Increased levels of VCAM-1 in sera and VLA-4 expression on neutrophils in dermatomyositis with interstitial lung disease

Abstract Background: Vascular cell adhesion molecule-1(VCAM-1) and its ligand very late antigen (VLA-4) play important roles in many autoimmune diseases. Our study aimed to investigate serum VCAM-1 level and VLA-4 expression on peripheral blood neutrophil surface in patients with dermatomyositis (DM), especially focusing on patients with interstitial lung disease(ILD). Methods: Blood specimens of 30 patients with DM and 30 healthy controls matched for age and gender were recruited. Total serum VCAM-1 level was measured using commercial enzyme-linked immunosorbent assay (ELISA) and the percentages of VLA-4 expression on the surface of neutrophils were analyzed by flow cytometry. We divided patients into subgroups according to whether they had ILD and whether they exhibited diffuse alveolar damage (DAD) via high-resolution computed tomography (HRCT).Results: Serum VCAM-1 levels were increased in DM patients compared with healthy controls (p<0.001). Patients with DM-ILD had higher serum VCAM-1 levels than those with none-ILD (p=0.015). The VCAM-1 levels were significantly increased in the DM-DAD group compared to the none-DAD group (p=0.002). The percentages of VLA-4 expression on neutrophils surface in DM patients were significantly elevated than that in healthy controls (p<0.001). The percentage of VLA-4 expression on neutrophils in DM patients with ILD were higher than none-ILD group(p=0.013). In the patients with ILD, DAD group had higher percentage of VLA-4 expression on neutrophils than none-DAD group (p=0.008).Conclusions: Our findings indicated that serum VCAM-1 level could be used as a potential serological biomarker for DM-ILD.

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Increased levels of HE4 (WFDC2) in systemic sclerosis: a novel biomarker reflecting interstitial lung disease severity?

Therapeutic Advances in Chronic Disease ◽

10.1177/2040622320956420 ◽

2020 ◽

Vol 11 ◽

pp. 204062232095642

Author(s):

Mingxia Zhang ◽

Liyun Zhang ◽

Linning E ◽

Ke Xu ◽

Xu Fei Wang ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Lavage Fluid ◽

Ct Scans ◽

Function Parameter ◽

Enzyme Linked Immunosorbent Assay ◽

Healthy Controls ◽

High Resolution Computed Tomography

Background: Human epididymis protein 4 (HE4, also known as WFDC-2) has been implicated in fibrotic disorders pathobiology. We tested the hypothesis that HE4 may be used as a candidate biomarker for systemic sclerosis (SSc)-related interstitial lung disease (SSc-ILD). Methods: A total of 169 consecutive SSc patients and 169 age-and sex-matched healthy controls were enrolled and blood samples were collected. Pulmonary function tests (PFTs) and paired lavage was performed on 169 patients and 37 healthy controls. All patients were classified as having SSc-no ILD or SSc-ILD, based on high-resolution computed tomography (CT) scans of the chest, and a semiquantitative grade of ILD extent was evaluated through CT scans (grade 1, 0–25%; grade 2, 26–50%; grade 3, 51–75%; grade 4, 76–100%). Serum and bronchoalveolar lavage fluid (BALF) HE4 levels were measured by enzyme-linked immunosorbent assay. Results: Serum HE4 levels were higher in SSc patients [median (interquartile range), 139.4 (85.9–181.8) pmol/l] compared with healthy controls [39.5 (24.3–54.2) pmol/l, p < 0.001] and were higher in patients with SSc-ILD [172.1 (94.8–263.3) pmol/l] than in those with SSc-no ILD [97.4 (85.5–156.5) pmol/l, p < 0.001]. This observation was replicated in the BALF samples. Corresponding values were 510.8 (144.6–1013.8) pmol/l for SSc cohort, 754.4 (299–1060) pmol/l for SSc-ILD, 555.1 (203.7–776.2) pmol/l for SSc-no ILD, and 238.7 (97.7–397.6) pmol/l for controls. The semiquantitative grade of ILD on CT scan was significantly proportional to the HE4 levels and the lung function parameter (i.e., FVC) had a negative correlation with the HE4 levels. Conclusion: This is the first study to demonstrate the potential clinical utility of blood and BALF HE4 as a biomarker for SSc-ILD. Future prospective validation studies are warranted.

