Characteristics Of Interstitial Lung Disease Research Articles

Clinical and Functional Characteristics of Interstitial Lung Disease in Algeria: A Single-Center Prospective Study

Introduction: There are a limited number of epidemiological studies describing the global burden of chronic diffuse interstitial lung diseases (ILD) and their subtypes’ heterogeneity worldwide. Our main is to characterize new-onset ILDs in Algeria and compare our results with data from other populations. Materials and Methods: Newly diagnosed ILDs were prospectively collected in a single-center observational cohort study including all patients diagnosed as ILDs in the pulmonology, phthisiology, and allergology departments between 2015 and 2019. Detailed anamnestic and clinical data were collected at the time of diagnosis. The results of high-resolution computed tomography (HRCT), serological tests, biology data, and respiratory functional exploration were systematically performed and collected. Results: A total of 455 cases were included. The mean age was 59.4 ± 13.2 years. There was a slight predominance of females (300; 65.9%). The most common disease was ILD secondary to connective tissue disease (CTD) or ILD-CTD (48.1%), followed by idiopathic interstitial pneumonias (IIPs) (23.5%), sarcoidosis (16.9%), interstitial pneumonia with autoimmune features (IPAF) (12.1%), and hypersensitivity pneumonitis (HP) (2.4%). Idiopathic pulmonary fibrosis (IPF) was present in 8.6% and unclassifiable ILD in 4.6% of the total ILD cases. Conclusions: ILD-CTD, IIP, and sarcoidosis were the most frequently observed ILDs in this Algerian population. Similarities and many differences were found compared to previous data from other countries.

Epidemiology and clinical characteristics of interstitial lung disease in patients with rheumatoid arthritis from the JointMan database

Interstitial lung disease (ILD) is a progressive fibrotic disease associated with rheumatoid arthritis (RA); real-world data for evaluating RA–associated ILD (RA–ILD) are limited. We evaluated prevalence, time to onset, clinical characteristics and prognostic factors in patients diagnosed with RA (n = 8963) in the Discus Analytics JointMan database (2009–2019) with and without ILD. ILD prevalence was 4.1% (95% confidence interval 3.7–4.5); > 90% had an ILD diagnosis after RA diagnosis (mean time to onset 3.3 years). At baseline, a higher proportion of patients with RA–ILD were older (> 65 years), male, with history of chronic obstructive pulmonary disease (COPD) compared with patients in the RA cohort. Patients in the RA–ILD cohort were likely to have more severe RA characteristics and joint evaluation compared with patients without ILD, at baseline and before/after ILD diagnosis. In this large, real-world database patients with (vs without) ILD had a higher burden of RA characteristics. Previously established risk factors for RA–ILD were confirmed (age, baseline COPD, anti-cyclic citrullinated peptide positivity, C-reactive protein, Clinical Disease Activity Index score); thus, recognition of these factors and tracking routine disease activity metrics may help identify patients at higher risk of RA complications and lead to improved diagnosis and earlier treatment.

Retrospective analysis and a cross-sectional questionnaire survey of lung cancer concomitant with interstitial lung disease

