Fibrosing interstitial pneumonia predicts survival in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD).

Rheumatoid arthritis (RA) is a systemic autoimmune disorder with a variety of extra-articular manifestations. The lung is a common target and diffuse parenchymal lung disease can appear as any of the patterns found with idiopathic interstitial pneumonia. Controversy exists as to the prognostic significance of these patterns among patients with RA-ILD. We retrospectively identified 48 patients with a diagnosis of RA-ILD confirmed by surgical lung biopsy. The pathology was reviewed by four expert pulmonary pathologists. We examined survival after stratifying on the presence or absence of fibrotic ILD, and contrasted it with a matched idiopathic pulmonary fibrosis (IPF) population. The Cox proportional hazards model was used to identify independent predictors of survival. The majority of subjects were male smokers with physiologic restriction. A usual interstitial pneumonia (UIP)-pattern was identified in 31% of subjects. Median survival time for the entire cohort was 1360 days. Subjects with fibrotic ILD had worse survival than subjects with non-fibrotic ILD (log rank p = 0.02). There was no difference in survival between UIP-pattern RA-ILD subjects and IPF controls (log rank p = 0.94). Multivariable analysis revealed that age (hazard ratio [HR] = 1.04, p = 0.01) and fibrosis (HR = 2.1, p = 0.02) were independent predictors of mortality. Both cellular and fibrosing ILD patterns are common among RA-ILD patients who undergo surgical lung biopsy. These patients have a shortened survival when compared to the general population and all-comers with RA. Age and the presence of a fibrosing interstitial pneumonia predict shortened survival in these patients. Survival in UIP is similar to matched IPF patients.

Solomon JJ ，Ryu JH ，Tazelaar HD ，Myers JL ，Tuder R ，Cool CD ，Curran-Everett D ，Fischer A ，Swigris JJ ，Brown KK ... -  《-》

High resolution computed tomography pattern of usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease: Relationship to survival.

Interstitial lung disease is a common extra-articular manifestation of rheumatoid arthritis (RA-ILD) and is associated with significant morbidity and mortality. However, limited data exist regarding predictors of mortality. We sought to examine the prognostic value of the high-resolution computed tomography (HRCT) patterns in patients with RA-ILD. RA-ILD patients with HRCT patterns of usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) were identified among a longitudinal cohort of individuals evaluated at National Jewish Health. A total of 158 subjects were included in the study. For each subject, the earliest available HRCT was reviewed independently by two expert thoracic radiologists blinded to clinical data. HRCT patterns were classified as demonstrating definite UIP, possible UIP, or NSIP. Kaplan-Meier curves were generated and survival was compared among the three patterns using a log rank test for trend. One hundred subjects (63%) had HRCT findings classified as definite UIP, 23 (15%) as possible UIP and 35 (22%) as NSIP. No difference in survival was seen between subjects with definite UIP versus those with possible UIP. The combined group of subjects with either definite- or possible UIP had significantly worse survival than those with NSIP (log-rank p = 0.03). In patients with RA-ILD, patients with either definite UIP or possible UIP have equally poor survival when compared to those with an NSIP pattern.

Yunt ZX ，Chung JH ，Hobbs S ，Fernandez-Perez ER ，Olson AL ，Huie TJ ，Keith RC ，Janssen WJ ，Goldstein BL ，Lynch DA ，Brown KK ，Swigris JJ ，Solomon JJ ... -  《-》

Rheumatoid Arthritis (RA) associated interstitial lung disease (ILD).

Rheumatoid Arthritis (RA) is the most common Connective Tissue Disease (CTD) and represents an increasing burden on global health resources. Interstitial lung disease (ILD) has been recognised as a complication of RA but its potential for mortality and morbidity has arguably been under appreciated for decades. New studies have underscored a significant lifetime risk of ILD development in RA. Contemporary work has identified an increased risk of mortality associated with the Usual Interstitial Pneumonia (UIP) pattern which shares similarity with the most devastating of the interstitial pulmonary diseases, namely Idiopathic Pulmonary Fibrosis (IPF). In this paper, we discuss recent studies highlighting the associated increase in mortality in RA-UIP. We explore associations between radiological and histopathological features of RA-ILD and the prognostic implications of same. We emphasise the need for translational research in this area given the growing burden of RA-ILD. We highlight the importance of the respiratory physician as a key stakeholder in the multidisciplinary management of this disorder. RA-ILD focused research offers the opportunity to identify early asymptomatic disease and define the natural history of this extra articular manifestation. This may provide a unique opportunity to define key regulatory fibrotic events driving progressive disease. We also discuss some of the more challenging and novel aspects of therapy for RA-ILD.

O'Dwyer DN ，Armstrong ME ，Cooke G ，Dodd JD ，Veale DJ ，Donnelly SC ... -  《-》

Review series: Aspects of interstitial lung disease: connective tissue disease-associated interstitial lung disease: how does it differ from IPF? How should the clinical approach differ?

de Lauretis A ，Veeraraghavan S ，Renzoni E 《-》

Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease.

Kim EJ ，Elicker BM ，Maldonado F ，Webb WR ，Ryu JH ，Van Uden JH ，Lee JS ，King TE Jr ，Collard HR ... -  《-》