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Serum levels of interleukins and S100A8/A9 correlate with clinical severity in patients with dermatomyositis-associated interstitial lung disease

10.21203/rs.3.rs-15869/v1 ◽

2020 ◽

Author(s):

Yueyan Lou ◽

yu zheng ◽

Xiaoming Tan ◽

Bijun Fan ◽

Liyan Zhang ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Serum Levels ◽

Clinical Severity ◽

Enzyme Linked Immunosorbent Assay ◽

Inflammatory Disorder ◽

Healthy Controls ◽

Serum Samples ◽

High Resolution Computed Tomography ◽

Highly Correlated

Abstract Background: Dermatomyositis (DM) is a systemic autoimmune inflammatory disorder that affects primarily skin, muscle and lung, frequently associated with interstitial lung disease (ILD). The objective of this study is to investigate the association between serum cytokines and clinical severity in patients with DM-ILD. Methods: Serum samples of 40 DM-ILD patients and 30 healthy controls were collected. Expressions of S100A8/A9 were analyzed by enzyme-linked immunosorbent assay (ELISA) and interleukins were analyzed by cytometric beads array (CBA). Results: Serum IL-4, IL-6 and S100A8/A9 were observably higher in DM-ILD than those in healthy controls ( p = 0.0013, 0.0017 and < 0.0001, respectively). Serum IL-10 level of patients was dramatically lower than that in controls ( p = 0.0001). IL-4 ( r = 0.1171, p = 0.0040), IL-6 ( r = 0.1174, p = 0.0040) and IL-10 ( r = -0.1829, p = 0.0003) were significantly correlated with S100A8/A9 in DM-ILD patients. S100A8/A9 was significantly correlated with high-resolution computed tomography (HRCT) ( r = 0.1642, p = 0.0157) and lung function (DLCO%: r = -0.2066, p = 0.0061, FVC%: r = -0.2156, p = 0.0050). Conclusions: Serum level of S100A8/A9 may be a valuable marker for assessing the clinical severity of DM-ILD patients. Serum IL-4, IL-6 and IL-10 levels were highly correlated with S100A8/A9, so these cytokines may play a synergistic effect on the progression of DM-ILD. Keywords : Dermatomyositis, Interstitial lung disease, S100A8/A9, Interleukin

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The value of serum Krebs von den lungen-6 as a diagnostic marker in connective tissue disease associated with interstitial lung disease

BMC Pulmonary Medicine ◽

10.1186/s12890-019-1043-z ◽

2020 ◽

Vol 20 (1) ◽

Cited By ~ 1

Author(s):

Hua Ma ◽

Junhui Lu ◽

Yuanyuan Song ◽

Huixuan Wang ◽

Songlou Yin

Keyword(s):

Interstitial Lung Disease ◽

Connective Tissue ◽

Lung Disease ◽

Connective Tissue Disease ◽

Diagnostic Marker ◽

Pulse Therapy ◽

Enzyme Linked Immunosorbent Assay ◽

High Resolution Computed Tomography ◽

The Difference ◽

Diagnostic Indicator

Abstract Objectives The purpose of this study was to evaluate the value of serum krebs von den lungen-6 (KL-6) level as a diagnostic indicator for connective tissue disease associated with interstitial lung disease (CTD-ILD). Methods One hundred fifty five patients with newly diagnosed CTD in our hospital were enrolled and divided into two groups by their ILD manifestations, the CTD-ILD group and the CTD group. In parallel, 61 patients with pulmonary infection and 60 cases of healthy subjects were also enrolled into the study. The difference of serum KL-6 level among the four groups were compared. In CTD-ILD group, carbon monoxide diffusing capacity (DLCo) and high-resolution computed tomography (HRCT) of lung were also tested. The serum KL-6 level of 32 patients from the CTD-ILD group who received cyclophosphamide (CTX) pulse therapy were sampled and measured, by enzyme linked immunosorbent assay (ELISA), at three time points: before treatment, 3 months after treatment and 6 months after treatment. Results The serum KL-6 level in the CTD-ILD group (1004.9 (676.41738.1) IU/ml) is significantly higher than three other groups (χ2 = 72.29, P < 0.001). In the CTD-ILD group the level of serum KL-6 was positively correlated with disease severity on HRCT (r = 0.75, P < 0.001), while was negatively correlated with DLCo (r = − 0.50, P < 0.001). In 32 patients who received CTX pulse therapy, the level of serum KL-6 was gradually decreased in 20 cases whose lesions were absorbed within 6 months (F = 13.67, P < 0.001), whereas it remained unchanged in the rest of 12 patients (Z = -1.328, P = 0.198). Conclusions Serum KL-6 level can potentially serve as a diagnostic marker for CTD-ILD and be utilized to evaluate the effectiveness of CTX pulse therapy.

