6 Patterns > Fibrosis Pattern > Additional Features > w/ Variable Involvement > Other Variable Fibrosis Diseases

Other Variable Fibrosis Diseases

A number of other diseases (less common than UIP, CrHP, and CTD) may present with variable amounts of fibrosis and may mimic a UIP-pattern at first glance.  These include Sarcoidosis/Beryllosis, IgG4 Disease, Erdheim-Chester Disease, Hermansky-Pudlak Syndrome, Pleuroparenchymal fibroelastosis, and the Fibrotic (late) Phase of Pulmonary Langerhans Cell Histiocytosis.

The secrete is to be able to identify the particular histologic features that point to the specific etiology.  Explore these more uncommon diseases below.  Each image is a link to additional details and sample signouts.   


Sarcoidosis/BeryllosisAdvanced pulmonary fibrosis with architectural distortion of the lung


IgG4 Disease 


Erdheim-Chester Disease

Hermansky-Pudlak Syndrome


Pleuroparenchymal fibroelastosis


Fibrotic (late) Phase of Pulmonary Langerhans Cell Histiocytosis