Diffuse Pulmonary Meningotheliomatosis: A Rare Lung Disease Presenting with Diffuse Ground-Glass Opacities and Cavitation

BACKGROUND Diffuse pulmonary meningotheliomatosis (DPM) is an exceedingly rare diffuse pulmonary disease with a female predominance. It is characterized by the presence of widespread bilateral minute pulmonary meningothelial-like nodules (MPMNs) on chest imaging. Patients are generally asymptomatic or may present with nonspecific symptoms such as dyspnea. The nodules are typically detected incidentally on imaging for other indications. Here, we present a rare case of DPM in a 55-year-old woman. CASE REPORT A 55-year-old woman presented to the clinic with non-exertional chest pressure and dry cough of 4-month duration. She had a history of hypertension, hypercholesterolemia, hypothyroidism, gastroesophageal reflux disease, and impaired fasting blood glucose and was a lifelong nonsmoker. Physical examination was unremarkable. High-resolution chest computed tomography (CT) showed innumerable diffuse small ground-glass nodules. An extensive laboratory workup was negative for autoimmune and infectious etiologies. The patient underwent uncomplicated right video-assisted thoracoscopic surgery, and lung biopsy showed multiple well-circumscribed interstitial meningothelial-like nodules in perivenular distribution with occasional whorling of cells. The diagnosis of diffuse pulmonary meningotheliomatosis (DPM) was confirmed. The patient continued to complain of non-exertional chest pressure without pulmonary complaints, and a repeat chest CT showed stable findings 1 year after the diagnosis. CONCLUSIONS DPM should be considered in the differential diagnosis for patients presenting with diffuse bilateral pulmonary nodules. Patients are typically asymptomatic and it is most commonly detected incidentally. Further research is needed to better understand this disease and its clinical significance.