According to a story from Pulmonary Hypertension News, many patients with the rare disease sarcoidosis frequently experience lung complications. Included in these complications is pulmonary hypertension, a condition of elevated blood pressure in the lungs that affects at least five percent of patients. There are no specific treatments for sarcoidosis associated pulmonary hypertension, but a recent study found that some patients could see improvement using medications developed for other types of pulmonary hypertension.
Sarcoidosis is a rare disease which is characterized by the appearance of unusual aggregates of inflammatory cells. These aggregates are called granulomas. The disease can affect almost any part of the body, but it most commonly appears in the lungs, lymph nodes, and skin. Unfortunately, the precise cause of sarcoidosis remains a mystery. The prevailing theory is that it is the result of alterations to the immune response following exposure to a certain trigger, such as an infectious pathogen. Certain genetic mutations, affecting BTLN2 and possibly HLA-DR, are considered risk factors. Sarcoidosis is also associated with certain autoimmune diseases. Symptoms vary depending on the organs affected but may include weight loss, fatigue, bone pain, cough, wheezing, shortness of breath, chest pain, skin ulcers and lumps, and skin discoloration. Symptoms are mild in most cases and many patients can control their disease with ibuprofen. Other treatments for more serious disease include steroids, glucocorticoids, antimetabolites, and immunosuppressants. To learn more about sarcoidosis, click here.
About The Study
The study evaluated a total of 95 patients, most of whom were African American women. Endpoints for this study were the six minute walk distance test and N-terminal pro-brain natriuretic peptide (NT-proBNP), a biomarker for pulmonary hypertension. 70 percent of patients in the study had advanced, stage four sarcoidosis. 74 of the patients used therapies specifically to treat their pulmonary hypertension. The researchers found that in about 33 of these patients their NT-proBNP values improved at a median change of 51.2 percent. However, results from the six minute walk distance did not change significantly.
More study will be needed, but the researchers were able to conclude that a significant portion of patients saw some improvement in a critical biomarker after using pulmonary hypertension-specific treatments.
Check out the original study here.