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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.)

Diffuse interstitial lung diseases (DILD) cover more than 200 conditions classified in 4 groups: secondary DILD; sarcoidosis; particular DILDs and idiopathic interstitial pneumonias. Overall, these diseases have a prevalence of 60-80/100,000 and an incidence around 30/100,000. Sarcoidosis and idiopathic pulmonary fibrosis are the 2 most frequent diseases, accounting together for more than 50% of all cases, followed by DILD related to connective tissue disease and to immunologic lung diseases. The incidence of different causes of DILD depends on epidemiologic factors: age, sex, race, smoking habits, and history. Mortality is very elevated in idiopathic pulmonary fibrosis, linked in more than 80% of cases to the fibrosis itself, with a median survival between 24 and 36 months. The epidemiologic studies on DILD have substantial limitations, and new studies must be conducted, in particular in France where we lack epidemiologic data beyond one old study of sarcoidosis and one more recent study focused on farmer's lung in the Doubs., (Copyright 2009 Elsevier Masson SAS. All rights reserved.)

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.)

Subacute and chronic diffuse interstitial lung diseases Computed tomography (CT) plays an important role in all stages of management: positive diagnosis, etiological diagnosis, evaluation of lesions, ongoing monitoring, screening for complications, and prognosis. The etiological diagnosis is based on the imaging and analysis of patterns or groups of basic lesions often characteristics of a disease. Assessment of the images, the patient history, and the epidemiologic, clinical, laboratory, functional and cytologic data generally make it possible to reach a diagnosis. A pulmonary biopsy is rarely necessary. Acute diffuse interstitial lung diseases In the absence of an obvious clinical direction, CT, electrocardiography, and echocardiography are the first-line examinations to identify or rule out cardiogenic edema. CT can be used to guide bronchoalveolar lavage (BAL), widely used when the patient's respiratory condition permits. BAL can provide a diagnosis of diverse infections or help determine the cytologic type of alveolitis. CT also makes it possible to evaluate the lesions and plays a role in assessing severity. It makes it possible to choose the best sampling method and in principle directs sampling towards the most useful areas. It allows monitoring of disease course, screening of some complications, and precise localizing of tubes, drains, and catheters. Finally, it is used to assess the sequelae., (Copyright 2009 Elsevier Masson SAS. All rights reserved.)