Nonspecific interstitial pneumonia: a real clinical entity?

Nagai, Sonoko; Handa, Tomohiro; Tabuena, Rollin P.; Kitaichi, Masanori; Izumi, Tohru

doi:10.1016/j.ccm.2004.04.009

Cited by 25 publications

(18 citation statements)

References 43 publications

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“…24 (24%) patients fulfilled the criteria for this algorithm, which was associated with a 10-fold increase in likelihood of NSIP. The striking demographic associations with NSIP observed in the present study mirror previous observations [17,[46][47][48][49], and lend further support for the inclusion of a typical demographic profile in diagnostic and prognostic algorithms. The age threshold of 50 yrs in females evaluated in our study was based upon the statement by an expert group that age ,50 yrs is an atypical feature in IPF and the fact that 50 yrs was the mean age for females in this study [23].…”

Section: Discussionsupporting

confidence: 89%

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Significance of connective tissue disease features in idiopathic interstitial pneumonia

Corte

Copley

Desai

et al. 2011

European Respiratory Journal

182

169

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In idiopathic interstitial pneumonia (IIP), the significance of connective tissue disease (CTD) features in the absence of a specific CTD diagnosis remains unclear. We studied the clinical and prognostic utility of a diagnosis of undifferentiated CTD (UCTD) in patients with biopsy-proven IIP.IIP patients undergoing surgical lung biopsy were studied (nonspecific interstitial pneumonia (NSIP), n545; idiopathic pulmonary fibrosis, n556). UCTD was considered present when serum autoantibodies were present and symptoms or signs suggested CTD. The relationship between UCTD and NSIP histology was evaluated. A clinical algorithm that best predicted NSIP histology was constructed using a priori variables. The prognostic utility of UCTD, and of this algorithm, was evaluated.UCTD was present in 14 (31%) NSIP and seven (13%) IPF patients. UCTD was not associated with a survival benefit. The algorithm predictive of NSIP (OR 10.4, 95% CI 3.21-33.67; p,0.0001) consisted of the absence of typical high-resolution computed tomography (HRCT) features for IPF and 1) a compatible demographic profile (females aged ,50 yrs) or 2) Raynaud's phenomenon. In patients with an HRCT scan not typical for IPF, this algorithm predicted improved survival (hazard ratio 0.35, 95% CI 0.14-0.85; p50.02) independent of IIP severity.UCTD is associated with NSIP histology. However, the diagnostic and prognostic significance of UCTD in IIP patients remains unclear.

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Section: Discussionsupporting

confidence: 89%

“…A recent expert commentary has highlighted the fact that many IIP patients do not satisfy specific CTD diagnostic criteria, but clearly have significant end-organ disease [16]. The majority of patients with these autoimmune features have NSIP at surgical lung biopsy [17,18]. Thus, it has been proposed that UCTD manifests in the lung as ''idiopathic NSIP'' [9].…”

mentioning

confidence: 99%

Significance of connective tissue disease features in idiopathic interstitial pneumonia

Corte

Copley

Desai

et al. 2011

European Respiratory Journal

182

169

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“…Finally, according to Riha and Nagai et al [1122] the honeycombing pattern was found in HRCTs of NSIP patients. We suggest that this may have been because UIP often coexists with NSIP and, hence, a small proportion of HRCTs in NSIP patients shows the honeycombing pattern.…”

Section: Discussionmentioning

confidence: 98%

Comparison of clinicoradiologic manifestation of nonspecific interstitial pneumonia and usual interstitial pneumonia/idiopathic pulmonary fibrosis: A report from NRITLD

