may also cause dyspnea. Alveolar hemorrhage, which occurred in only 4% of our patients (16), resulted from lung capillary involvement, causing dyspnea, hemoptysis, anemia, and lung alveolar infiltrates. It is associated with the detection of anti-MPO Ab (2,3). Phrenic palsy, probably because of phrenic nerve vasculitis (36), and enlarged mediastinal lymph nodes are rarely observed (39). 

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