Objective: To describe the clinical characteristics and prognosis of lung cancer concomitant with interstitial lung disease (LC-ILD), and to understand the current status of knowledge of LC-ILD by physicians in the departments related to the treatment of the disease. Methods: We conducted a retrospective analysis of in-hospitalized pathology identified lung cancer (LC) patients who were admitted to our hospital between January 2014 and December 2018. After reviewing their chest CT imagings and pathological reports, 70 patients who were concomitant with interstitial lung disease (ILD) were enrolled in our study. On the other hand, a cross-sectional survey using an online questionnaire was conducted in LC-ILD management doctors who came from 29 provincial hospitals. The perceptions of demographic features, LC characteristics and management, ILD characteristics and management, and the prognosis of LC-ILD were investigated. Results: Among the 70 enrolled LC-ILD cases, there were 52 males, and the mean age was (64.3±7.63) years (ranged from 49 years to 84 years). There were 51 patients who were older than 59 years. The most common pathological pattern of LC was adenocarcinoma. Most of them were diagnosed with LC and ILD simultaneously, and they were usually treated with chemotherapy while unresectable. There were 11 patients (15.7%) with positive EGFR or ALK mutation. Forty-five patients (64.3%) died during the follow-up, and 33 were died from LC progression. There were no significant differences between the surgical group and non-surgical group on age, pathological patterns, EGFR or ALK mutation. However, LC-ILD patients in the surgical group were diagnosed with earlier TNM classification and with better prognosis. A total of 1 014 doctors answered the questionnaire completely. In the feedback, patients aged 60 years and older (785 doctors/77.4%), and male patients (720 doctors/71%) were the predominant LC-ILD patients. Adenocarcinoma (390 doctors/38.5%), adenocarcinoma or squamous-cell cancer (SCC) (182 doctors/17.9%), and SCC (151 doctors/14.9%) were considered as the common pathological patterns of LC-ILD patients. In most doctors' feedback, the EGFR or ALK mutation was not common for LC-ILD: low (646 doctors/63.7%) or hardly (306 doctors/30.5%) positive mutation. The diagnosis of ILD was earlier than LC (506 doctors/49.9%) or there was no identified precedence of LC and ILD diagnosis (208 doctors/20.5%). Most of the doctors (693 doctors/68.3%) agreed that the vital factor for surgery or not was the severity of ILD for LC-ILD patients. There were great divergences on the treatment protocol both for the advanced LC and ILD. The patients with LC-ILD were died mostly from LC progression and ILD exacerbation (542 doctors/53.5%), followed by ILD exacerbation (237 doctors/23.4%) or LC progression (226 doctors/22.3%). Conclusions: The elderly male patients were predisposed to LC-ILD, and adenocarcinoma was the common pathological pattern. The LC-ILD patients with non-advanced LC who were performed with surgery had better prognosis. However, it is recommended to consider whether to perform surgery in combination with the severity of the ILD.

Idiopathic inflammatory myopathies: CT characteristics of interstitial lung disease and their association(s) with myositis-specific autoantibodies.

Interstitial lung disease (ILD), one of the most common extramuscular manifestations of idiopathic inflammatory myopathies (IIMs), carries a poor prognosis. Myositis-specific autoantibody (MSA)-positivity is a key finding for IIM diagnosis. We aimed to identify IIM-associated lung patterns, evaluate potential CT-ILD finding-MSA relationships, and assess intra- and interobserver reproducibility in a large IIM population. All consecutive IIM patients (2003-2019) were included. Two chest radiologists retrospectively assessed all chest CT scans. Multiple correspondence and hierarchical cluster analyses of CT findings identified and characterized ILD-patient subgroups. Classification and regression-tree analyses highlighted CT-scan variables predicting three patterns. Three independent radiologists read CT scans twice to assign patients according to CT-ILD-pattern clusters. Among 257 IIM patients, 94 (36.6%) had ILDs; 87 (93%) of them were MSA-positive. ILD-IIM distribution was 54 (57%) ASyS, 21 (22%) DM, 15 (16%) IMNM, and 4 (4%) IBM. Cluster analysis identified three ILD-patient subgroups. Consolidation characterized cluster 1, with significantly (p < 0.05) more frequent anti-MDA5-autoantibody-positivity. Significantly more cluster-2 patients had a reticular pattern, without cysts and with few consolidations. All cluster-3 patients had cysts and anti-PL12 autoantibodies. Clusters 2 and 3 included significantly more ASyS patients. Intraobserver concordances to classify patients into those three clusters were good-to-excellent (Cohen κ 0.64-0.81), with good interobserver reliability (Fleiss's κ 0.56). Despite the observed IIM heterogeneity, CT-scan criteria enabled ILD assignment to the three clusters, which were associated with MSAs. Radiologist identification of those clusters could facilitate diagnostic screening and therapeutics. Interstitial lung disease in patients with idiopathic inflammatory myopathy could be classified into three clusters according to CT-scan criteria, and these clusters were significantly associated with myositis-specific autoantibodies. • Cluster analysis discerned three homogeneous groups of interstitial lung disease (ILD) for which cysts, consolidations, and reticular pattern were discriminatory, and associated with myositis-specific autoantibodies. • Like muscle- and extramuscular-specific phenotypes, myositis-specific autoantibodies are also associated with specific ILD patterns in patients with idiopathic inflammatory myopathies.