et al. 2008

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BACKGROUND:Ever since Katzenstein and Fiorelli introduced the term nonspecific interstitial pneumonia (NSIP) to denote those cases of interstitial pneumonia that cannot be categorized as any of the other types of idiopathic interstitial pneumonias (IIP), there has been continuing debate on whether it is a real clinical entity or not. The American Thoracic Society/European Respiratory Symposium task group tried to identify idiopathic NSIP as a separate disease and exclude it from the category of IIP. However, it appears that the clinical presentation of NSIP and usual interstitial pneumonia (UIP) are the same.OBJECTIVE:To show that the radiologic features of NSIP and UIP should be relied upon, instead of clinical presentation and pathologic findings, to differentiate between the two.MATERIALS AND METHODS:Consecutive patients who had received a diagnosis of either NSIP or UIP on the basis of open lung biopsy between January 2001 and December 2007 were identified for inclusion in this retrospective review. The study included 61 subjects: 32 men and 29 women with a mean age of 59.39 ± 14.5 years. Chest computed tomography images of all the cases were collected for a review. High resolution computed tomography (HRCT) and all pathologic specimens were also evaluated. A weighted kappa coefficient was used to evaluate whether radiology can be used instead of biopsy for the diagnosis of NSIP and UIP. Comparison of the mean ages and the time intervals (i.e., interval between symptom onset and the time of diagnosis) in the UIP and NSIP groups was done using the Mann-Whitney U test. Association between gender and biopsy result was evaluated by the Fisher exact test. Data were evaluated using SPSS, v.13.RESULTS:Sixty-one patients were included in this study, 32 were male and 29 were female. On the basis of biopsy findings, 50 (82%) patients had UIP and 11 (18%) had NSIP. Thirty (60%) of the 50 patients who had UIP were male and 20 (40%) were female; 2 (18.2%) of the 11 patients who suffered from NSIP were male and 9 (81.8%) were female. Based on HRCT findings, 36 (60%) patients were diagnosed to have UIP and 24 (40%) were diagnosed with NSIP. When diagnosis was based on biopsy findings, the time interval in the UIP group was 13.59 ± 8.29 months and in the NSIP group it was 7.90 ± 4.18 months. When diagnosed on the basis of HRCT findings, the time interval in the UIP group was 14.22 ± 8.94 months and in the NSIP group it was 10.54 ± 5.78 months. When diagnosis was on the basis of biopsy, the mean age in the UIP group was 61.30 ± 14.18 years and in the NSIP group it was 50.73 ± 13.14 years.CONCLUSION:HRCT can be used instead of invasive methods like lung biopsy to differentiate between UIP and NSIP.

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“…Furthermore, surgical lung biopsy specimens of early stages from patients with IPF who were detected by CT scan showed the same temporal heterogeneity (i.e., UIP pattern) as seen in advanced cases of IPF; NSIP pattern was not observed (26). Based on these data, current evidence suggest that, in general, idiopathic fibrosing NSIP pattern represents a distinct clinical condition, and not an early (or inactive) stage of IPF (58). The finding of both UIP and NSIP pattern in multiple biopsies from the same patients (59,60) raises the possibility that NSIP pattern may, on occasion, be a nonspecific manifestation of IPF.…”

Section: Nsipmentioning

confidence: 93%

Classification and Natural History of the Idiopathic Interstitial Pneumonias

Kim¹

2006

Proceedings of the American Thoracic Society

388

290

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In the American Thoracic Society/European Respiratory Society consensus classification, idiopathic interstitial pneumonias are classified into seven clinicopathologic entities. The classification is largely based on histopathology, but depends on the close interaction of clinician, radiologist, and pathologist. An accurate diagnosis can be very difficult, especially when deciding between idiopathic pulmonary fibrosis and fibrotic nonspecific interstitial pneumonia; better diagnostic markers are needed. The prognosis of idiopathic pulmonary fibrosis is very poor, with median survival of 2-4 yr after the diagnosis, yet the course of individual patients is highly variable. Predicting prognosis in the individual patient is challenging but various clinical and radiologic variables have been identified. According to several recent clinical trials, the natural history of this disease may involve periods of relative stability punctuated by acute exacerbations of disease that result in substantial morbidity or death. Nonspecific interstitial pneumonia is characterized by a distinct histopathologic appearance and a better prognosis than idiopathic pulmonary fibrosis. However, there is still confusion and controversy over the relationship between idiopathic pulmonary fibrosis and fibrotic nonspecific interstitial pneumonia.

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Nonspecific interstitial pneumonia: a real clinical entity?

Cited by 25 publications

References 43 publications

Significance of connective tissue disease features in idiopathic interstitial pneumonia

Significance of connective tissue disease features in idiopathic interstitial pneumonia

Comparison of clinicoradiologic manifestation of nonspecific interstitial pneumonia and usual interstitial pneumonia/idiopathic pulmonary fibrosis: A report from NRITLD

Classification and Natural History of the Idiopathic Interstitial Pneumonias

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