Nonspecific interstitial pneumonia: survival is influenced by the underlying cause.

Idiopathic, nonspecific interstitial pneumonia (NSIP) is most often associated with various clinical disorders, including connective tissue diseases (CTDs) and chronic hypersensitivity pneumonitis (cHP). Emerging evidence also suggests that "idiopathic" NSIP may be the lung manifestation of undifferentiated CTD (UCTD). However, whether or not NSIP outcome is influenced by the underlying cause remains uncertain. This retrospective study included 127 biopsy-proven NSIP patients (65 women, mean ± sd age 55 ± 12 years). Survivals were estimated using a Kaplan-Meier curve and compared using the log-rank test. Multivariate analyses were based on a Cox model. 15 (11.8%) patients had cHP, 29 (22.8%) had CTD, 32 (25.2%) satisfied the Kinder criteria for UCTD and 51 (40.1%) had idiopathic NSIP. At the end of follow-up (mean ± sd 64 ± 54 months), a difference in survival was observed between aetiological groups (p=0.002). Survival was better for UCTD than for idiopathic NSIP (p=0.020) and similar to that observed for CTD. cHP survival tended to be poorer than that of idiopathic NSIP (p=0.087) and was an independent predictor of mortality (hazard ratio 2.17, 95% CI 1.05-4.47; p=0.035). NSIP outcome is influenced by its cause. cHP exhibits the highest mortality. UCTD does not differ from CTD supporting the concept of autoimmune NSIP, with a prognosis that is better than that of idiopathic NSIP.

Nunes H ，Schubel K ，Piver D ，Magois E ，Feuillet S ，Uzunhan Y ，Carton Z ，Tazi A ，Levy P ，Brillet PY ，Nicholson AG ，Kambouchner M ，Valeyre D ... -  《-》

Broader criteria of undifferentiated connective tissue disease in idiopathic interstitial pneumonias.

Kinder et al. proposed a broader definition of undifferentiated connective tissue disease (UCTD) and reported that the entity of nonspecific interstitial pneumonia (NSIP) is a lung manifestation of this more broadly defined UCTD. However, a retrospective study did not support their findings and its clinical significance remains unclear. We prospectively evaluated the significance of this broadly defined UCTD in idiopathic interstitial pneumonias (IIPs) in consecutive patients with surgical lung biopsy. Patients were evaluated with a symptoms check list and underwent comprehensive serologic testing as screening for UCTD. Clinical characteristics, high-resolution CT images, lung biopsy specimens, serial FVC change, and survival were analyzed. Among 76 patients with IIPs, 24 patients (32%) fulfilled the UCTD criteria. Diagnosis of 24 patients with UCTD was usual interstitial pneumonia in 12 (50%), NSIP in 7 (29%), and unclassifiable interstitial lung disease (ILD) in 5 (21%). The diagnosis of 52 patients who did not have UCTD was idiopathic pulmonary fibrosis in 27 (52%), NSIP in 11 (21%), unclassifiable ILD in 13 (25%) and cryptogenic organizing pneumonia in 1 (2%). One-year and two-year FVC changes showed no significant difference between UCTD and non-UCTD, however, significant differences in FVC change were observed among histopathological diagnoses both in UCTD and in non-UCTD. In multivariate survival analysis, %FVC and histopathological UIP pattern were independent predictors for survival but UCTD diagnosis was not. A diagnosis of UCTD was not useful in discriminating NSIP or in predicting disease progression and prognosis in our cohort of IIPs. Histopathological UIP pattern was an independent predictor for mortality irrespective of a diagnosis of UCTD.

Kondoh Y ，Johkoh T ，Fukuoka J ，Arakawa H ，Tanaka T ，Watanabe N ，Sakamoto K ，Kataoka K ，Kimura T ，Taniguchi H ... -  《-》

Significance of connective tissue disease features in idiopathic interstitial pneumonia.

Corte TJ ，Copley SJ ，Desai SR ，Zappala CJ ，Hansell DM ，Nicholson AG ，Colby TV ，Renzoni E ，Maher TM ，Wells AU ... -  《-》

Distinct prognosis of idiopathic nonspecific interstitial pneumonia (NSIP) fulfilling criteria for undifferentiated connective tissue disease (UCTD).

Suda T ，Kono M ，Nakamura Y ，Enomoto N ，Kaida Y ，Fujisawa T ，Imokawa S ，Yasuda K ，Hashizume H ，Yokomura K ，Toyoshima M ，Koshimizu N ，Suganuma H ，Shirai T ，Hashimoto D ，Inui N ，Colby TV ，Chida K ... -  《-》

Interstitial pneumonia related to undifferentiated connective tissue disease: pathologic pattern and prognosis.

Kim HC ，Ji W ，Kim MY ，Colby TV ，Jang SJ ，Lee CK ，Han SB ，Kim DS ... -  《-》