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Sarcoidosis is a systemic disease, with lung involvement in almost all cases. Abnormal chest radiography is usually a key step for considering diagnosis. Lung impact is investigated through imaging; pulmonary function; and, when required, 6-minute walk test, cardiopulmonary exercise testing, or right heart catheterization. There is usually a reduction of lung volumes, and forced vital capacity is the most accurate parameter to reflect the impact of pulmonary sarcoidosis with or without pulmonary infiltration at imaging. Various evolution patterns have been described. Increased risk of death is associated with advanced pulmonary fibrosis or cor pulmonale, particularly in African American patients., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Pulmonary hypertension is a challenging complication of sarcoidosis, which reported rates of prevalence largely depend on the advancement of pulmonary disease. About 6% of unselected sarcoidosis patients suffer from PH. Although destruction of the distal capillary bed and resultant hypoxemia are important, the mechanisms of sarcoidosis-PH are multifactorial, including specific vasculopathy, local increased vasoreactivity, extrinsic compression of pulmonary vessels and portal hypertension. As a result, a proportion of patients exhibit "out of proportion" PH, i.e. more severe than expected from functional impairment (mean PAP>35-40mmHg). The sarcoidosis vasculopathy prevails in the venous side, reflecting the spreading of granulomatous process, and can cause pulmonary veno-occlusive disease. The responsibility of left-heart dysfunction is probably underestimated by echocardiography. There is no validated screening algorithm for the detection of sarcoidosis-PH but recent studies have underlined the role of right heart catheterisation to exclude post-capillary PH. PH carries a poor prognosis in sarcoidosis patients, with a significantly increased morbidity and mortality. Management of sarcoidosis-PH mainly relies on supportive therapy (supplemental oxygen and diuretics as needed) and lung transplantation in otherwise eligible patients. Rare cases of sarcoidosis-PH with nonfibrotic pulmonary disease respond to corticosteroids. Data on the efficacy and safety of PAH agents are scarce and discrepant. Further controlled trials are warranted and should integrate the concept of disproportionate PH in their design., (Copyright © 2012. Published by Elsevier Masson SAS.)

Approximately 90% of patients with sarcoidosis have thoracic involvement, of the lungs or mediastinum or both, isolated in half of all cases and associated with one or several extrathoracic sites in the other half. Diagnosis is based on a compatible clinical and radiologic history, the demonstration of noncaseous granulomas, and the ruling out of other causes of granulomatosis. Histologic proof is not necessary in cases of Löfgren syndrome, or when documentation shows a sequence of progression from stage I to stage II, or when the radiography shows the typical latent stage I picture. The radiographic stage has major prognostic value. Pulmonary fibrosis is the principal cause of morbidity and mortality in patients with sarcoidosis, especially when pulmonary hypertension is also present. Approximately half of all patients recover spontaneously in less than 2 years, while the other half require a systemic treatment. The official indications for treatment are: symptomatic stage II-III disease with functional respiratory or progressive involvement, stage IV with signs of persistent activity, granulomatous bronchial involvement with obstructive ventilatory disorders or endobronchial stenosis and extrinsic bronchial compression by adenopathies. Systemic corticosteroid therapy remains the reference treatment for thoracic involvement in sarcoidosis., (Copyright 2009 Elsevier Masson SAS. All rights reserved.)