Non-cystic fibrosis (CF) bronchiectasis may develop as the primary manifestation of endobronchial sarcoidosis, a disease that affects the lungs’ bronchi (the tiny sacs that make up the lungs, where respiratory gases are exchanged), according to a case study presented at the American College of Chest Physicians (CHEST 2016) Oct. 22-26 in Los Angeles.
The study, “Diffuse Bronchiectasis As The Primary Manifestation Of Endobronchial Sarcoidosis,” was conducted by a research team led by Jean Coviello, MD, from the San Antonio Military Medical Center in Texas.
Sarcoidosis is a multi-organ disorder of unknown cause that is characterized by the appearance of non-caseating granulomas – collections of immune system cells that form in a variety of organs, impairing their function. The symptoms of sarcoidosis are usually nonspecific and generalized, including fever, fatigue, anorexia, weight loss, and malaise. Symptoms vary depending on the organs affected.
Given that sarcoidosis mostly infiltrates the lungs, patients often exhibit respiratory symptoms. The disease can also affect the lungs’ bronchi, as this case study showed.
Researchers report the case of a 50-year-old woman who had been referred for bronchiectasis. Doctors carried out a series of different analyses to exclude the possibility of infections and other pathologies. They then found that her airway had endobronchial cobblestoning (nodules), suggestive of sarcoidosis.
They decided to take endobronchial biopsies as well as samples of BAL fluid (the fluid present in the bronchi) to analyze the presence of immune system cells. The fluid had a ratio between immune cells expressing CD4:CD8 of 8.9, and biopsies showed non-caseating granulomas, which led doctors to diagnose the patient with sarcoidosis.
“Non-caseating granulomas are the hallmark of sarcoidosis and have been found on endobronchial biopsy in over 60% of patients,” the authors wrote. “Biopsies are more likely to be positive with an abnormal airway mucosa, but still reveal granulomas in 30% of normal airways. The pathogenesis of bronchiectasis in sarcoidosis is almost exclusively traction bronchiectasis.”
The authors believe that the presence of granulomas in the bronchi (endobronchial sarcoidosis), as well as the inflammation and edema, led to the development of bronchiectasis in the patient.
The results of this case study led the team to conclude that bronchoscopy with endobronchial biopsy should be considered as part of the diagnostic evaluation to assess endobronchial sarcoidosis.