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Serum Biomarkers in Differential Diagnosis of Idiopathic Pulmonary Fibrosis and Connective Tissue Disease-Associated Interstitial Lung Disease

Journal of Clinical Medicine ◽

10.3390/jcm10143167 ◽

2021 ◽

Vol 10 (14) ◽

pp. 3167

Author(s):

Eva Cabrera Cesar ◽

Lidia Lopez-Lopez ◽

Estrella Lara ◽

M. Victoria Hidalgo-San Juan ◽

Concepcion Parrado Romero ◽

...

Keyword(s):

Differential Diagnosis ◽

Interstitial Lung Disease ◽

Idiopathic Pulmonary Fibrosis ◽

Connective Tissue ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Connective Tissue Disease ◽

Serum Levels ◽

Enzyme Linked Immunosorbent Assay ◽

Healthy Controls

Introduction: The goal of this study is to determine whether Advanced glycosylated end-products (AGE), Advanced oxidation protein products (AOPP) and Matrix metalloproteinase 7 (MMP7) could be used as differential biomarkers for idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated interstitial lung disease (CTD-ILD). Method: Seventy-three patients were enrolled: 29 with IPF, 14 with CTD-ILD, and 30 healthy controls. The study included a single visit by participants. A blood sample was drawn and serum was analysed for AGE using spectrofluorimetry, AOPP by spectrophotometry, and MMP7 using sandwich-type enzyme-linked immunosorbent assay. Results: AGE, AOPP and MMP7 serum levels were significantly higher in both IPF and CTD-ILD patients versus healthy controls; and AGE was also significantly elevated in CTD-ILD compared to the IPF group. AGE plasma levels clearly distinguished CTD-ILD patients from healthy participants (AUC = 0.95; 95% IC 0.86–1), whereas in IPF patients, the distinction was moderate (AUC = 0.78; 95% IC 0.60–0.97). Conclusion: In summary, our results provide support for the potential value of serum AGE, AOPP and MMP7 concentrations as diagnostic biomarkers in IPF and CTD-ILD to differentiate between ILD patients and healthy controls. Furthermore, this study provides evidence, for the first time, for the possible use of AGE as a differential diagnostic biomarker to distinguish between IPF and CTD-ILD. The value of these biomarkers as additional tools in a multidisciplinary approach to IPF and CTD-ILD diagnosis needs to be considered and further explored. Multicentre studies are necessary to understand the role of AGE in differential diagnosis.

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Interstitial lung disease pathology in systemic sclerosis

Therapeutic Advances in Musculoskeletal Disease ◽

10.1177/1759720x211032437 ◽

2021 ◽

Vol 13 ◽

pp. 1759720X2110324

Author(s):

Kristine E. Konopka ◽

Jeffrey L. Myers

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Interstitial Pneumonia ◽

Diffuse Alveolar Damage ◽

Usual Interstitial Pneumonia ◽

High Resolution Computed Tomography ◽

Restrictive Lung Disease ◽

Diffuse Parenchymal Lung Disease ◽

Lung Biopsies

Interstitial lung disease is a relatively frequent manifestation of systemic sclerosis with approximately one-third of patients developing clinical restrictive lung disease. Fibrotic nonspecific interstitial pneumonia is the most common cause of diffuse parenchymal lung disease in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), followed by usual interstitial pneumonia (UIP). Radiographic pleuroparenchymal fibroelastosis-like changes may accompany other forms of interstitial lung disease, most commonly UIP. In an appropriate clinical setting with supportive high-resolution computed tomography findings, lung biopsy is not needed to confirm the presence of interstitial lung disease and surgical lung biopsies are often reserved for atypical presentations. In this review, we discuss the histological findings that define the most common patterns of SSc-ILD and outline other findings sometimes encountered in lung biopsies obtained from systemic sclerosis patients, including pulmonary vascular changes, aspiration, chronic pleuritis, and diffuse alveolar damage.

