Necrotising sarcoid granulomatosis: clinical, functional, endoscopical and radiographical evaluations.

Necrotising sarcoid granulomatosis (NSG) is a rare disease diagnosed on the basis of pathological features. The present study reports the characteristics of 14 cases of NSG. The mean age at the appearance of first symptoms was 37 yrs and the mean delay between first symptoms and diagnosis was 1 yr. Extrarespiratory symptoms were more common (12 out of 14) than respiratory symptoms (eight out of 14). Seven patients had inflammatory syndrome. Bronchoalveolar lavage was performed in eight patients and found to be normal in three cases. Respiratory function was normal in 13 patients, but carbon monoxide diffusing capacity was slightly decreased in eight of the 11 patients tested. A computed tomography scan showed a solitary nodule in four out of 14 cases, bilateral nodules in three and infiltrates in seven. One patient died from neurological complications despite treatment with corticosteroids and immunosuppressive drugs. Two cases of relapse were observed in patients initially treated with corticosteroids, and there were two cases of relapse after surgery. No relapse occurred in the five untreated patients. During the follow-up, lung cancer was detected at 26 months and 8 yrs, respectively, after NSG diagnosis in two patients. In conclusion, no one treatment is associated with a better outcome than the others, although lung biopsy might be necessary in case of isolated nodule or cavitation. Greater vigilance is required during the follow-up.