Diffuse pulmonary meningotheliomatosis: A literature review of a rare diffuse parenchymal lung disease with unclear clinical significance

Abstract

Introduction: Diffuse pulmonary meningotheliomatosis is a rare disease, with unclear clinical significance and very few reported cases in the literature. In this study, we review the demographics, presentation, imaging, diagnostic workup, and histologic findings of the 25 patients previously published in the literature with an outline of the disease history. Materials and Methods: We conducted a review of the literature through July 2016 for studies reporting cases of diffuse pulmonary meningotheliomatosis by searching multiple scholarly databases. Results: Of the 25 cases identified 2 were male (8%), and 23 were female (92%). Ages ranged from 37 to 73 with a median age of 59.5 years at diagnosis. 15 (60%) were asymptomatic and imaging abnormalities were discovered incidentally. 8 (32%) had unexplained respiratory complaints. 11 (44%) had history of or active malignancy. 3 (12%) were diagnosed by transbronchial biopsy while the remainder had surgical lung biopsies. Conclusion: Diffuse pulmonary meningotheliomatosis should be considered in all patients with diffuse bilateral pulmonary nodules on HRCT. The condition is more prevalent in females and its clinical significance is unclear, although nearly half of those diagnosed had a history of malignancy. CT imaging and surgical lung biopsy are the modalities of choice for diagnosis but transbronchial biopsies have recently been used obtain the diagnosis. Additional research needs to be done to further characterize the nature of this condition and the clinical scenarios in which is presents.

Keywords

• Diffuse parenchymal lung disease
• interstitial lung disease
• meningotheliomatosis
• micronodules
• miliary nodules
• transbronchial biopsy