Interstitial lung disease is not rare in immune-mediated necrotizing myopathy with anti-signal recognition particle antibodies

ObjectivesThe purpose was to clarify the characteristics of interstitial lung disease (ILD) in immune-mediated necrotizing myopathy (IMNM) patients with anti-signal recognition particle (SRP) antibodies.MethodsMedical records of IMNM patients with anti-SRP antibodies were reviewed retrospectively.ResultsA total of 60 patients were identified. Twenty-seven (45.0%) patients were diagnosed with ILD based on lung imaging: nonspecific interstitial pneumonia (NSIP) in 17 patients (63.0%) and organizing pneumonia in 9 patients (33.3%). Reticulation pattern was identified in 17 patients (63.0%) whereas 10 cases (37.0%) showed ground glass opacity and patchy shadows by high-resolution computed tomography (HRCT). Pulmonary function tests (PFTs) were available in 18 patients, 6 (33.3%) and 10 (55.6%) patients were included in the mild and moderate group, respectively. The average age at the time of ILD onset was significantly older than those without ILD (48.6 ± 14.4 years vs. 41.2 ± 15.4 years, p < 0.05), and the frequency of dysphagia in the ILD group was higher than the group without ILD (p < 0.05). Long-term follow-up was available on 9 patients. PFTs were stable in 8 (88.9%), and the HRCT remained stable in 6 (66.7%) patients.ConclusionsILD is not rare in IMNM patients with anti-SRP antibodies, most being characterized as mild to moderate in severity. NSIP is the principal radiologic pattern, and ILD typically remains stable following treatment.

Characteristics of interstitial lung disease in patients from post-marketing data on metastatic breast cancer patients who received abemaciclib in Japan

BackgroundThis study evaluated characteristics of patients treated with abemaciclib and diagnosed with interstitial lung disease (ILD), using 12-month post-marketing data from the real-world setting in Japan.MethodsSpontaneous reports of adverse events in patients receiving abemaciclib were collected regularly from healthcare providers (HCPs) from November 30, 2018, to November 29, 2019. Detailed follow-up was requested on suspected ILD cases via questionnaires and/or interviews. Radiological images (when available) were reviewed by an ILD adjudication committee of specialists. The age distribution of patients prescribed abemaciclib in Japan was estimated based on insurance claims data.ResultsOf 4700 patients estimated to be exposed to abemaciclib, 82 cases of ILD were reported (46 serious, 13 fatal). Most (91%) had ≥ 1 symptom at diagnosis, commonly dyspnea/shortness of breath (59%), cough (44%), and/or fever (37%). The majority (68%) received steroid therapy (24 [56%] recovered/recovering; 5 [12%] not recovered; 13 [30%] deaths, 1 [2.3%] unknown). No specific imaging patterns or sites of predilection were identified, but a diffuse alveolar damage (DAD) pattern was observed at outcome in 3 of 4 evaluated fatal cases (16 in total evaluated). Features of fatal cases included advanced age, pre-existing interstitial change, and advanced Eastern Cooperative Oncology Group Performance Status.ConclusionAdvanced age and a DAD pattern were identified as potential risk factors for cases with poorer outcomes, as previously reported for drug-induced ILD. HCPs should consider the benefit–risk profile when prescribing abemaciclib, informing patients of risks and regularly monitoring treated patients to ensure early detection and treatment of ILD.