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Aberrantly Expressed Galectin-9 is Involved in the Immunopathogenesis of anti-MDA5-Positive Dermatomyositis-Associated Interstitial Lung Disease

10.21203/rs.3.rs-100255/v1 ◽

2020 ◽

Author(s):

Lin Liang ◽

Ya-Mei Zhang ◽

Ya-Wen Shen ◽

Ai-Ping Song ◽

Wen-Li Li ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Disease Activity ◽

Lung Disease ◽

Enzyme Linked Immunosorbent Assay ◽

Type I ◽

Mrna Levels ◽

Healthy Controls ◽

Necrotizing Myopathy ◽

Immune Mediated

Abstract Background: Rapidly progressive interstitial lung disease (RP-ILD) has high mortality rate and poor prognosis. Galectin-9 (Gal-9) plays multiple functions in immune regulation. We investigated Gal-9 expression in patients with dermatomyositis (DM) and the impact of Gal-9 on the development of DM-ILD. Methods: Enzyme-linked immunosorbent assay and qRT-PCR were used to examine Gal-9 expression in the sera and isolated peripheral blood mononuclear cells (PBMCs) from patients with DM. Immunohistochemistry was performed to analyze the expression of Gal-9 and its ligand (T-cell immunoglobulin mucin (Tim)-3 and CD44) in lung tissues from patients who were positive for anti-melanoma differentiation-associated gene 5 (MDA5). The effect of Gal-9 on human lung fibroblasts (MRC-5) was also investigated in vitro. Results: Serum Gal-9 levels were significantly higher in patients with DM than in those with immune-mediated necrotizing myopathy and healthy controls (p < 0.001). Higher levels of serum Gal-9 were observed in anti-MDA5-positive patients with DM than in anti-MDA5-negative patients with DM (33.8 (21.9–44.7) vs 16.2 (10.0–26.9) ng/mL, p < 0.001). Among the anti-MDA5-positive patients with DM, serum Gal-9 levels were associated with ILD severity. Serum Gal-9 levels were significantly correlated with disease activity in anti-MDA5-positive patients with DM in both cross-sectional and longitudinal studies. PBMCs isolated from anti-MDA5-positive patients with DM (3.7 ± 2.3 ng/mL) produced higher levels of Gal-9 than those from patients with immune-mediated necrotizing myopathy (1.1 ± 0.3 ng/mL, p = 0.022) and healthy controls (1.4 ± 1.2 ng/mL, p = 0.045). The mRNA levels of Gal-9 were positively correlated with levels of type-I interferon-inducible genes MX1 (r = 0.659, p = 0.020) and IFIH1 (r = 0.787, p = 0.002) in PBMCs from anti-MDA5-positive patients with DM. Immunohistochemistry revealed increased Gal-9 and Tim-3 expression in the lung tissues of patients with DM and RP-ILD. In vitro stimulation with Gal-9 protein increased CCL2 mRNA expression in MRC-5 fibroblasts.Conclusions: Among anti-MDA5-positive patients with DM, Gal-9 could be a promising biomarker for monitoring disease activity, particularly for RP-ILD severity. Aberrant expression of the Gal-9/Tim-3 axis may be involved in the immunopathogenesis of DM-ILD.

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Soluble Programmed Death Molecule 1 (sPD-1) As Predictor of Interstitial Lung Disease in Rheumatoid Arthritis

10.21203/rs.3.rs-587880/v1 ◽

2021 ◽

Author(s):

Li Xu ◽

Lichun Jiang ◽

Liuyan Nie ◽

Songzhao Zhang ◽

Lie Liu ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Logistic Regression ◽

Regression Analysis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Logistic Regression Analysis ◽

