Teaching Case

Complaints of chest pain and non-productive cough. Symptoms had become apparent five weeks before. Personal history of the patient disclosed insulin-dependent diabetes. Clinical examination and extensive laboratory investigations, including renal function tests, were normal. Tuberculin reaction was negative.

The patient was admitted to the hospital with complaints of chest pain and non-productive cough. Symptoms had become apparent five weeks before. A general practitioner made the diagnosis of common cold. Personal history of the patient disclosed insulin-dependent diabetes. Clinical examination and extensive laboratory investigations, including renal function tests, were normal. Tuberculin reaction was negative. Chest radiography and CT scan of the lungs were performed. Based on the findings, a percutaneous needle aspiration of one of the pulmonary lesions was carried out under CT guidance. This procedure was repeated twice but yielded only a non-diagnostic, serosanguilent fluid. Finally, a video-thoracoscopic resection of a peripheral lesion was performed, followed by microscopic examination of the specimen.

Most patient with necrotizing sarcoid granulomatosis (NSG) are middle aged. A varying female predominance is seen. The disease usually presents with a specific symptoms, such as dry cough, chest pain and fatigue. NSG has to be differentiated from pulmonary sarcoidosis. Massive necrosis and vasculitis, which are typical findings in NSG, are very uncommon in sarcoidosis. Moreover, T- and B-cell activity, as well as the angiotensin converting enzyme levels in serum and tissue are normal in this disease. On chest radiographs, NSG is characterized by presence of a solitary, or more often, multiple pulmonary nodules. The latter image may be mistaken for malignancy. Pleural effusion and mediastinal lymphadenopathy are uncommon. Differential diagnosis of NSG includes pulmonary sarcoidosis, being the most important, and pulmonary tuberculosis, Wegener’s granulomatosis, and lymphomatoid granulomatosis. The final diagnosis of NSG is made histologically. Pathohistologic hallmarks of NSG are necroziting, angiocentric, confluent granulomas with epitheloid and giant cells, as well as focal destructive vasculitis of small to medium sized vessels. The prognosis of NSG is usually good, with mostly complete response after corticosteroid therapy.