NATIONAL REGISTRY OF INTERSTITIAL LUNG DISEASE FROM PAKISTAN

SESSION TITLE: Diffuse Lung Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: The aim of this study was to determine the epidemiology and characteristics of interstitial lung disease from Pakistan. METHODS: This web-based registry which is the first multicenter registry of ILD from Pakistan recruited patients from 10 centers of 5 major cities between January 2016 and March 2019.The ILD diagnosis was based on high-resolution computed tomography (HRCT) and histopathology in cases where biopsy was performed. The opinion of rheumatologist was also sought in cases of CTD related ILDs. The purpose of this multidisciplinary discussion (MDD) resource group was to maintain uniformity in the diagnostic criteria. RESULTS: A total of 744 patients were enrolled in the registry. The final ILD diagnoses were as follows: IPF 256 (34.4%), HP 132 (17.7%), iNSIP 125 (16.8%), CTD-ILD 121 (16.3%), sarcoidosis 68 (9.1%) and 42 (5.7%) were other ILDs. The median age was 57.5 years with interquartile range 46–65 years and gender was predominantly female (65.6%). A mean age greater than 60 years and history of smoking was most significant in IPF patients (P<0.001). The female gender was significant in all ILD's except IPF (P<0.001). The most common clinical symptoms were cough in 674 (90.6%) and dyspnea in 519 (69.75%). On physical examination, crepitations were audible in 674 (90.6%) and rhonchi in 83 (11.2%) while clubbing was seen in 247 (33.2%) which were mostly IPF. Gastroesophageal reflux disease (GERD) was present in 195 (26.2%) patients and was statistically significant with iNSIP and IPF (P=0.021). Echocardiography was done in 432 (58.1%) patients, of which 206 (47.7%) showed pulmonary arterial hypertension (PAH); 44.7% had mild PAH, 30.1% had moderate PAH and 25.2% had severe PAH. The overall predicted forced vital capacity (FVC) was 58.2 ±18.7%. FVC % of predicted was presented as box-and-whiskers plot showing the median, interquartile range and outliers (if any) for ILD patients. The median FVC for IPF was 59 (range: 25-99.99), for iNSIP was 54 (range: 25-99.99), for CTD-ILD was 60.5 (range: 26-99.99), for sarcoidosis it was 67 (range: 26-99.99). The HP patients had the lowest FVC % of predicted median that was 45.5 (range: 25-82) where four cases had ≥85 FVC and considered as outliers (Figure 2). An avian exposure was reported in 105 (79.5%) HP patients and out of which 86.8% were females. CONCLUSIONS: IPF is the most prevalent ILD in Pakistan, followed by HP and iNSIP. An ongoing prospective registry with longitudinal follow-up will help us further elaborate the clinical characteristics, treatment and survival outcome of patients with ILD. CLINICAL IMPLICATIONS: An expansion of the ILD registry to other cities and rural areas of the country with longitudinal follow-up of currently enrolled patients and more stringent validation of data by MDD in tertiary care and National Coordinating Centers could further expand the network and refine disease information. DISCLOSURES: no disclosure on file for Mosavir Ansarie; No relevant relationships by Ali Zubairi, source=Web Response

Mechanical Ventilation and Predictors of In-Hospital Mortality in Fibrotic Interstitial Lung Disease With Acute Respiratory Failure: A Cohort Analysis Through the Paradigm of Acute Respiratory Distress Syndrome.

