The relationship between statin medication and improvement in symptoms of rare lung disease

Rare Pulmonary Alveolar Proteolytic Disease
Rare Pulmonary Alveolar Proteolytic Disease


Researchers have found that statins can improve conditions in people with a rare lung disease called autoimmune pulmonary alveolar proteinosis. The research also suggested that two new tests could help diagnose the condition.


***Rare Pulmonary Alveolar Proteolytic Disease.


In the first study of its kind, researchers found that cholesterol-lowering statins improved the condition of people with a rare lung disease called pulmonary alveolar proteinuria, and the research also suggested that two new tests could help diagnose the condition, and alveolar proteinuria is a rare syndrome. It affects 1 out of every 150,000 people around the world, and the condition appears due to the abnormal accumulation of a fatty substance, consisting of cholesterol, phospholipids and proteins in the air sacs, or alveoli in the lungs.


The accumulation occurs because cells called alveolar macrophages, which normally help remove this substance, do not function properly, and when the alveoli are blocked, patients suffer shortness of breath and the possibility of respiratory failure, and at present the only treatment for the disease is “lung lavage.” Entirely”, a surgical procedure performed under the influence of anesthesia, which removes the accumulation of material from the lungs, and statins are a class of drugs that lower blood cholesterol, and can reduce the risk of heart attack and stroke, a substance that researchers have discovered can be effective in improving the severity of this disease.


*** The relationship between cholesterol drugs and rare lung disease.


The main observations that began in this study were serendipitous, as the researchers noted that a female patient with a rare pulmonary alveolar proteinuria disease responded poorly to the entire lung lavage, but improved significantly with oral statin therapy, and after six months of starting statin therapy for a high rate of In the blood cholesterol, the 58-year-old patient's breathing improved unexpectedly, and she no longer required supplemental oxygen therapy. Then, the researchers identified another patient, a 66-year-old woman, whose LAP also improved after treatment with an oral statin.


After the researchers verified clinical improvements in these women, the researchers took cells derived from a group of people with PAP and exposed the cells directly to statins. Next, the researchers were able to demonstrate that statin treatment improves markers of pulmonary alveolar protons in mice.


*** Using technology to discover lung diseases.


The study also highlights the potential use of two new measures to diagnose alveolar proteins, one of which is a computer program that analyzes computed tomographic scans of the lungs and, in doing so, improved assessment of disease severity, and a second method is to measure cholesterol levels in fluids, obtained during lung dialysis treatment. In conjunction with an endoscopic evaluation, a technique that could replace the need for a more serious lung biopsy.


The study did not determine the optimal dose of statin therapy, or the time required to reach maximum improvement, and the next steps will be to search for specific mechanisms of action, how statins affect disease, and the potential role of statin therapy for endogenous pulmonary alveolar proteins. Clinical trials will assess the relative safety and potential efficacy of statins in patients, and this study suggests that oral statin therapy, may be a new approach for patients with this disease.


Co-authors of the study were Dr. Elizabeth Tarling of the University of California, and Dr. Bruce Ternell of the Children's Hospital Medical Center in Cincinnati, Ohio. The study was published in the journal Nature Communications, and was funded by the National Institutes of Health.


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