Multivariate Logistic Regression Analysis ◽

Enzyme Linked Immunosorbent Assay ◽

Healthy Controls ◽

Programmed Death

Abstract Objective To determine the relationship between serum soluble programmed death molecule-1 (sPD-1) levels and the development of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA). Methods Blood samples were obtained from 87 patients with RA (58 with ILD and 29 without ILD) and 45 healthy controls. Serum sPD-1 was measured by enzyme-linked immunosorbent assay. The pulmonary interstitial disease score was completed by a pulmonary physician and a radiologist through chest high-resolution computed tomography. Patients with RA-ILD were tested for lung function [e.g., forced vital capacity (FVC%), diffusing capacity of lungs for carbon monoxide (DLCO%)]. Associations between ILD and various markers, including sPD-1 and confounding factors, were investigated by logistic regression analysis. Diagnostic values of sPD-1 for the presence of ILD were investigated using receiver operating characteristic curve analysis. Results Serum sPD-1 levels were higher in RA patients with ILD than in RA patients without ILD and healthy controls (185.1 ± 109.0 pg/ml vs. 119.1 ± 77.5 pg/ml vs. 52.1 ± 21.7 pg/ml, P < 0.05). Serum sPD-1 levels were positively correlated with RF titer (P = 0.02, r = 0.249), anti-cyclic citrullinated peptide antibody status (P = 0.02, r = 0.243), and serum IgG levels (P < 0.001, r = 0.368), negatively associated with FVC% (P = 0.02, r = − 0.344), forced expiratory volume (FEV1%) (P = 0.01, r = − 0.354), total lung capacity % (P = 0.046, r = − 0.302), and was independently associated with the presence of ILD in RA patients by multivariate logistic regression analysis. The sensitivity and specificity of sPD-1 levels for the detection of ILD in RA patients were 58.6% and 75.9%, respectively. The area under the curve was 0.689. Conclusion Serum sPD-1 levels were increased in RA patients with ILD. Increased sPD-1 may be a valuable biomarker to predict the presence of ILD in patients with RA.

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Soluble programmed death molecule 1 (sPD-1) as a predictor of interstitial lung disease in rheumatoid arthritis

BMC Immunology ◽

10.1186/s12865-021-00460-6 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Li Xu ◽

Lichun Jiang ◽

Liuyan Nie ◽

Songzhao Zhang ◽

Lei Liu ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Logistic Regression ◽

Regression Analysis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Logistic Regression Analysis ◽

Multivariate Logistic Regression Analysis ◽

Enzyme Linked Immunosorbent Assay ◽

Healthy Controls ◽

Programmed Death

Abstract Background Previous studies have indicated that the programmed death molecule 1 (PD-1) signaling pathway may play a key role in rheumatoid arthritis (RA). However, the pathogenesis of rheumatoid arthritis-related interstitial lung disease (RA-ILD) is not clear. We examined the serum levels of soluble PD-1 in patients with RA and its relationship with RA-ILD. Methods Blood samples were obtained from 87 patients with RA (58 with ILD and 29 without ILD) and 45 healthy controls. Serum sPD-1 was measured by Enzyme-Linked Immunosorbent Assay. The pulmonary interstitial disease score was completed by a pulmonary physician and a radiologist through chest high-resolution computed tomography. Patients with RA-ILD were tested for lung function [e.g., forced vital capacity (FVC%), diffusing capacity of lungs for carbon monoxide (DLCO%)]. Associations between ILD and various markers, including sPD-1 and confounding factors, were investigated by logistic regression analysis. Diagnostic values of sPD-1 for the presence of ILD were investigated using receiver operating characteristic curve analysis. Results Serum sPD-1 levels were higher in RA patients with ILD than in RA patients without ILD and healthy controls (185.1 ± 109.0 pg/ml vs. 119.1 ± 77.5 pg/ml vs. 52.1 ± 21.7 pg/ml, P < 0.05). Serum sPD-1 levels were positively correlated with RF titer (P = 0.02, r = 0.249), anti-cyclic citrullinated peptide antibody status (P = 0.02, r = 0.243), and serum IgG levels (P < 0.001, r = 0.368), negatively associated with FVC% (P = 0.02, r = − 0.344), forced expiratory volume (FEV1%) (P = 0.01, r = − 0.354), total lung capacity (TLC%) (P = 0.046, r = − 0.302), and was independently associated with the presence of ILD in RA patients by multivariate logistic regression analysis. The sensitivity and specificity of sPD-1 levels for the detection of ILD in RA patients were 58.6% and 75.9%, respectively. The area under the curve was 0.689. Conclusion Serum sPD-1 levels were increased in RA patients with ILD. Increased sPD-1 may be a valuable biomarker to predict the presence of ILD in patients with RA.

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AB0605 CLINICAL PROFILE AND CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY (HRCT) FINDINGS IN PATIENTS WITH CONNECTIVE TISSUE DISEASES AND INTERSTITIAL LUNG DISEASE: EXPERIENCE OF A SINGLE REFERENCE RHEUMATOLOGY CENTER

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.3758 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1598.2-1599

Author(s):

I. Rusu ◽

L. Muntean ◽

M. M. Tamas ◽

I. Felea ◽

L. Damian ◽

...

