Combined Sarcoidosis and Idiopathic Pulmonary Fibrosis (CSIPF): Coincidence, Association or Mimicry?
Journal Paper/Review - Feb 21, 2022
Kluckert Jörg-Thomas, Brutsche Martin
A 70-year old patient with longstanding history of sarcoidosis (initially ATS- stage II), presented
with progressive dyspnea and dry cough. Although corticosteroid therapy initially improved lung
function, a decline (Figure 1) was observed. Thoracic CT-scan was compatible with definite unusual
interstitial pneumonia potentially engrafted on the preexisting sarcoidosis. After multidisciplinary
discussion antifibrotic therapy with Nintedanib was started. Lung function and fibrosis on the
CT-scan improved (Figure 1, 2). Collins et al.  found a series of cases and coined the term
“Combined Sarcoidosis and Idiopathic Pulmonary Fibrosis CSIPF”. IPF is defined as fibrosis
without inflammation and sarcoidosis as a multisystem chronic inflammatory condition – these supposedly being two distinct entities [2,3]. Did we experience
coincidence, association or mimicry between these two entities?
Lucky enough antifibrotic treatments is also licensed for progressive
non-IPF fibrotic lung diseases.