Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry

Abstract

Background The objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis of these patients. Methods A longitudinal observational multicenter study was performed (NEREA registry). It was carried out by a multidisciplinary team in seven Hospitals of Madrid. Patients were included from IPAF diagnosis. Main outcome: poor prognosis as functional respiratory impairment (relative decline in FVC % defined as ≥ 5% every 6 months). Covariates: therapy, sociodemographic, clinical, radiological patterns, laboratory and functional tests. Statistics: Survival techniques were used to estimate IR per 100 patients-semester with their 95% confidence interval [CI]. The influence of covariates in prognosis were analyzed through cox multivariate regression models (hazard ratio (HR) and [CI]). Results 79 IPAF were included, with a mean and a maximum follow-up of 3.17 and 12 years respectively. Along the study, 77.2% received treatment (52 glucocorticoids, 25 mycophenolate, 21 azathioprine, 15 rituximab and 11 antifibrotics). IR was 23.9 [19.9–28.8], and 50% of IPAF developed functional respiratory impairment after 16 months from its diagnosis. Multivariate analysis: usual interstitial pneumonia (UIP) had poorer prognosis compared to non-specific interstitial pneumonia (NSIP) (p = 0.001). In NSIP, positive ANA, increased the risk of poor prognosis. In UIP, glucocorticoids (HR: 0.53 [0.34–0.83]), age (HR: 1.04 [1.01–1.07]), and Ro-antibodies (HR: 0.36 [0.19–0.65]) influenced the prognosis. Conclusions IPAF have functional impairment during the first years of disease. Factors predicting deterioration differ between radiographic patterns. Our real-life study suggests the potential benefit of particular therapies in IPAF.

The Pattern and Progression of “Usual” Interstitial Pneumonia with Autoimmune Features: Comparison with Patients with Classic Interstitial Pneumonia with Autoimmune Features and Idiopathic Pulmonary Fibrosis

“…While immunosuppression was associated with increased mortality in IPF, immunosuppressive treatment was found to improve pulmonary functions in UIP associated with RA and CTD-ILD [43][44][45]. Similar results were also obtained in IPAF patients: treatment with Micophenolate Mofetil and Prednisone was associated with non-progression in IPAF patients, even controlling for the presence of a radiological pattern of UIP [46,47]. Recently, Yamano Y et al reported an interesting study on histologically proven UIP-IPAF patients, comparing the treatment of these patients [48].…”

Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study

“…The effectiveness of these drugs has been proved by several clinical trials. In some studies, the action of these molecules resulted in improvements when associated with immunosuppressants [36], although it should be noted that the treatment with immunosuppressants in IPF was also associated with an increased risk of acute exacerbation (AE) [37]; however, UIP-IPAF patients had significant benefit from the immunosuppressive treatment, despite the fact that the nuanced clinical picture did not allow for a specific classification as an autoimmune disease [30,38,39]. One of the current unmet needs for the coming years is the recognition of a subgroup of UIP patients who may have benefited from combined treatment with immunosuppressants and antifibrotics.…”