Abstract
BackgroundAlthough usual interstitial pneumonia (UIP) appears to portend better survival when associated with connective tissue disease (CTD-UIP), little is known about the presenting clinical, radiologic, and pathologic features that differentiate pathologically confirmed UIP with CTD from idiopathic pulmonary fibrosis (IPF). In patients with atypical radiologic and clinical features, what specific findings predict underlying IPF vs. CTD-UIP diagnosis and their respective long term survival?MethodsA large retrospective cohort analysis was done of consecutive patients seen from 1995 through 2010 with biopsy confirmed UIP completed or reviewed at our institution. CTD-UIP was defined by independent rheumatology consultation with exclusion of all other secondary causes of lung fibrosis. Primary clinical data was collected and compared for IPF and CTD-UIP along with logistic regression performed for predictors of disease likelihood and Cox proportional hazards analysis for predictors of survival.ResultsSix hundred and twenty five patients were included in the study of which 89 had diagnosed CTD-UIP representing 7 disease entities. Survival was better among those with CTD-UIP except in UIP associated with rheumatoid arthritis, which had similar presenting features and survival to IPF. Predictors of underlying CTD included female gender, younger age, positive autoimmune serology, and inconsistent presenting radiologic findings. Only age and forced vital capacity corrected for a priori covariates were predictive of survival in CTD-UIP.ConclusionsUIP pathology occurs frequently among patients with atypically presenting clinical and radiologic features, and may represent IPF or CTD-UIP with improved prognosis if underlying CTD is diagnosed. Presenting radiologic and pathologic features alone are not predictive of underlying secondary cause or survival between the two groups.
Highlights
Usual interstitial pneumonia (UIP) is characterized by temporally heterogenous parenchymal fibrosis with architectural distortion, interstitial thickening, fibroblast foci, and honeycombing [1]
A defining pathologic finding in idiopathic pulmonary fibrosis (IPF), it has been found in other chronic fibrotic lung disease such as the connective tissue-disease associated interstitial lung disease (CTD-ILD) [2,3], chronic hypersensitivity pneumonitis (HP) [4], sarcoidosis [5], and advanced asbestosis [6]
It is well known that usual interstitial pneumonia (UIP) has worse prognosis than non-specific interstitial pneumonia (NSIP), the two most commonly presenting pathologies [8,9]
Summary
Usual interstitial pneumonia (UIP) is characterized by temporally heterogenous parenchymal fibrosis with architectural distortion, interstitial thickening, fibroblast foci, and honeycombing [1]. A defining pathologic finding in idiopathic pulmonary fibrosis (IPF), it has been found in other chronic fibrotic lung disease such as the connective tissue-disease associated interstitial lung disease (CTD-ILD) [2,3], chronic hypersensitivity pneumonitis (HP) [4], sarcoidosis [5], and advanced asbestosis [6]. It is well known that UIP has worse prognosis than non-specific interstitial pneumonia (NSIP), the two most commonly presenting pathologies [8,9] Both again may be idiopathic or associated with known etiologies, which has clinical significance in terms of survival and response to therapy [10]. Usual interstitial pneumonia (UIP) appears to portend better survival when associated with connective tissue disease (CTD-UIP), little is known about the presenting clinical, radiologic, and pathologic features that differentiate pathologically confirmed UIP with CTD from idiopathic pulmonary fibrosis (IPF). In patients with atypical radiologic and clinical features, what specific findings predict underlying IPF vs. CTD-UIP diagnosis and their respective long term survival?
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