Distinctive characteristics and prognostic significance of interstitial pneumonia with autoimmune features in patients with chronic fibrosing interstitial pneumonia

Yoshimura, Katsuhiro; Kono, Masato; Enomoto, Yasunori; Nishimoto, Koji; Oyama, Yoshiyuki; Yasui, Hideki; Hozumi, Hironao; Karayama, Masato; Suzuki, Yuzo; Furuhashi, Kazuki; Enomoto, Noriyuki; Fujisawa, Tomoyuki; Nakamura, Yutaro; Inui, Naoki; Sumikawa, Hiromitsu; Johkoh, Takeshi; Colby, Thomas V.; Sugimura, Haruhiko; Suda, Takafumi

doi:10.1016/j.rmed.2018.02.024

Cited by 54 publications

(80 citation statements)

References 43 publications

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“…Patients with IPAF most commonly meet criteria in the serologic and morphologic domains. [16][17][18] Oldham and colleagues observed that only 26.4% of patients with IPAF met criteria in all three domains, and only 6.1% of patients with a diagnosis of IPF met criteria in all three domains. 16…”

Section: Ipaf Classification Criteriamentioning

confidence: 99%

“…However, there is variability: results from one U.S. single-center study revealed that 67.9% of patients with IPAF never smoked, and in a study from Japan, 59.4% of the cohort was male. 18,20 In several cohorts, the most common symptom was Raynaud's phenomenon, present in 40 to 75% of patients. 16,17,20,22 The vast majority of research on IPAF has been retrospective, so firm conclusions should not be drawn about the frequency of symptoms at presentation.…”

Section: Clinical Domainmentioning

confidence: 99%

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Interstitial Pneumonia with Autoimmune Features and Undifferentiated Connective Tissue Disease

Chernau

Leone

Swigris

2019

Semin Respir Crit Care Med

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In 2015, a multidisciplinary task force comprising pulmonologists, rheumatologists, pathologists, and radiologists representing the European Respiratory Society and American Thoracic Society published a diagnostic classification schema for individuals with interstitial lung disease and autoimmune features who did not meet criteria for a defined connective tissue disease. The term interstitial pneumonia with autoimmune features (IPAF) was applied. Classification criteria are often nonspecific, but up to 90% of subjects with IPAF have serological evidence for autoimmunity (particularly (+) antinuclear antibodies). Distinguishing patients with IPAF from idiopathic pulmonary disorders may be difficult. The natural history and appropriate management of IPAF have not been clarified, as data are largely limited to retrospective studies. In this review, we discuss the salient clinical, serologic, histologic, and radiographic features of IPAF and discuss an approach to management.

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Section: Ipaf Classification Criteriamentioning

confidence: 99%

Section: Clinical Domainmentioning

confidence: 99%

Interstitial Pneumonia with Autoimmune Features and Undifferentiated Connective Tissue Disease

Chernau

Leone

Swigris

2019

Semin Respir Crit Care Med

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“…In contrast, diagnosing autoimmune features in patients with ILD is of importance, as it confers a better prognosis than idiopathic forms: ILD with autoimmune features but without classification criteria for connective tissue diseases (CTD) as well as connective tissue disease (CTD)-related ILD have a better prognosis than idiopathic ILD (3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment

Hervier

Uzunhan

2020

Front. Med.

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Inflammatory myopathies (IM) are auto-immune connective tissue diseases characterized by muscle involvement and by extramuscular manifestations. As such, pulmonary manifestations, which mainly include interstitial lung disease (ILD), often darken two out of four distinct IM, namely dermatomyositis and overlapping myositis. Being the initiation site of the disease and being the leading cause of morbidity and mortality, ILD is of major importance in this context. ILD has a heterogeneous expression among the patients, with various onset mode, various radiological pattern, various severity and finally with different prognoses, which are particularly difficult to predict at the time of IM diagnosis. Therefore, ILD is a challenging issue. Treatments are based on steroids and immunosuppressive or targeted therapies. Their respective place is yet poorly codified however and remains often based on clinician expertise. Dedicated clinical trials are lacking to date and are also difficult to build, due to difficulty of constituting large and homogeneous patient groups and to rigorously evaluate disease outcomes. Indeed, pulmonary function tests alone are being regularly defeated in IM, in which respiratory muscles are often involved. Composite scores, bringing together several lung parameters, should thus be developed and validated in the future, to better assess the disease response to treatment. This review aims to describe the current knowledge of IM immuno-pathogenesis, the clinical features associated with IM related-ILD, focusing of both severity and prognosis, and the actual therapeutic approaches.

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“…4 Since the initial publication of IPAF, several publications from diverse ILD programs describe characteristics and the natural history of IPAF from their respective centers around the world. [103][104][105][106][107] Each of the cohorts was retrospectively formed and affected by referral bias and/or how IPAF criteria were applied. It has become clear that as devised, the current definition of IPAF has been noted to allow for significant heterogeneity.…”

Section: Classification Criteria For Ipafmentioning

confidence: 99%

Interstitial Lung Disease and Other Pulmonary Manifestations in Connective Tissue Diseases

Mira-Avendano

Abril

Burger

et al. 2019

Mayo Clinic Proceedings

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Distinctive characteristics and prognostic significance of interstitial pneumonia with autoimmune features in patients with chronic fibrosing interstitial pneumonia

Abstract: A diagnosis of IPAF might predict a favorable prognosis and less risk of AEs in patients with CFIP.

Cited by 54 publications

References 43 publications

Interstitial Pneumonia with Autoimmune Features and Undifferentiated Connective Tissue Disease

Interstitial Pneumonia with Autoimmune Features and Undifferentiated Connective Tissue Disease

Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment

Interstitial Lung Disease and Other Pulmonary Manifestations in Connective Tissue Diseases

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