Higher Prevalence Of Interstitial Lung Disease Research Articles

Assessment of risk factors in patients with rheumatoid arthritis-associated interstitial lung disease.

To assess the risk factors for interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) and to evaluate the association of ILD with the use of methotrexate as well as with joint disease activity. A retrospective, cross-sectional study conducted between March and December 2019 at a tertiary healthcare center, in a follow-up of RA patients who had undergone pulmonary function tests (PFT) and chest computed tomography. We evaluated the tomographic characteristics, such as the presence of ILD and its extension, as well as joint disease activity. Functional measurements, such as forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO), were also assessed. After this, a multivariate logistic regression analysis was applied in order to identify risk factors associated with ILD. We evaluated 1.233 patients, of which 134 were eligible for this study. The majority were female (89.6%), with a mean age of 61 years old and with a positive rheumatoid factor (86.2%). RA-associated ILD (RA-ILD) was detected in 49 patients (36.6%). We found an association of RA-ILD with age ≥= 62 year, male sex, smoking history and fine crackles in lung auscultation and a decreased DLCO. The indicators of being aged ≥ 62 years old and having moderate or high RA disease activity were both independent factors associated with RA-ILD, with an odds ratio of 4.36 and 3.03, respectively. The use of methotrexate was not associated with a higher prevalence of ILD. Age and RA disease activity are important risk factors associated with RA-ILD. Methotrexate was not associated with the development of RA-ILD in the present study.

Elderly-onset primary Sjögren's syndrome focused on clinical and salivary gland ultrasonographic features.

To assess the clinical, laboratory, and salivary gland ultrasound (SGUS) characteristics of elderly-onset of primary Sjögren's syndrome (EopSS). We included pSS patient from two referral hospitals over a 4-year period. The SGUS scores (0-48) and SG volumes were assessed. Clinical, laboratory, and SGUS data were compared according to age at onset: EopSS (≥65 years), adult-onset (AopSS) (≥40 and <65 years), and young-onset (YopSS) (<40 years). This cross-sectional study included a total of 221 patients, 43 (19.5%) of which had EopSS. Subjective sicca symptoms, results of the Schirmer's test, and unstimulated salivary flow rate revealed no significant differences between the groups. EopSS patients presented a significantly higher frequency of interstitial lung disease (ILD) (EopSS: 51.2% vs. AopSS: 13.5% vs. YopSS: 8.7%, P<0.001) and lower frequency of arthritis (7% vs. 22.6% vs. 39.1%, P<0.01). They also had significantly lower positivity of anti-Ro/SSA (51.2%) and anti-La/SSB (7.0%) and lower levels of rheumatoid factor, C4, and IgG. The EopSS group had significantly lower SGUS positivity (defined as total scores ≥14) (44.2% vs. 64.5% vs. 78.3%, P<0.05), lower SGUS scores, and smaller submandibular gland volume. We report a specific phenotype of EopSS, characterised by high prevalence of ILD, less involvement of the peripheral joint, and low biological activity. SGUS evaluation showed less parenchymal abnormalities but more atrophic changes in major SGs in EopSS patients. Considering the low positivity of anti-Ro/SSA and SGUS in EopSS, SG biopsy remains the only way to confirm the diagnosis of pSS, especially in elderly patients.

THU0120 OVERLAPPING SJOGREN’S SYNDROME AND ULTRASOUND REMISSION, FUNCTIONAL DISABILITY AND MANAGEMENT IN RHEUMATOID ARTHRITIS PATIENTS: A PROPENSITY SCORE MATCHED REAL-WORLD COHORT FROM 2009 TO 2019