Prior studies report significant mortality in fibrotic interstitial lung disease patients undergoing mechanical ventilation. Little is known about baseline characteristics or ventilator strategies that might improve outcomes. We analyzed the ventilator characteristics of a large cohort of fibrotic interstitial lung disease patients from the perspective of an acute respiratory distress syndrome paradigm to see if any specific mechanical ventilation strategies might improve in-hospital mortality. Retrospective cohort study. Single-center, multihospital medical ICUs. Consecutive fibrotic interstitial lung disease patients who experienced mechanical ventilation for acute respiratory failure. Interstitial lung disease characteristics, demographics, and ventilator variables were analyzed for univariable and multivariable predictors of in-hospital mortality, adjusted for confounding with an a priori causation model. A total of 111 patients accounted for 114 admissions. Idiopathic pulmonary fibrosis comprised 34% with idiopathic acute exacerbation (65%) being the most common admission type. Ninety-five percent were initiated on mandatory volume-control ventilation with only 50% achieving a low tidal volume strategy (plateau pressure ≤ 30 cm H2O) within 3 hours of intubation. Unadjusted clinical predictors of in-hospital mortality included age (unit odds ratio, 1.05; 1.01-1.10; p = 0.015), time from admission to intubation (hr) (unit odds ratio, 1.01; 1.01-1.03; p = 0.017), and use of paralytics (relative risk, 1.54; 1.26-1.90, p < 0.001). Adjusted mechanical ventilation-related predictors of in-hospital mortality included achieving early targeted plateau pressures (odds ratio, 0.23; 0.07-0.76; p = 0.016), PaO2/FIO2 ratio at 3 (unit odds ratio, 0.98; 0.96-0.99, p = 0.002) and 48 hours (unit odds ratio, 0.98; 0.97-0.99, p = 0.018), initial mean airway pressure (unit odds ratio, 1.13; 1.02-1.28, p = 0.019), and total net fluid status (mL) (unit odds ratio, 1.01; 1.001-1.02, p = 0.0001). Several factors predict in-hospital mortality in fibrotic interstitial lung disease-associated mechanical ventilation when viewed through an acute respiratory distress syndrome model. Further research is needed to refine strategies that may perhaps improve survival if mechanical ventilation is pursued in this set of patients.

Immune checkpoint inhibitor-associated interstitial lung diseases correlate with better prognosis in patients with advanced non-small-cell lung cancer.

BackgroundInterstitial lung disease (ILD) induced by immune checkpoint inhibitors (ICIs) is a potentially life‐threatening adverse event. The purpose of this study was to evaluate whether the development of immune‐related adverse events (irAEs), especially ILD, was associated with treatment efficacy and to research the features and risk factors of ILD in advanced non‐small cell lung cancer (NSCLC).MethodsBetween December 2015 and November 2018, 130 advanced NSCLC patients were treated with nivolumab, pembrolizumab or atezolizumab. The patients were categorized into two groups (irAEs group or non‐irAEs group). Subsequently, we divided the irAEs group into two groups based on the incidence of ILD (ILD group and irAEs‐non‐ILD group). Treatment efficacy and the characteristics of ILD were evaluated.ResultsA total of 39 (30%) patients developed irAEs. ILD was observed in 16 (12%) patients. Patients with ILD had a higher objective response rate (ORR) compared with irAEs‐non‐ILD patients and non‐irAEs patients (63%, 43% and 22%, respectively). Median progression‐free survival (mPFS) was 15.9 months in ILD patients, 5.4 months in irAEs‐non‐ILD patients and 3.3 months in non‐irAEs patients (log‐rank test, P = 0.033). Pre‐existing interstitial pneumonia (IP) was an independent risk factor for ILD‐induced ICIs (odds ratio [OR] 14.7; 95% confidence interval [CI]: 2.16–99.6, P = 0.006).ConclusionsORR and PFS were significantly better in ILD patients than in irAEs‐non‐ILD and non‐irAEs patients. Pre‐existing history of IP was an independent risk factor for ILD‐induced ICIs.

Interstitial lung disease associated with anti-citrullinated peptide/protein antibody-positive anti-synthetase syndrome

Little has been reported on the characteristics of interstitial lung disease (ILD) associated with anti-citrullinated peptide/protein antibody (ACPA)-positive anti-synthetase syndrome (ASS). We sought to investigate the clinical, radiologic, and pathologic features of patients with ILD associated with ACPA-positive ASS. Medical records of seven ILD patients with positive results of both ACPA and anti-aminoacyl-tRNA synthetase antibody were retrospectively reviewed. Five patients had clinical symptoms associated with ASS other than ILD. On high-resolution computed tomography (HRCT) analysis, a nonspecific interstitial pneumonia (NSIP) pattern was shown in 3 patients and NSIP with organizing pneumonia (OP) overlap in 2 patients. Coronal slices of these 5 patients showed lower lung disease predominance with traction caudally on major fissures due to lower lobe volume loss. These were features that could commonly be observed in ASS-associated ILD. Pathological findings available for 3 patients showed NSIP. The characteristics of ILD associated with ACPA-positive ASS appear to be similar to those of ILD associated with ASS, but not to rheumatoid arthritis (RA) or ACPA, especially in terms of the radiological findings.

