Background: Pulmonary hypertension (PH) is a rare complication of sarcoidosis, although it is not uncommon in advanced disease. Methods: A retrospective series of 22 sarcoidosis patients (16 men) of mean (SD) age 46 (13) years with PH was divided into two groups depending on the absence (stage 0: n = 2, stage II: n = 4, stage III: n = 1) or presence (n = 15) of radiographic pulmonary fibrosis at the time of PH diagnosis. Results: In both groups PH was moderate to severe and there was no response to acute vasodilator challenge. In non-fibrotic cases no other cause of PH was found, suggesting a specific sarcoidosis vasculopathy, although no histological specimens were available. In cases with fibrosis there was no correlation between haemodynamics and lung volumes or arterial oxygen tensions, suggesting other mechanisms for PH in addition to pulmonary destruction and hypoxaemia. These included extrinsic arterial compression by lymphadenopathies in three cases and histologically proven pulmonary veno-occlusive disease in the five patients who underwent lung transplantation. Ten patients received high doses of oral prednisone for PH (stage 0: n = 1, stage II: n = 4 and stage IV: n = 5); three patients without pulmonary fibrosis experienced a sustained haemodynamic response. Survival of the overall population was poor (59% at 5 years). Mortality was associated with NYHA functional class IV but not with haemodynamic parameters or with lung function. Conclusion: Two very different phenotypes of sarcoidosis combined with PH are observed depending on the presence or absence of pulmonary fibrosis. PH is a severe complication of sarcoidosis.
Pulmonary CT allows good assessment of the evolution of LCH lesions. Nodular lesions probably represent active disease and often undergo regression or transform into cysts.
By comparing serial computed tomographic (CT) scans obtained when sarcoidosis was clinically active and after the onset of remission, an attempt was made to differentiate inflammatory from fibrotic lesions in the lungs of patients with sarcoidosis. Twenty patients with pulmonary infiltration seen on their chest radiographs were studied. For each patient, lesions found on the first CT scan were assessed by two observers as being decreased or increased on the second CT scan. Nodules (n = 8), irregularly marginated nodules (n = 5), and alveolar or pseudoalveolar consolidation (n = 5) always disappeared or clearly decreased. Septal lines (n = 10), nonseptal lines (n = 9), and lung distortion (n = 7) remained unchanged or increased. Some findings varied among patients: Micronodules (n = 9) and subpleural thickening (n = 5) disappeared or decreased in sarcoidosis of recent origin. Many findings of pulmonary infiltration seen on the first CT scan can be considered expressions of either inflammatory (reversible CT findings) or fibrotic (irreversible CT findings) lesions.
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