Background:Rheumatoid arthritis (RA) patients with Sjogren’s syndrome (SS) are often referred to as more severe synovitis.Objectives:We intend to clarify the impact of overlapping SS on ultrasound remission, functional ability improvement and clinical decision-making in RA patients in a real-world cohort from 2009 to 2019.Methods:The medical records of RA patients in our medical center from 2009 to 2019 were reviewed. Cox proportional hazards models of ultrasound remission and health assessment questionnaire (HAQ) improvement were conducted in both the 1-to-1 nearest propensity score matched (PSM) and unmatched cohorts between those RA patients with SS (RA-SS) and without (RA-noSS) to correct critical confounders. Four kinds of PSM methods were used and the corresponding average treatment effect on the treated (ATT) was calculated to clarify the effect of overlapping SS on distinguishable characteristics or drug prescription in RA patients.Results:A total of 1100 RA patients were included in the study, of which 133 (12.1%) overlapped with SS. Among 256 patients consisting of 128 RA-SS and 128 RA-noSS after 1-to-1 nearest PSM, overlapping SS was associated with a 44%, 32% lower probability of reaching ultrasound remission, no-functional disability in RA patients, respectively. More prevalent interstitial lung disease (ILD), leukopenia, hypergammaglobulinemia, rheumatoid factor (RF) positivity, higher erythrocyte sedimentation rate (ESR) and more hydroxychloroquine (HCQ) usage, less biologic disease-modifying anti-rheumatic drugs (bDMARDs) prescription were confirmed to be correlated with overlapping SS by the robust PSM.Conclusion:Overlapping SS is associated with a lower probability of reaching ultrasound remission and functional activity improvement, higher prevalence of ILD, leukopenia and hypergammaglobulinemia in RA patients. Weaker interventions such as HCQ may be the mainstream of clinical decision making.Table.Hazard Ratios for Ultrasound Remission/No Functional Disability Associated with Overlapping SSUnmatched cohortMatched cohortUS remissionNo functional disabilityUS remissionNo functional disabilityUnstratified0.63 (0.51, 0.79)0.60 (0.52, 0.70)0.56 (0.42, 0.74)0.68 (0.55, 0.83)Stratified Gender0.62 (0.50, 0.77)0.63 (0.54, 0.74)0.53 (0.40, 0.71)0.66 (0.54, 0.80) Age0.63 (0.51, 0.78)0.60 (0.52, 0.70)0.54 (0.40, 0.72)0.62 (0.50, 0.76) RF0.64 (0.52, 0.80)0.63 (0.54, 0.73)0.56 (0.42, 0.74)0.68 (0.55, 0.83) ACPA0.63 (0.51, 0.79)0.59 (0.50, 0.68)0.57 (0.43, 0.76)0.63 (0.51, 0.78) Seropositivity0.64 (0.52, 0.80)0.63 (0.54, 0.74)0.59 (0.45, 0.79)0.75 (0.61, 0.92) RA duration0.64 (0.51, 0.79)0.61 (0.52, 0.71)0.58 (0.44, 0.77)0.70 (0.57, 0.86) BLDAS28CRP0.64 (0.51, 0.80)0.62 (0.53, 0.72)0.54 (0.41, 0.72)0.66 (0.54, 0.81)Values are presented as total and stratified Hazard Ratio (95% CI) for ultrasound remission and no functional disability associated with overlapping SS in RA patients according to gender, age, RF/ACPA status, RA duration and DAS28-CRP at 1stvisit. US remission refers to ultrasound GS&lt;2 and PD=0. Unmatched cohort refers to whole sample (n=1100), matched cohort refers to propensity score matched (PSM) patients (n=256). The values are statistically significant at the level of 0.01.Disclosure of Interests:None declared

Unique clinical and autoantibody profile of a large Asian Indian cohort of scleroderma-do South Asians have a more aggressive disease?

