New clinical practice guidelines have been issued for diagnosing lymphangioleiomyomatosis (LAM) and managing pneumothoraces in patients with LAM, reveals a new study.
The guidelines have been published by the American Thoracic Society (ATS) and the Japanese Respiratory Society (JRS). The latest guidelines supplement LAM guidelines that the two societies issued in 2016 for the diagnosis and management of the rare lung disease that primarily affects women of child-bearing age. The latest guidelines are published in the American Thoracic Society's American Journal of Respiratory and Critical Care Medicine.
LAM is a systemic disease that affects about five out of every million women. In patients with LAM, neoplastic smooth muscle-like cells arise from an unknown source, infiltrate the lung and result in cystic changes. Lung function declines at two to four times the normal rate, often punctuated by repeated lung collapses. Most patients are breathless with daily activities and require supplemental oxygen within 10 years of onset of symptoms.
The authors base this recommendation on the studies that have shown that the patient outcomes following lung transplantation in patients with prior pleurodesis were not substantially different than the patients who had not undergone prior pleurodesis.
Similar to these guidelines, the International Society for Heart and Lung Transplantation recently stated that pleurodesis should not be considered a contraindication to lung transplantation and that patients should be offered the best immediate care for pneumothorax. (This is a conditional recommendation with very low confidence in the estimated effects.)
"We continue to adjust our guidelines to keep pace with the evidence from clinical research," said Joel Moss, MD, PhD, co-chair of the guideline committee and deputy chief, Pulmonary Branch of the National Heart, Lung and Blood Institute.
"Patients are individuals and clinicians look at their specific cases when making treatment decisions. Their awareness of the guidelines and of the level of confidence in the potential effects of any course of treatment informs a doctor's decision making, without constraining it. That is the key benefit to patients."
Francis X. McCormack, MD, co-chair of the guideline committee and director of the Division of Pulmonary, Critical Care and Sleep Medicine at the University of Cincinnati, said, "Our hope is that these additional recommendations regarding diagnostic approach and pleural disease management can help protect patients from inappropriate drug exposures, unnecessary surgical procedures and recurrent pneumothoraces."
Nishant Gupta, MD, first author of the guidelines and assistant professor of pulmonary, critical care and sleep medicine at the University of Cincinnati, emphasized the need for more research. "The body of evidence the guidelines committee had to work with was small, and we identified several research directions that would greatly strengthen future recommendations," he said.
Among these were to: