DIFFUSE BRONCHIOLECTASIS WITH MUSCULAR HYPERPLASIA ("MUSCULAR CIRRHOSIS OF THE LUNG"): RELATIONSHIP TO CHRONIC FORM OF HAMMAN-RICH SYNDROME

The literature on the rare lung disease, historically called "muscular cirrhosis of the lung" ("bronchiolar emphysema") and in this article "diffuse bronchiolectasis with muscular hyperplasia" as the preferred name, was reviewed and 3 new proven cases are reported. The literature on the acute and chronic forms of Hamman-Rich syndrome (diffuse interstitial fibrosis of the lungs) was also reviewed and all findings compared to each other and to our 3 new cases. We conclude that "diffuse bronchiolectasis with muscular hyperplasia" ("muscular cirrhosis of the lung") represents, in all likelihood, the advanced stage of the chronic form of the Hamman-Rich syndrome with variable amounts of smooth muscle hyperplasia.All the roentgenologic, clinical, laboratory, pathophysiologic, gross and micropathologic aspects were found essentially the same in both diseases.The diagnosis, the roentgenologic differential diagnosis and the similarity with "collagen disease" are discussed.