The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies

RationaleAnti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined. As our previous studies revealed that ARS-ILD without PM/DM was similar to CTD-associated ILD, and that ARS-ILD with PM/DM was radiologically suggestive of a nonspecific interstitial pneumonia (NSIP) pathological pattern, we hypothesized that the prognosis of ARS-ILD might be distinct from that of idiopathic pulmonary fibrosis (IPF) without anti-ARS. ObjectivesTo elucidate the long-term outcome of ARS-ILD with and without PM/DM and compare it to that of IPF. MethodsA two-center retrospective study was conducted. The study population comprised 36 patients with ARS-ILD (8 with PM, 12 with DM, and 16 without myositis throughout the course), 100 patients with IPF without anti-ARS, and 7 patients with NSIP without anti-ARS. The presence of anti-ARS was determined by RNA immunoprecipitation using the sera obtained at the time of diagnosis before specific treatment. Measurements and main resultsDuring the observational period (median 49 months; range, 1–114 months), 7 patients with ARS-ILD (19%; 3 with PM, 1 with DM, and 3 without PM/DM) and 51 patients with IPF (51%) died. Patients with ARS-ILD had better overall survival than those with IPF (log-rank test, P < 0.001) and similar survival compared to those with NSIP (log-rank test, P = 0.59). The prognosis for patients with ARS-ILD was similar between those with and without myositis (log-rank test, P = 0.91). At the median follow-up time of 76.5 months, 14 of the 36 patients with ARS-ILD had deteriorated. Both a decline in forced vital capacity or an initiation of long-term oxygen therapy during the course (odds ratio [OR], 5.34) and acute exacerbation (OR, 28.4) significantly increased the mortality risk. ConclusionsThe long-term outcome of ARS-ILD was significantly better than that of IPF regardless of the presence or absence of myositis.

Clinical characteristics associated with occurrence and poor prognosis of interstitial lung disease in rheumatoid arthritis.

Background/AimsTo analyze clinical characteristics of interstitial lung disease (ILD) associated with rheumatoid arthritis (RA), especially in patients with poor prognosis. MethodsSeventy-seven RA patients with ILD and 231 age, sex, and disease duration-matched RA patients without ILD were enrolled in this retrospective study. Epidemiologic, clinical, and laboratory information were obtained through a medical chart review. Logistic regression analysis was used to estimate the risk of mortality in RA patients with ILD. ResultsCompared to the RA without ILD group, the RA with ILD group had significantly higher titers of rheumatoid factor and the anti-cyclic citrullinated peptide (p = 0.001 for both), higher levels of C-reactive protein (CRP) at the time of RA diagnosis (p = 0.014), and a higher erythrocyte sedimentation rate (p = 0.022) and CRP levels (p < 0.001) throughout the 10-year follow-up period. These patients also received a higher mean daily dose of corticosteroids (p < 0.001). In the subgroup analysis of RA patients with ILD, 28 patients (36.4%) died during follow-up. Multivariate analysis revealed that older age at the time of ILD diagnosis was significantly associated with mortality. Usual interstitial pneumonia (UIP) subtype on high-resolution computed tomography (HRCT) was also suggested as a poor prognostic factor. ConclusionsThe survival of RA patients with ILD is adversely affected by age at the time of ILD diagnosis. RA-ILD patients diagnosed after age 65 or with a UIP subtype on HRCT may have a poor prognosis.

Recent Treatments of Interstitial Lung Disease with Systemic Sclerosis.

