Interstitial Pneumonia With Autoimmune Features: Overview of Proposed Criteria and Recent Cohort Characterization

Lee, Cathryn T.; Oldham, Justin M.

doi:10.1097/cpm.0000000000000227

Cited by 12 publications

(5 citation statements)

References 54 publications

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“…As demonstrated, IPAF studies have shown substantial heterogeneity among patients meeting IPAF criteria with regards to clinical features, disease behaviour and response to treatment. This heterogeneity has been well described in previous reviews [45–49] and confirmed in more recent studies. As such, we suggest that IPAF is unlikely to represent a distinct interstitial lung disease.…”

Section: Discussionsupporting

confidence: 83%

Interstitial pneumonia with autoimmune features: from research classification to diagnosis

Glenn

Pugashetti

Oldham

et al. 2021

Current Opinion in Pulmonary Medicine

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Purpose of reviewThe term interstitial pneumonia with autoimmune features (IPAF) was first proposed by an international task force in 2015 as a research classification to standardise nomenclature regarding patients with idiopathic interstitial pneumonia and features of connective tissue disease. However, how the use of this term and its proposed definition translates to clinical practice remains uncertain. This review will provide a comprehensive overview of studies of IPAF cohorts to date, discuss the consideration of IPAF as a distinct diagnostic entity and outline a suggested approach to patient management.Recent findingsConsiderable heterogeneity exists between published IPAF cohorts, with some cohorts exhibiting similarities to those with connective tissue disease-associated interstitial lung disease (CTD-ILD), and others more similar to idiopathic interstitial pneumonias including idiopathic pulmonary fibrosis (IPF). Little data exist to inform the management of patients who fulfil the IPAF criteria. Preliminary data supports pragmatic management of these patients as having a working clinical diagnosis of either idiopathic interstitial pneumonia or CTD-ILD. Future research studies into this approach are required.SummaryThe term IPAF, and its definition, have been of fundamental benefit to facilitating research in this diverse patient group. However, to date, there remain many unanswered questions regarding their natural histories and response to treatment.

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Section: Discussionsupporting

confidence: 83%

Interstitial pneumonia with autoimmune features: from research classification to diagnosis

Glenn

Pugashetti

Oldham

et al. 2021

Current Opinion in Pulmonary Medicine

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“…Similarly, in recent years a subset of patients has been described, who have idiopathic interstitial pneumonia (IIP) and autoimmune features (IPAF), who do not meet the criteria for a connective tissue disease (CTD) but have at least one sign or symptom suggestive of a CTD and at least one serological test reflective of an autoimmune process [ 21 ]. These patients have been better characterised, and some studies also suggest that patients who met the IPAF criteria had a significantly worse survival than those with IIP without autoimmune features [ 22 ], although substantial variability in outcome has been reported [ 23 ]. As in hypersensitivity pneumonitis, the mechanisms and genetic susceptibility triggering the autoimmune process is unknown.…”

Section: Discussionmentioning

confidence: 99%

A major genetic determinant of autoimmune diseases is associated with the presence of autoantibodies in hypersensitivity pneumonitis

et al. 2020

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BackgroundHypersensitivity pneumonitis (HP) is an immune-mediated disease triggered by exposure to organic particles in susceptible individuals. It has been reported that a subgroup of patients with HP develops autoantibodies with or without clinical manifestations of autoimmune disease. However, the mechanisms involved in this process and the effect of the autoantibodies on clinical course in HP is unknown. We evaluated the association between HLA class II alleles and HP patients with and without autoantibodies.MethodsOne hundred seventy HP patients were included. We analysed the presence of antinuclear antibodies, rheumatoid factor, anti-SSA/Ro, anti-SSB/La, and anti-CCP at the time of diagnosis. In addition, in a subset of patients, we evaluated anti-Scl-70, ANCA, and anti-DNA. HLA typing was performed by PCR-SSP in a high-resolution modality, including HLA-DRB1 and HLA-DQB1 loci. Statistical analysis was performed employing Epi-Info v7 and SPSSv20.ResultsSixty HP patients showed sera autoantibodies (HPAbs+), and 110 HP patients did not (HPAbs−). The frequency of the allele HLA-DRB1*03:01 was remarkably increased in the HPAbs+ group (10.8% versus 0.45%; OR=30.14, 95%CI 3.83–237.1; p=1.65E-04 after Bonferroni's correction). Likewise, we found that the haplotype DRB1*03:01-DQB1*02:01, which is part of the 8.1 ancestral haplotype, a major genetic determinant of autoimmune diseases confers significant risk to develop autoantibodies (OR=19.23, 95%CI 2.37–155.9; p=0.0088 after Bonferroni's correction). Also, the HLA-DRB1*03:01 allele was associated with higher mortality in patients with HP (adjusted OR=5.9, 95%IC 1.05–33.05; p=0.043).ConclusionsA subset of HP patients presents circulating autoantibodies and higher mortality, that are associated with some alleles of 8.1 ancestral haplotype.

