Clinical significance of autoantibody positivity in idiopathic pulmonary fibrosis

Ghang, Byeongzu; Lee, Jung Sun; Kwon, Oh Chan; Ahn, Soo Min; Oh, Ji Seon; Hong, Sungeun; Yoo, Bin; Jeong, Wooseong; Kim, Jinseok; Lee, Chang‐Keun

doi:10.1016/j.rmed.2019.07.001

Cited by 17 publications

(19 citation statements)

References 22 publications

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“…The rate of autoantibody positivity in patients with IPF in this study was lower than that reported in other studies [12,13,22], but similar to that observed in healthy adults [6,23]. This study was performed at a tertiary university hospital with a medical team specialized in interstitial lung diseases, and only respiratory specialists were authorized to prescribe pirfenidone.…”

Section: Discussionsupporting

confidence: 79%

“…Autoantibody positivity has previously been reported in 23-41% of patients with IPF [9][10][11][12][13]. To date, little is known about the clinical implications of autoantibody positivity in IPF, and the reported results are somewhat controversial.…”

Section: Introductionmentioning

confidence: 99%

“…To date, little is known about the clinical implications of autoantibody positivity in IPF, and the reported results are somewhat controversial. Some studies reported that autoantibody positivity was associated with a better survival outcome [10,12], while others reported no survival difference between autoantibody-positive and autoantibody-negative patients [11,13]. One retrospective study reported that treating autoantibody-positive IPF with immunomodulators was associated with a superior survival outcome [12].…”

Section: Introductionmentioning

confidence: 99%

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Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study

et al. 2021

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Background Pirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF). However, its effectiveness in association with serological autoimmune features in IPF remains unclear. Methods We retrospectively reviewed the medical records of patients with IPF treated at a tertiary care hospital in South Korea. The autoantibody status was defined as positive if we detected autoantibodies meeting the serological domain criteria for interstitial pneumonia with autoimmune features or anti-neutrophil cytoplasmic antibodies. Results We included 142 patients with IPF treated with pirfenidone for over six months (93 were autoantibody-positive and 49 were autoantibody-negative). The mean age was 69.5 ± 7.3 years, and 77.5% of the patients were male. The adjusted mean changes over one year were − 34.4 and − 112.2 mL (p = 0.168) in forced vital capacity (FVC), and − 0.53 and − 0.72 mL/mmHg/min (p = 0.356) in the lungs diffusion capacity for carbon monoxide (DLCO) in the autoantibody-negative and autoantibody-positive groups, respectively. Conclusions Reductions in FVC and DLCO were similar in autoantibody-positive and autoantibody-negative patients with IPF treated with pirfenidone. Pirfenidone is effective in attenuating the progression of IPF, irrespective of the autoantibody status.

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Section: Discussionsupporting

confidence: 79%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study

et al. 2021

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“…Therefore, under conditions of ongoing pathology, pathology-specific autoAbs might show selective and measurable titer increases in the blood and can be used as prognostic indicators of disease [11]. Indeed, in chronic fibrosing IIPs, autoAbs were reported to correlate with disease severity and prognosis [21,22].…”

Section: Chronic Fibrosing Idiopathic Interstitial Pneumoniasmentioning

confidence: 99%

Natural Autoantibodies in Chronic Pulmonary Diseases

Fukushima

Tsujino

Futami

et al. 2020

IJMS

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In autoantibody-mediated autoimmune diseases, pathogenic autoantibodies generated by a failure of central or peripheral tolerance, have different effects mediated by a variety of mechanisms. Interestingly, even non-autoimmune chronic diseases have a set of disease-specific natural autoantibodies that are maintained for a long time. Because most of these natural autoantibodies target intracellular proteins or long non-coding RNAs, they are speculated to be non-pathological and have some important as yet unrecognized physiological functions such as debris clearance. Recently, we revealed a set of disease-specific natural autoantibodies of chronic pulmonary diseases with unknown etiology by protein arrays that enable detection of specific autoantibodies against >8000 targets. Surprisingly, some of the targeted antigens of disease-specific autoantibodies were subsequently reported by other laboratories as strongly associated with the disease, suggesting that these antigens reflect the pathology of each disease. Furthermore, some of these autoantibodies that target extracellular antigens might modify the original course of each disease. Here, we review the disease-specific natural autoantibodies of chronic pulmonary diseases, including chronic fibrosing idiopathic interstitial pneumonias, sarcoidosis, and autoimmune pulmonary alveolar proteinosis, and discuss their utility and effects.

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“…Previously, we identi ed that the autoantibody-positive IPF patients were associated with a good prognosis; moreover, the use of immunosuppressants was associated with a better prognosis in these patients as opposed to the autoantibody-negative patients. (13) At present, autoantibody-positive IPF cases have not been classi ed as interstitial pneumonitis with autoimmune features (IPAF), and thus, the use of immunosuppressants is not recommended. (14) The clinical characteristics of autoantibody-positive IPF are similar to those of CTD/UIP patients.…”

Section: Introductionmentioning

confidence: 99%

Subsequent Identification of a Connective Tissue Disease Amongst Patients with Idiopathic Pulmonary Fibrosis : A Long-term Observational Study in 527 Patients

Ghang

Nam

Lee

et al. 2020

Preprint

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Background: Connective tissue disease (CTD) might occur during the course of idiopathic pulmonary fibrosis (IPF). Clinical factors associated with CTD development in IPF patients have still not been identified. We investigated which antibodies have a significant association with the development of CTD during the clinical course of IPF.Methods: We retrospectively reviewed the records of 527 patients with a first diagnosis of IPF between January 2007 and March 2014 and investigated the time to CTD development after IPF diagnosis in these patients.Results: CTD developed in 15 patients at a median of 2.1 years (range 1.2–4.8) after IPF diagnosis. All patients had anti-neutrophil cytoplasmic antibodies (ANCA) or autoantibodies that met the serology criteria for interstitial pneumonitis with autoimmune features. Survival duration for IPF patients with progression to CTD was 5.3 (3.8, 6.7) years, which was significantly longer than for IPF patients without progression to CTD [2.9 (1.7, 4.8), p = 0.001]. Independent risk factors for CTD development in IPF patients included female gender [adjusted hazard ratio (HR) 5.319, p = 0.0082], titer of rheumatoid factor (RF; adjusted HR, 1.006; p = 0.022), titer of anti-citrullinated protein antibody (ACPA; adjusted HR, 1.009; p = 0.0011), and titer of myeloperoxidase (MPO)-ANCA (adjusted HR, 1.02; p < 0.0001).Conclusions: Progression to CTD is uncommon in IPF patients. However, a significant number of IPF patients with high titers of RF, ACPA, or MPO-ANCA progressed to CTD. RF, ACPA, and MPO-ANCA might be significantly associated with CTD development in IPF patients.

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Clinical significance of autoantibody positivity in idiopathic pulmonary fibrosis

Cited by 17 publications

References 22 publications

Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study

Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study

Natural Autoantibodies in Chronic Pulmonary Diseases

Subsequent Identification of a Connective Tissue Disease Amongst Patients with Idiopathic Pulmonary Fibrosis : A Long-term Observational Study in 527 Patients

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