.It is a chronic multiorgan inflammatory disorder of unknown etiology, characterized by non-caseating granulomas consisting of epitheliod mononuclear cells, giant cells and lymphocytes that primarily affect the lungs and the lymphatics.
.The disease can affect people of every race, sex and age à more in young African-American adults (more in females) between 20-40 yrs.
< 30 year old
> 30 year old
Spontaneous regression within 2 yrs
Still present after 2 yrs.
Bad. Lungs show extensive fibrosis ® Corpulmonale ® CHF.
· Grading (radiologically):
i. Stage 0: None
ii. Stage 1: Bilateral hilar (BHL) or mediastinal adenopathy (bilateral symmetrical, more in para-tracheal and less in peripheral LNs as cervical, supraclavicular, axillary, epitrochlear and right scalene)
iii. Stage 2: BHL with parenchymal infiltration (UL>LL)
iv. Stage 3: Parenchymal infiltration without BHL
v. Stage 4: Bullous, cystic and emphysematous changes (irreversible pulmonary fibrosis)
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