Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features

Abstract

BackgroundTo identify the prognostic factors for survival in patients with interstitial pneumonia with autoimmune features (IPAF) who meet the serological domain of the IPAF criteria.MethodsWe retrospectively analysed 99 IPAF patients who met the serological domain and were hospitalised at the Respiratory Medicine Unit of Kurashiki Central Hospital from 1999 to 2015. The high-resolution computed tomography findings were usual interstitial pneumonia (UIP; n = 1), non-specific interstitial pneumonia (NSIP; n = 63), NSIP with organizing pneumonia (OP) overlap (n = 15), and OP (n = 20). One patient who had radiological UIP pattern, and met the serological and clinical domains was excluded. The clinical characteristics, radiological findings, administered therapy, and prognosis of the remaining 98 IPAF patients who met the serological and morphological domains were analysed.ResultsThe median age of the 98 IPAF patients was 68 years, and 41 (41.8%) of them were men. Twelve (12.2%) of the 98 IPAF patients developed other characteristics and were diagnosed with connective tissue disease (CTD) later during the median follow-up of 4.5 years. Univariate Cox analysis revealed systemic sclerosis (SSc)-specific and SSc-associated antibodies (ANA nucleolar pattern, ANA centromere pattern, anti-ribonucleoprotein and anti-Scl-70) positive IPAF, radiological NSIP pattern, bronchoalveolar lavage fluid lymphocytes >15%, and age as significant prognostic factors for survival. Multivariate Cox analysis revealed radiological NSIP pattern (hazard ratio [HR], 4.48; 95% confidence interval [CI], 1.28–15.77, p = 0.02) and age (HR, 1.07; 95% CI, 1.02–1.11, p = 0.01) were significantly associated with worse survival.ConclusionsWe confirmed that radiological NSIP pattern and age are poor prognostic factors for the survival of IPAF patients. This study suggested that the autoantibodies that are highly specific for certain connective tissue diseases might be less important for the prognosis of IPAF compared with the radiological-pathological patterns. The relatively high proportion of IPAF patients who developed CTD later suggests the importance of careful observation for evolution to CTD in IPAF.

State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances

“…CHARTRAND et al [22] described the youngest population ( p<0.0001 compared with others), and the highest percentage of females ( p=0.03 versus OLDHAM et al [18] and p=0.01 versus AHMAD et al [23], but similar to ITO et al [24]). In the study by OLDHAM et al [18], the population included the highest proportion of smokers ( p=0.03 versus CHARTRAND et al [22], p=0.01 versus AHMAD et al [23] and p=0.01 versus ITO et al [24]) and the lowest proportion of patients treated with corticosteroids ( p<0.0001).…”

“…The last contribution, produced by ITO et al [24], investigated 99 patients with IPAF. The only patient with a UIP pattern and at least one clinical and serological criterion for IPAF was excluded.…”

“…For the morphological domain, the populations in the studies by CHARTRAND et al [22] and ITO et al [24] presented higher proportions of patients with significantly higher presence of NSIP patterns ( p=0.009 2). In addition, differences in terms of treatment were reported.…”

“…Considering the serological domain, the population in the study by CHARTRAND et al [22] showed the lowest proportion of ANA positivity (considering a minimum titre of 1:320 for any pattern and any titre for centromeric and nucleolar patterns) ( p<0.0001 compared to OLDHAM et al [18] and ITO et al [24]; p=0.0001 compared to AHMAD et al [23]). Conversely, the population in CHARTRAND et al [22] showed a significantly higher proportion of anti-Ro/Sjögren syndrome-A, anti-RNP, anti-Smith and ATSA antibodies compared to the other studies (table 3).…”

“…In the study by OLDHAM et al [18], the population included the highest proportion of smokers ( p=0.03 versus CHARTRAND et al [22], p=0.01 versus AHMAD et al [23] and p=0.01 versus ITO et al [24]) and the lowest proportion of patients treated with corticosteroids ( p<0.0001). Regarding UIP pattern, each study is different from the others, with the highest percentage in the population of OLDHAM et al [18] population and the lower in the study by ITO et al [24] (table 2).…”