Keyword(s):

Computed Tomography ◽

Systemic Sclerosis ◽

High Resolution ◽

Interstitial Lung Disease ◽

Connective Tissue ◽

Lung Disease ◽

Interstitial Pneumonia ◽

Connective Tissue Diseases ◽

High Resolution Computed Tomography ◽

Hrct Patterns

Background:Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs), and is associated with significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) play an important role in the diagnosis of ILD and may provide prognostic information.Objectives:We aimed to characterize the clinical profile and chest HRCT abnormalities and patterns of patients diagnosed with CTDs and ILD.Methods:In this retrospective, observational study we included 80 consecutive patients with CTDs and ILD referred to a tertiary rheumatology center between 2015 and 2019. From hospital charts we collected clinical data, immunologic profile, chest HRCT findings. HRCT patterns were defined according to new international recommendations.Results:Out of 80 patients, 64 (80%) were women, with a mean age of 55 years old. The most common CTD associated with ILD was systemic sclerosis (38.8%), followed by polymyositis (22.5%) and rheumatoid arthritis (18.8%). The majority of patients had dyspnea on exertion (71.3%), bibasilar inspiratory crackles were present in 56.3% patients and 10% had clubbing fingers. Antinuclear antibodies (ANA) were present in 78.8% patients, and the most frequently detected autoantibodies against extractable nuclear antigen were anti-Scl 70 (28.8%), followed by anti-SSA (anti-Ro, 17.5%), anti-Ro52 (11.3%) and anti-Jo (7.5%). Intravenous cyclophosphamide therapy for 6-12 months was used in 35% of patients, while 5% of patients were treated with mycophenolate mofetil.The most frequent HRCT abnormalities were reticular abnormalities and ground glass opacity. Non-specific interstitial pneumonia (NSIP) was identified in 46.3% CTDs patients. A pattern suggestive of usual interstitial pneumonia (UIP) was present in 32.5% patients, mainly in patients with systemic sclerosis. In 21.3% patients the HRCT showed reticulo-nodular pattern, micronodules and other abnormalities, not diagnostic for UIP or NSIP pattern.Conclusion:Nonspecific interstitial pneumonia (NSIP) is the most common HRCT pattern associated with CTDs. Further prospective longitudinal studies are needed in order to determine the clinical and prognostic significance of various HRCT patterns encountered in CTD-associated ILD and for better patient management.References:[1]Ohno Y, Koyama H, Yoshikaua T, Seki S. State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 2015;17(12):69.[2]Walsh SLF, Devaraj A, Enghelmeyer JI, Kishi K, Silva RS, Patel N, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150)Disclosure of Interests:None declared

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Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association

Journal of Clinical Medicine ◽

10.3390/jcm10122548 ◽

2021 ◽

Vol 10 (12) ◽

pp. 2548

Author(s):

Marco Sebastiani ◽

Fabrizio Luppi ◽

Gianluca Sambataro ◽

Diego Castillo Villegas ◽

Stefania Cerri ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Rheumatic Disease ◽

Lung Disease ◽

Interstitial Pneumonia ◽

Usual Interstitial Pneumonia ◽

Clinical History ◽

High Resolution Computed Tomography ◽

Function Testing ◽

And Diffusion

Anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (MPO) antibodies, have been frequently identified in patients with idiopathic pulmonary fibrosis (IPF). However, their role remains unclear, and only 7–23% of these patients develops clinically overt vasculitis. We aimed to investigate the clinical, serological, and radiological features and prognosis of anti-MPO-positive interstitial lung disease (ILD) patients. Fifty-eight consecutive patients firstly referred for idiopathic interstitial pneumonia and showing serological positivity of anti-MPO antibodies were retrospectively enrolled. For each patient, clinical data, lung function testing, chest high-resolution computed tomography (HRCT) pattern, and survival were recorded. Thirteen patients developed a rheumatic disease during a median follow-up of 39 months. Usual interstitial pneumonia (UIP) was the most frequent ILD pattern, significantly influencing the patients’ survival. In fact, while the 52-week survival of the overall population was 71.4 ± 7.5%, significantly higher than IPF, survivals of anti-MPO patients with UIP pattern and IPF were similar. Forced vital capacity and diffusion lung capacity for CO significantly declined in 37.7 and 41.5% of cases, respectively, while disease progression at chest HRCT was observed in 45.2%. A careful clinical history and evaluation should always be performed in ILD patients with anti-MPO antibodies to quickly identify patients who are developing a systemic rheumatic disease.

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