Sarcoidosis is a granulomatous disease of unknown cause with almost constant lung involvement and a natural history difficult to predict. Diagnosis is based on triple context with an evocative clinical and radiological presentation, the demonstration of non-caseating and typical granulomas by biopsy samples of superficial lesions, perendoscopic bronchial biopsies or endobronchial ultrasound-guided transbronchial needle aspiration and the exclusion of other causes of granulomatosis. Corticotherapy is the first-line of treatment and is indicated in almost half of patients. Second – and third-line of treatments are respectively cytotoxic agents and TNF-α blockers. Severe pulmonary sarcoidosis including fibrosis, pulmonary hypertension, bronchial stenosis and aspergillosis are the leading causes of morbidity and mortality. The challenge in pulmonary involvement management is especially not to miss an alternative diagnosis, to know when and how to treat patients and to better target those at risk of developing severe forms.
Read full abstract