Nonspecific interstitial pneumonia: survival is influenced by the underlying cause

Nunès, Hilario; Schubel, Kirsten; Piver, Diane; Magois, Eline; Feuillet, S.; Uzunhan, Y.; Carton, Zohra; Tazi, Abdellatif; Lévy, Pierre; Brillet, Pierre-Yves; Nicholson, Andrew G.; Kambouchner, Marianne; Valeyre, Dominique

doi:10.1183/09031936.00148613

Cited by 65 publications

(55 citation statements)

References 34 publications

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“…Idiopathic (non‐IPAF) f‐NSIP was a negative prognostic factor for all‐cause mortality, and survival was poorer than that for IPAF f‐NSIP and CTD‐ILD. This result was similar to a previous UCTD cohort . However, another study recently reported that survival for IPAF was worse than that for CTD‐ILD, but the results were inconsistent because multiple radiological and pathological patterns such as UIP pattern were included.…”

Section: Discussionsupporting

confidence: 88%

“…Importantly, NSIP is heterogeneous, and outcomes of NSIP are influenced by its cause including connective tissue disease (CTD), hypersensitivity pneumonitis and undifferentiated CTD (UCTD) . Classification as UCTD with a clinical flavour of CTD associated with NSIP is a useful concept to identify the subgroup of idiopathic NSIP patients with good prognosis . New terminology of ‘interstitial pneumonia with autoimmune features (IPAF)’ was recently proposed .…”

Section: Introductionmentioning

confidence: 99%

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Prognostic factors and disease behaviour of pathologically proven fibrotic non‐specific interstitial pneumonia

et al. 2018

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Section: Discussionsupporting

confidence: 88%

Section: Introductionmentioning

confidence: 99%

Prognostic factors and disease behaviour of pathologically proven fibrotic non‐specific interstitial pneumonia

et al. 2018

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“…Wang et al 32 also noted that the greater the amount of fibrosis in their fibrotic NSIP cases, the worse the prognosis. Nunes et al 51 reported a median survival of 50 months for patients with CHP and an NSIP picture, a survival which was, interestingly, considerably worse than that for idiopathic NSIP or NSIP associated with a connective tissue disease.…”

Section: Relationship Of Pathologic Findings In Chp Tomentioning

confidence: 99%

Pathology of Chronic Hypersensitivity Pneumonitis What Is It? What Are the Diagnostic Criteria? Why Do We Care?

Churg

Bilawich

Wright

2017

Archives of Pathology &Amp; Laboratory Medicine

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Context.— Chronic hypersensitivity pneumonitis (CHP) has emerged from obscurity during the past 15 years and is now recognized as a very common form of fibrosing interstitial pneumonia but one that is frequently misdiagnosed both clinically and on surgical lung biopsy as usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) or fibrotic nonspecific interstitial pneumonia. Objective.— To review the pathologic features of CHP. Data Sources.— Clinical, pathology, and radiology literature were used. Conclusions.— Upper lobe–predominant fibrosis and/or air-trapping on computed tomography scan are features of CHP but not UIP/IPF; however, radiologic separation is possible in only about 50% of cases. Morphologically, CHP sometimes mimics UIP/IPF, but CHP often shows isolated foci of peribronchiolar (centrilobular) fibrosis, frequently associated with fibroblast foci, and in CHP, fibrosis may bridge from the centrilobular region to another bronchiole, an interlobular septum, or the pleura (“bridging fibrosis”). This set of findings is uncommon in UIP/IPF. In addition, CHP may produce a picture of fibrotic nonspecific interstitial pneumonia. Although giant cells/granulomas are usually present in subacute hypersensitivity pneumonitis, they are much less frequently found in CHP, and their absence does not contradict the diagnosis. This diagnostic separation is clinically important because CHP is treated differently than UIP/IPF is (immunosuppressive agents versus antifibrotic agents); further, there are some data to suggest that removing the patient from antigen exposure improves outcome, and there is evidence that patients with CHP have a much better survival prognosis after lung transplantation than do patients with UIP/IPF. In most cases, accurate diagnosis of CHP requires consultation among clinicians, radiologists, and pathologists.

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“…Our study population contains only iNSIP patients with no indication of connective tissue or immune disease [195]. Although the clinical features are the same, NSIP is reported to have a better prognosis than iNSIP, [196]. The incidence of iNSIP is estimated to be 3 per million [197].…”

Section: Idiopathic Nonspecific Interstitial Pneumoniamentioning

confidence: 99%

Regulation of MUC5B Expression in Idiopathic Pulmonary Fibrosis

Helling

Gerber

Kadiyala

et al. 2017

Am J Respir Cell Mol Biol

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Idiopathic pulmonary fibrosis (IPF) is the most common and the most aggressive fibrosing interstitial lung disease (ILD). Despite recent promising clinical trials, IPF remains incurable and largely untreatable. Genetic studies have identified several risk loci for both sporadic and familial forms of IPF. A single variant upstream of MUC5B is predicted to account for more than 30% of all IPF risk. This variant, rs35705950, is associated with expression of MUC5B in healthy lung tissue. However, the mechanism underlying the relationship between rs35705950 and MUC5B expression remains unclear.The first goal of my thesis research was to determine whether rs35705950 is also a risk factor for other forms of ILD. Genomic DNA from individuals with IPF, HP, COPD, iNSIP, and RB-ILD was obtained and genotyped for the common MUC5B promoter variant. In these populations the risk allele was associated with IPF and iNSIP which suggested a potential shared disease mechanism. Additionally, I showed that in the IPF and control populations rs35705950 is associated with lung MUC5B expression.The second goal of my thesis research was to investigate MUC5B promoter DNA methylation which has previously been shown to play a role in MUC5B expression.Methylation analysis of the 4KB region upstream of MUC5B identified two differentially methylated regions (DMRs) associated with IPF, one DMR associated with MUC5B iv expression, and one DMR associated with rs35705950. The IPF, MUC5B expression, and rs35705950 associated DMRs are all differentially methylated at a cluster of 11 CpG motifs. overlapping DMR, including the motif disrupted by rs35705950. These findings suggest that DNA methylation may play a role in MUC5B gene regulation and IPF risk.Next, I used cross species analysis to identify highly conserved domains within the overlapping DMR. Evolutionary conservation indicated selective pressures to maintain sequences overtime and suggests biological importance. The overlapping DMR contains a highly conserved binding motif for FOXA2, a transcription factor. Further analysis using ChIP-qPCR, reporter constructs, and siRNA, established a role for FOXA2 in regulation of MUC5B expression in lung tissue. Additionally, siRNA knock down identified 3 other transcription factors which are associated with MUC5B expression; STAT3, HOXA9 and ZBTB7A. These transcriptional regulators provide insight into possible therapeutic intervention for MUC5B dysregulation and IPF.The form and content of this abstract are approved. I recommend its publication.

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Nonspecific interstitial pneumonia: survival is influenced by the underlying cause

Cited by 65 publications

References 34 publications

Prognostic factors and disease behaviour of pathologically proven fibrotic non‐specific interstitial pneumonia

Prognostic factors and disease behaviour of pathologically proven fibrotic non‐specific interstitial pneumonia

Pathology of Chronic Hypersensitivity Pneumonitis What Is It? What Are the Diagnostic Criteria? Why Do We Care?

Regulation of MUC5B Expression in Idiopathic Pulmonary Fibrosis

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