Lung sarcoidosis: Clinical features and therapeutic issues

作者：Uzunhan Y; Jeny F; Crockett F; Piver D; Kambouchner M; Valeyre D; Nunes H

来源：Revue de Medecine Interne, 2016, 37(9): 594-607.

DOI：10.1016/j.revmed.2016.01.010

• Sarcoidosis
• Lung
• Severe forms
• Chronic disease
• Treatment
• Biotherapy

Sarcoidosis is a granulomatous disease of unknown cause. This proteiform disease is characterized by an almost constant and often predominant lung involvement. The natural history of disease is difficult to predict at presentation. Diagnosis is based on a compatible clinical and radiological presentation and evidence of non-caseating granulomas. Exclusion of alternative diseases is also required according to clinical presentation. Biopsy samples of superficial lesions should be considered before other sites like per-endoscopic bronchial biopsies or endobronchial ultrasound-guided transbronchial needle aspiration. Therapeutic strategy for lung disease has to take into account the possible spontaneous resolution observed in newly diagnosed patients. Corticosteroids are the first choice when a treatment is decided, which concerns half of patients. Second and third line therapy are based respectively on immunosuppressive drugs and anti-TNF alpha drugs. Sarcoidosis mortality and morbidity are mainly linked to advanced pulmonary sarcoidosis lung fibrosis, pulmonary hypertension, bronchial stenosis and chronic pulmonary aspergillosis. "Non anti-inflammatory" treatments have to be considered as well. Clinicians have an essential role in treatment indication, end-point targets and evaluation of response to treatment during follow-up and in finding the best benefice to risk balance. Progress made on pharmacogenetics may offer more personalized treatments for the patients.

• 出版日期2016-9