Idiopathic nonspecific interstitial pneumonia: an interstitial lung disease associated with autoimmune disorders?

Romagnoli, Micaela; Nannini, Carlotta; Piciucchi, Sara; Girelli, Francesco; Casoni, Gian Luca; Ravaglia, Claudia; Tomassetti, Sara; Gurioli, Carlo; Gavelli, Giampaolo; Carloni, Angelo; Dubini, Alessandra; Cantini, Fabrizio; Chilosi, Marco; Poletti, Venerino

doi:10.1183/09031936.00094910

Cited by 80 publications

(91 citation statements)

References 33 publications

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“…This is consistent with several studies that show a clear link between NSIP histology and CTD [9,42,43]. Two recent studies have demonstrated that 10% and 52% of their patients who were considered to have idiopathic NSIP developed CTD during the follow-up period [43,44]. In another study, a close association between NSIP histology and UCTD was demonstrated: NSIP was the histological diagnosis in 15 out of 17 patients with UCTD (OR 50; p50.0001), leading the authors to surmise that the application of diagnostic criteria for UCTD may identify NSIP before obtaining surgical lung biopsy [9].…”

Section: Discussionsupporting

confidence: 80%

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Significance of connective tissue disease features in idiopathic interstitial pneumonia

et al. 2011

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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: 80%

Section: Discussionsupporting

confidence: 77%

Significance of connective tissue disease features in idiopathic interstitial pneumonia

et al. 2011

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“…As in previous studies, our patients with UCTD were more likely to be females [5,8,30] and nonsmokers [5,30] than those with idiopathic NSIP. UCTD patients were similar to CTDs patients, including in terms of demographic characteristics and survival.…”

Section: Discussionsupporting

confidence: 56%

Nonspecific interstitial pneumonia: survival is influenced by the underlying cause

et al. 2014

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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. @ERSpublications NSIP patients should be investigated for the presence of an underlying cause, which significantly impacts survival

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“…Indeed, the pathologic pattern observed in CTD does not seem to affect the prognosis and survival as clearly as in the idiopathic setting. Although patients with idiopathic NSIP [20] or idiopathic desquamative interstitial pneumonia [21] have a dramatically better long-term survival than those with IPF [22][23][24], such a difference was not found in patients with systemic sclerosis or rheumatoid arthritis [3,10]. Studies correlating the pathological pattern with outcome all suffer the limitation of a high risk of constitutive selection bias, with lung biopsy being performed in individuals with the least typical pattern of disease at imaging.…”

Section: Does the Diagnosis Of Established Ctd Affect Prognosis Or Mamentioning

confidence: 99%

“…In one series of ILD associated with anti-synthetase antibodies, respiratory manifestations occurred before the onset of muscle or skin disease in 55% of the patients [32]. Long-term follow-up of patients with NSIP may lead to the subsequent diagnosis of CTD in a significant proportion of patients [20,33,34], suggesting that subtle extra-thoracic features of systemic disease present at baseline might have been overlooked. Identification of the CTD may be difficult when the onset of disease is acute or subacute, as often observed in inflammatory idiopathic myopathy (especially associated with anti-melanoma differentiation associated gene (MDA5) antibodies) [35].…”

Section: Ctd Features May Be Missed In the Diagnostic Approach Of Ildmentioning

confidence: 99%

Significance of connective tissue diseases features in pulmonary fibrosis

Cottin

2013

Eur Respir Rev

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Interstitial lung disease (ILD) can occur in any of the connective tissue diseases (CTD) with varying frequency and severity, and an overall long-term prognosis that is less severe than that of idiopathic pulmonary fibrosis (IPF). Because ILD may be the presenting manifestation of CTD and/or the dominant manifestation of CTD, clinical extra-thoracic manifestations should be systematically considered in the diagnostic approach of ILD. When present, autoantibodies strongly contribute to the recognition and classification of the CTD. Patients with clinical extrathoracic manifestations of CTD and/or autoantibodies (especially with a high titer and/or the antibody is considered “highly specific” of an autoimmune condition), but who do not fit with established international CTD criteria may be called undifferentiated CTD or “lung-dominant CTD”. Although it remains to be determined which combination of symptoms and serologic tests best identify the subset of patients with clinically relevant CTD features, available evidence suggests that such patients may have distinct clinical and imaging presentation and may portend a distinct clinical course. However, autoantibodies alone when present in IPF patients do not seem to impact prognosis or management. Referral to a rheumatologist and multidisciplinary discussion may contribute to management of patients with undifferentiated CTD

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Idiopathic nonspecific interstitial pneumonia: an interstitial lung disease associated with autoimmune disorders?

Cited by 80 publications

References 33 publications

Significance of connective tissue disease features in idiopathic interstitial pneumonia

Significance of connective tissue disease features in idiopathic interstitial pneumonia

Nonspecific interstitial pneumonia: survival is influenced by the underlying cause

Significance of connective tissue diseases features in pulmonary fibrosis

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