Purpose of review: To examine recent advancements in the clinical characteristics, treatment and prognosis of diffuse parenchymal disease and pulmonary hypertension due to idiopathic pulmonary fibrosis, scleroderma and sarcoidosis.
Recent findings: Bosentan can be used in delaying the time to progression of disease in biopsy-proven idiopathic pulmonary fibrosis patients. There are other clinical trials for the utility of pharmaceutical control of lung fibrosis. The use of cyclophosphamide in scleroderma-associated lung fibrosis has a benefit up to 1 year but not so afterwards. Despite many case reports showing benefit, infliximab does not appear to be effective in randomized-controlled trials for the use of pulmonary sarcoidosis. The presence of pulmonary hypertension appears to be an independent risk factor for death. Unfortunately, not all pulmonary hypertension-specific agents are effective in secondary pulmonary hypertension.
Summary: Lung fibrosis by any cause can be a debilitating disease. Not all current medical options are equally effective under randomized-controlled trials. Further studies with novel therapies are needed to control symptoms. The compounding presence of pulmonary hypertension will further limit the patient activity, quality of life and survival. As it has been reviewed, not all pulmonary hypertension-specific agents effectively work in these three subset groups.