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“…All patients with IPAF underwent chest HRCT, with thin-layer (1-mm) scanning, at 2 time points, at baseline and 1 year later. Since there is no current unified scoring standard for IPAF, the IPAF classification standard published by ATS/ERS in 2015 was used, with reference to the image evaluation methods for ILD in Ying et al, [ 26 ] Man et al, [ 27 ] Zou et al, [ 28 ] and Lee et al [ 29 ] Some of the morphological classification criteria for IPAF are the same as those for CTD-ILD; therefore, we tentatively used part of the CTD-ILD criteria (ground glass shadow, bronchial pull, nodules, and honeycombing in 2 CT sections of the diaphragm and aortic arch). For a more comprehensive assessment, we also combined this with the changes in lesion area and position (affected).…”

Section: Methodsmentioning

confidence: 99%

Krebs von den Lungen-6 and surfactant protein-A in interstitial pneumonia with autoimmune features

et al. 2021

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Interstitial pneumonia with autoimmune features (IPAF) is a special subtype of interstitial lung disease that has received worldwide attention. Krebs von den Lungen-6 (KL-6) and surfactant protein-A (SP-A) can be used as an important biomarker of interstitial lung disease, but its exact relationship with IPAF is poorly understood. A total of 65 IPAF patients were included in the study and were followed up for 52 weeks. The KL-6 and SP-A were evaluated by chemiluminescence enzyme immunoassay. The above indicators were tested at 2 time points, baseline (the first admission of patients) and 52 weeks. We also collected the indicators of antinuclear antibodies and rheumatoid factor. Based on high-resolution computed tomography evaluations, patients were divided into: aggravation, stable, and improvement group. At same time, 30 age-matched normal people as normal control were recruited, the same information was collected. Correlations among the groups were compared and analyzed. The KL-6 and SP-A level in IPAF patients were significantly higher than normal controls (fold increase = 11.35 and 1.39, both P < .001) and differed significantly at baseline and 52 weeks in IPAF (difference ratio = 37.7% and 21.3%, P < .05, both). There were significant differences at baseline and 52 weeks ( r values of aggravation, improvement, and stable groups for KL-6 were 0.705, 0.770, and 0.344, P = .001, .001, and .163, and for SP-A the r value were 0.672, 0.375, and 0.316, P = .001, .126, and .152). In aggravation group, KL-6 and SP-A were correlated with CT scores (both P < .05). Diffusing capacity of the lung for carbon monoxide (DLCO) and forced vital capacity (FVC), % predicted showed a progressive downward trend, with a significant difference at baseline and 52 weeks in IPAF patients (difference ratio = 23.8% and 20.6%, both P < .05). There was a significant correlation between KL-6 and FVC % predicted and DLCO (both P < .05), SP-A showed negatively correlated with DLCO, but not significantly correlated with FVC % predicted ( P < .05 and .47). This study demonstrated that KL-6 and SP-A can reflect disease progression, and both 2 play a key role at reflection of lung epithelial cell injury and fibrosis degree in IPAF.

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Interstitial Pneumonia With Autoimmune Features: Overview of Proposed Criteria and Recent Cohort Characterization

Cited by 12 publications

References 54 publications

Interstitial pneumonia with autoimmune features: from research classification to diagnosis

Interstitial pneumonia with autoimmune features: from research classification to diagnosis

A major genetic determinant of autoimmune diseases is associated with the presence of autoantibodies in hypersensitivity pneumonitis

Krebs von den Lungen-6 and surfactant protein-A in interstitial pneumonia with autoimmune features

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