Introduction: Most common presentation of pulmonary sarcoidosis is mediastinal and hilar lymphadenopathy. Cystic or cavitary changes are usually found in advanced stages. Sarcoidosis presenting with primary cavitary lesions is rare with a reported incidence of 0.6 to 8.3%. We present such a rare case of primary cavitary sarcoidosis. Case: 39 y/o African American male presented with a two year history of cough and worsening exertional dyspnea. He also reported an unintentional weight loss of 20 lbs within the last year. There were no associated fevers, chills or hemoptysis. He reported no travel beyond the northeast. He was a non-smoker.
- The Initial evaluation revealed an abnormal chest CT scan which showed extensive cavitary lesions bilaterally. T-spot test and serum Aspergillus IgG level were negative.
- PFT was abnormal showing severe obstructive lung disease (FEV1 1.31/FVC 2.06 and FEV1/FVC 64%) with severely reduced DLCO (36% of predicted) and air trapping.
- Bronchoscopy with RLL transbronchial biopsy and EBUS with FNA of the subcarinal lymph nodes showed non-caseating granulomata. BAL fluid cultures were negative for bacteria and fungi.
- He was started on systemic corticosteroids and supplemental oxygen therapy.
Discussion: In general cavitary lesions in pulmonary sarcoidosis are classified into three types. Type 1, as in this case, is characterized by truly well-defined lesions found in primary cavitary sarcoidosis and have minimal fibrosis. The pathophysiology of the type 1 lesions remains unclear due to its rare incidence. Autopsy results of the reported cases suggest the formation is secondary to the expulsion of hyalinized material related to ischemic necrosis from conglomerate sarcoid granulomas. Destruction of elastic lamina and intimal thickening of arteries and veins at the center of the granuloma has also been noted. Data also suggests necrosis related to TNF alpha release from alveolar macrophages and CD4 lymphocytes also contributes to cavity formation. Studies have also demonstrated that type 1 cavities are unpredictable and do not correlate with disease progression. However thickening of the cavities may correspond with secondary infections, most commonly Aspergilloma. Types 2 & 3 are found in emphysematous bullae and in advanced fibrotic stages respectively. They differ histopathologically from type1 in the amount of fibrosis. They are noted in the lung regions with significant parenchymal fibrosis and structural distortion. We emphasize that primary cavitary sarcoidosis is rare, often missed or delayed and should be considered in the differential diagnosis in patients presenting with cavitary lesions without significant surrounding fibrosis.
Full conference title:
- ATS 2018