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2016 ; 22
(5
): 484-91
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From granuloma to fibrosis: sarcoidosis associated pulmonary fibrosis
#MMPMID27379967
Bonham CA
; Strek ME
; Patterson KC
Curr Opin Pulm Med
2016[Sep]; 22
(5
): 484-91
PMID27379967
show ga
PURPOSE OF REVIEW: Up to twenty percent of patients with sarcoidosis develop
pulmonary fibrosis, transforming an often benign disease into a highly morbid and
potentially fatal one. We highlight the fibrotic pulmonary sarcoidosis phenotype
as an area of intense clinical and translational investigation, review recent
developments in treatment, and provide a roadmap for future research in
sarcoidosis associated pulmonary fibrosis. RECENT FINDINGS: Granulomatous
inflammation in a lymphatic distribution is the hallmark finding of pulmonary
sarcoidosis and the nidus for fibrosis. Recent research demonstrates that
fibrotic sarcoidosis begins in the setting of persistent, uncontrolled
inflammation, and is aided by pro-fibrotic genetic features and immune responses.
Comparison to other fibrotic lung diseases also reveals key features that inform
our understanding of common pathways in fibrosis. SUMMARY: Understanding the
mechanisms of fibrotic transformation in sarcoidosis enhances clinical care and
facilitates development of novel therapeutic options. The impact of these
findings in fibrotic sarcoidosis may be amplified through application to other
interstitial lung diseases marked by inflammatory to fibrotic transformation.
Important aspects of clinical management of fibrotic sarcoidosis include
surveillance for co-morbidities, such as pulmonary hypertension, airway disease,
and infection, and assessment for pulmonary disease activity that may benefit
from immunosuppression.