Systemic sclerosis (SSc) is a disorder characterized by immune dysfunction, microvascular injury, and fibrosis. Organ involvement in patients with SSc is variable; however, pulmonary involvement occurs in up to 90% of patients with SSc. Interstitial lung disease (ILD) is a major cause of mortality and, thus, a major determinant in the prognosis of patients with SSc. This review summarizes current findings about the characteristics of ILD in patients with SSc, selection of patients with SSc-ILD who are candidates for the treatment, and current treatment options.

A retrospective study of clinical characteristics of interstitial lung disease associated with rheumatoid arthritis in Chinese patients.

BackgroundInterstitial lung disease (ILD) in rheumatoid arthritis (RA) is associated with a poor prognosis. The purpose of this study was to assess the characteristics of ILD that are associated with RA.Material/MethodsThis was a retrospective study of 544 Chinese patients with RA (427 women and 117 men). RA-ILD was diagnosed by high-resolution computed tomography (HRCT). Patients with RA-ILD or with RA alone were compared in terms of age, sex distribution, duration of disease, clinical and laboratory parameters, history of smoking, and medication.ResultsBased on HRCT imaging, 83 (15.26%) patients with RA were diagnosed with ILD. ILD was more frequent in older patients (59.60±9.66 vs. 50.54±13.76 years, P<0.001), in those with a longer duration of disease (7.46±7.40 vs. 5.27±6.32 years, P=0.013) and in male patients (34.9% vs. 19.1%, P=0.001). RA-ILD was found to be associated with hepatitis B surface antigen (HBsAg) positivity (odds ratio [OR]=2.56, 95% confidence interval [95% CI] 1.02–6.43) and smoking (OR=3.38, 95% CI 1.65–6.95). Higher levels of C-reactive protein (OR=3.59, 95% CI 1.58–8.15), anti-cyclic citrullinated peptide (CCP) (OR=2.24, 95% CI 2.09–4.13), and rheumatoid factor (OR=3.72, 95% CI 1.56–8.86) were detected in association with RA-ILD. RA-ILD was more frequently observed in patients treated with steroids (OR=1.91, 95% CI 1.18–3.09) or Tripterygium wilfordii (OR=2.56, 95% CI 1.21–5.40). Age (OR=2.20, 95% CI 1.04–4.65), age at RA onset (OR=2.55, 95% CI 1.11–5.90), anti-CCP (OR=2.47, 95% CI 1.19–5.17), and steroid use (OR=1.83, 95% CI 1.04–3.20) were independently associated with RA-ILD in multivariate analysis.ConclusionsRA-ILD was associated with age, age at RA onset, anti-CCP, and steroid use. Anti-CCP antibodies might be important biomarkers of RA-ILD.

THU0200 Clinical and radiological characteristics of interstitial lung disease in japanese patients with microscopic polyangiitis

BackgroundThe anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with poliangiitis (EGPA) frequently involve multiple internal organs including lung diseases, which...

Clinical and Pathological Findings of Interstitial Lung Disease Patients with Anti-Aminoacyl-tRNA Synthetase Autoantibodies

The aim of this study was to investigate the clinicopathological characteristics of interstitial lung disease (ILD) patients with anti-aminoacyl-tRNA synthetase (anti-ARS) autoantibodies. Patients and Methods We examined 14 ILD patients with anti-ARS autoantibodies between 2004 and 2007 and retrospectively investigated their clinical, radiographic, and pathological findings. Anti-Jo-1 antibodies were the most common (10 of 14), followed by anti-OJ, anti-KS, and anti-EJ (1 each for 3 patients); 1 patient with polymyositis had both anti-Jo-1 and anti-PL-12 antibodies. Ten patients had a chronic clinical course, whereas 4 presented with subacute deterioration. Of 8 patients with myositis, 1 (12.5%) had myositis-preceding ILD, 3 (37.5%) had ILD-preceding myositis, and 4 (50%) had simultaneous onset. Chest high-resolution computed tomography frequently showed lung-base predominant ground glass opacities (GGO) with volume loss. The results of surgical lung biopsies indicated that 4 patients had nonspecific interstitial pneumonia (NSIP) and/or organizing pneumonia (OP) patterns. All but 1 received corticosteroid therapy, and 6 patients were also given cyclosporin. The mean duration of follow-up was 22 months (range, 5-47 months). ILD improved in 9 patients and stabilized in 3; however, in 1 patient, it initially improved during 6 months, then progressively worsened despite treatment, and finally resulted in death. These results indicate that ILD patients with anti-ARS antibodies usually have a chronic clinical course, lung-base predominant GGO with volume loss, NSIP and/or OP patterns, and a good response to corticosteroid treatment; however, some have a rapidly worsening course and recurrence, despite therapy.