A single-centre retrospective study was conducted using electronic medical records (EMR) of inpatients and outpatients with the diagnosis of "scleroderma" or "systemic sclerosis" visiting our clinic over the preceding 5years. A total of 327 patients' charts met our selection criteria; 301 were females. The median (IQR (inter quartile range)) age at onset of first non-Raynaud's symptom was 34.67 (27-43) years and median (IQR) disease duration prior to presentation to our department was 2.5 (1-5) years. Of these, 310 (94.8%) belonged to diffuse systemic sclerosis variety, 13 (4%) had limited systemic sclerosis, and 4 (1.2%) were of sine scleroderma type. A total of 289/302 (95.7%) patients were positive for ANA; of them, 245/327 (74.9%) were Scl-70 antibody-positive and 4% were CENP antibody-positive. Interstitial lung disease (ILD) was present in 288/327 (88.1%) patients. Among patients with available baseline forced vital capacity (FVC) data, 20% had a normal lung function and 28.4% had severe restriction. Pulmonary hypertension as assessed by echocardiography was present in 8.1% of patients. A significant association of Scl-70 antibody positivity with the presence of interstitial lung disease (ILD) (p = 0.000) and pulmonary hypertension (p = 0.035) was seen. On the other hand, presence of CENP antibody showed a protective trend against muscle weakness and/or muscle enzyme elevation (p = 0.052). Presence of arthritis was protective against development of digital ulceration (p = 0.021) and PAH (0.004). Patients younger than 40years of age had significantly higher frequency of Scl-70 positivity (p = 0.038), whereas CENP antibody positivity was more likely in those aged > 40years (p = 0.002). Younger age of onset and high prevalence of Scl-70 antibody are unique South Asian features common with large Indian, Thai, and Chinese series. High prevalence of ILD is a feature common to Indian and Chinese series. Strong correlation of Scl-70 antibody with younger age and pulmonary hypertension were unique features of our cohort. • Asian Indian scleroderma patients are younger by 2 decades compared to Caucasian series. • Higher prevalence of Scl-70 antibody, its association with young age, interstitial lung disease and pulmonary hypertension are features of our cohort. • High prevalence of interstitial lung disease (88.1%) was noted ; among those with baseline spirometry data (141/327), two thirds(66%) had moderate to severe restriction. • Younger age at onset, higher prevalence of Scl-70 antibody are features common to other Indian, Thai and Chinese series.

Prognostic profile of systemic sclerosis: analysis of the clinical EUSTAR cohort in China

BackgroundSystemic sclerosis is a disease that has significant clinical heterogeneity. This study aims to determine the causes and risk factors of death in a single center European League Against Rheumatism Scleroderma Trials and Research Group (EUSTAR) cohort at the Peking Union Medical College Hospital (PUMCH) in China.MethodsPatients clinically diagnosed with systemic sclerosis (SSc) between Feb 2009 and Dec 2015 were prospectively recruited from the EUSTAR database and Chinese Rheumatism Data Center (CRDC) of the PUMCH. Baseline and follow-up data were collected. Kaplan-Meier analysis was used to estimate survival, and Cox proportional hazards regression analysis was used to identify factors associated with mortality.ResultsA total of 448 patients were included in the cohort, of whom 56.7% had limited cutaneous systemic sclerosis (lcSSc). The average age at diagnosis was 42.8 ± 12.1 years. The prevalence of interstitial lung disease (ILD) was 382/447 (85.5%). Among 402 patients, 348 of them took glucocorticoid during the disease course; 374 patients received immunosuppressors. Across 2167 patient-years, 40 patients died. Of these, 27 deaths were attributable to SSc, with pulmonary arterial hypertension (PAH) being the leading cause of death. The median survival time was 53 months. Survival rates from disease diagnosis were 97.0%, 94.6%, 91.1% and 87.8% at 1, 3, 5 and 10 years, respectively. Independent prognostic factors for mortality were PAH (HR 6.248, 95% CI 2.855, 13.674) and arrhythmia (HR 4.729, 95% CI 1.588, 14.082). Tripterygium wilfordii Hook F (TwHF) (log-rank test 7.851, p 0.005) and methotrexate (MTX) (log-rank test 7.925, p = 0.005) were found in survival analysis to be protective treatments against mortality. Patients who used cyclophosphamide (CTX) during the disease course had poorer prognosis (log-rank test 5.177, p = 0.023).ConclusionsIn china, although there is a high prevalence of ILD in patients with SSc (85.5%), most of them have reserved pulmonary function, which means that interstitial lung disease (ILD) is not the most important factor in the death of patients with SSc and also is not a risk factor for poor prognosis. Only ILD with pulmonary dysfunction is associated with poor outcome. The 10-year cumulative rate (87.8%) in patients with SSc in China is slightly lower than the Europe, and pulmonary arterial hypertension (PAH) and arrhythmia at baseline are independent prognostic factors, whereas PAH instead of ILD is the leading cause of death in patients with SSc. Interestingly, the Chinese traditional medicine TwHF, as a protective factor for survival deserves further study.

An analysis of the clinical features of lung cancer in patients with connective tissue diseases.