Characterization and peripheral blood biomarker assessment of anti–Jo‐1 antibody–positive interstitial lung disease

Using a combination of clinical, radiographic, functional, and serum protein biomarker assessments, this study was aimed at defining the prevalence and clinical characteristics of interstitial lung disease (ILD) in a large cohort of patients with anti-Jo-1 antibodies. A review of clinical records, pulmonary function test results, and findings on imaging studies determined the existence of ILD in anti-Jo-1 antibody-positive individuals whose data were accumulated in the University of Pittsburgh Myositis Database from 1982 to 2007. Multiplex enzyme-linked immunosorbent assays (ELISAs) for serum inflammation markers, cytokines, chemokines, and matrix metalloproteinases in different patient subgroups were performed to assess the serum proteins associated with anti-Jo-1 antibody-positive ILD. Among the 90 anti-Jo-1 antibody-positive individuals with sufficient clinical, radiographic, and/or pulmonary function data, 77 (86%) met the criteria for ILD. While computed tomography scans revealed a variety of patterns suggestive of underlying usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia, a review of the histopathologic abnormalities in a subset of patients undergoing open lung biopsy or transplantation or whose lung tissue was obtained at autopsy (n = 22) demonstrated a preponderance of UIP and diffuse alveolar damage. Analysis by multiplex ELISA yielded statistically significant associations between anti-Jo-1 antibody-positive ILD and elevated serum levels of C-reactive protein (CRP), CXCL9, and CXCL10, which distinguished this disease entity from idiopathic pulmonary fibrosis and anti-signal recognition particle antibody-positive myositis. Recursive partitioning further demonstrated that combinations of these and other serum protein biomarkers can distinguish these disease subgroups at high levels of sensitivity and specificity. In this large cohort of anti-Jo-1 antibody-positive individuals, the incidence of ILD approached 90%. Multiplex ELISA demonstrated disease-specific associations between anti-Jo-1 antibody-positive ILD and serum levels of CRP as well as the interferon-gamma-inducible chemokines CXCL9 and CXCL10, highlighting the potential of this approach to define biologically active molecules contributing to the pathogenesis of myositis-associated ILD.

Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients

The strongest predictive factor for the development of interstitial lung disease (ILD) in myositis (IIM) patients is the presence of different antisynthetase antibodies. The aim of this study was to compare the clinical characteristics, radiological findings and therapeutic response between the anti-SS-A positive and negative antisynthetase syndrome (ASS) patients. A prospective study of 315 IIM patients was conducted including 27 anti-Jo-1 positive ASS patients. Mean disease duration was 46.6 (range 4-198) months. All patients fulfilled the classification criteria for IIM. All patients underwent chest radiography, pulmonary function tests and HRCT at he time of diagnosis and 6 months after the immunosuppressive therapy. Routine laboratory tests, RF, ANA, anti-ENA, anti-SS-A, anti-histidyl-transfer RNA antibody (Jo-1) measurements were performed in all patients. ILD was found to be present in 70.4% of ASS patients. The anti-SS-A negative ASS group had a more frequent association with alveolitis and responded well to immunosuppressive therapy (p < 0.05). HRCT scan showed more fibrosis in the SS-A positive group. 15.8% of patients died due to pulmonary or cardiac complications. In conclusion, coexistence of anti-SS-A and anti-Jo-1 antibody may be a good predictor for a more coarse and severe ILD in IIM patients who require a more aggressive approach in therapy.