Patients with connective tissue diseases (CTDs) are at increased risk for lung cancer (LC); interstitial lung disease (ILD) is a common form of organ dysfunction in cases of CTD. However, the influence of ILD on the treatment and prognosis in LC patients with CTD is unclear. Between January 2010 and December 2014, 27 patients among all patients with CTD at our institution were diagnosed with primary LC. We retrospectively analyzed the clinical features, treatment modalities, and outcomes of these patients, and evaluated the potential prognostic factors. Forty-four LC patients without CTD were also analyzed as a control cohort. LC patients with CTD had a significantly higher incidence of ILD as a complication compared with those without CTD (52% and 14%, respectively). CTD-associated ILD (CTD-ILD) at diagnosis was associated with significantly worse survival in LC patients with CTD. Multivariate analysis demonstrated that the complication of CTD-ILD was an independent poor prognostic factor in LC patients with CTD. The incidence of acute exacerbation (AE) of CTD-ILD was 21% among LC patients with CTD, and all of these patients died despite intensive treatment including high-dose corticosteroids. The restrictions in curative therapy for LC due to the presence of ILD and AE of CTD-ILD were thought to be the major reasons for the poor outcome. LC patients with CTD had a high prevalence of ILD, and the presence of CTD-ILD was significantly associated with poor prognosis.

Incidence and predictors of interstitial lung disease (ILD) in Thai patients with early systemic sclerosis: Inception cohort study

Objectives: To determine and compare the prevalence of interstitial lung disease (ILD), the severity of high-resolution computed tomography (HRCT) score and incidence rate (IR) of ILD between the two subsets of early-SSc (systemic sclerosis) patients. We also determined the factors associated with ILD.Methods: We used an inception cohort of early-SSc patients seen between January 2010 and June 2014. All patients underwent HRCT at study entry and annually thereafter.Results: One hundred and thirteen patients (66 females and 89 diffuse cutaneous SSc [dcSSc]) with a mean ± SD age of 53.4 ± 8.4 years and mean disease duration of 12.9 ± 10.3 months at cohort entry were enrolled. At enrollment, patients with dcSSc had a higher prevalence of ILD (78.7% vs. 45.8%, p = 0.002), and a higher total HRCT score (10.3 ± 9.5 vs. 4.4 ± 5.6, p = 0.001) compared with limited cutaneous SSc (lcSSc). DcSSc patients had a higher IR of ILD than lcSSc patients (58.8 vs.17.3 per 100 person-years, p < 0.001). Univariable analysis revealed that male gender, presence of anti-Scl 70 and absent anti-centromere antibody was significant predictors of ILD. In Cox-regression analysis, a positive anti-centromere [hazard ratio (HR) 0.09 95% confidence interval (95% CI 0.01–0.73)] was a protective factor.Conclusions: DcSSc patients had more severe HRCT scores and higher IR of ILD compared with lcSSc patients. Male gender, presence of anti-Scl 70, and absent anti-centromere antibody predicted the future development of ILD in early-SSc patients.

Prevalence of interstitial lung involvement in patients with connective tissue diseases assessed with high‐resolution computed tomography

Objectives: To assess the prevalence of interstitial lung disease (ILD) in patients with different forms of connective tissue disease (CTD) using non‐invasive procedures including high‐resolution computed tomography (HRCT) and to evaluate the relationship between the imaging and functional status of the patients.Methods: Eighty‐one subjects with CTD (47 inpatients and 34 outpatients) were evaluated with pulmonary function tests (PFT) and radiological investigations. The extent and severity of lung disease was quantified with an HRCT scoring system previously used in patients with systemic sclerosis (SSc). Interstitial lung involvement was defined as predominantly fibrotic or inflammatory based on HRCT abnormalities.Results: HRCT abnormalities suggestive of ILD were observed in 69 patients (85.1%), whereas PFT and plain radiograph alterations occurred less frequently (40.7%). The most frequent HRCT abnormalities were septal/subpleural lines and ground‐glass appearance whereas lesions consistent with advanced fibrosis were observed in a minority of patients. The HRCT score was higher in patients with abnormal PFT (p<0.001). Thirty‐five patients had predominant fibrosis and 34 patients predominantly inflammatory abnormalities. A score of 10 points represented the best compromise between sensitivity and specificity in predicting functional impairment.Conclusions: A high prevalence of ILD was found based on HRCT abnormalities. However, HRCT scans characterized by minor abnormalities have poor specificity for clinically significant disease and functional findings should also be considered. The large number of patients with predominantly inflammatory HRCT abnormalities suggests that many cases of ILD may be diagnosed in a relatively early stage of the disease.