Development Of Interstitial Lung Disease In Patients Research Articles

Identification, Monitoring, and Management of Rheumatoid Arthritis-Associated Interstitial Lung Disease.

Interstitial lung disease (ILD) is a frequent complication of rheumatoid arthritis (RA) that is associated with a significant increase in mortality. Several risk factors for the development of ILD in patients with RA have been identified, but ILD can still develop in the absence of these risk factors. Screening tools for RA-ILD are required to facilitate early detection of RA-ILD. Close monitoring of patients with RA-ILD for progression is crucial to enable timely implementation of treatment strategies to improve outcomes. Patients with RA are commonly treated with immunomodulatory therapies, although their efficacy in slowing the progression of RA-ILD remains the subject of debate. Clinical trials have shown that antifibrotic therapies slow decline in lung function in patients with progressive fibrosing ILDs, including patients with RA-ILD. The management of patients with RA-ILD should be based on multidisciplinary evaluation of the severity and progression of their ILD and the activity of their articular disease. Close collaboration between rheumatologists and pulmonologists is essential to optimize patient care.

Intravenous Immunoglobulin Therapy for Maintenance Therapy of Interstitial Lung Disease Due to Antisynthetase Syndrome

Antisynthetase syndrome (AS) is a rare autoimmune condition characterized by fever, Raynaud's phenomenon, hyperkeratosis, inflammatory polyarthritis, and interstitial lung disease (ILD). The development of ILD in patients with antisynthetase syndrome (AS-ILD) portends a poor prognosis to an already rare and difficult to diagnose autoimmune disorder.

Decreased serum cathepsin S levels in patients with systemic sclerosis-associated interstitial lung disease.

Cathepsin S (CTSS) is a lysosomal proteolytic enzyme regulating intracellular and extracellular biological activities, including immunity/inflammation and remodeling of vasculature and extracellular matrix, which are the three cardinal pathological events associated with systemic sclerosis (SSc). To elucidate the potential role of CTSS in the development of SSc, we investigated the clinical correlation of serum CTSS levels. Because serum CTSS levels were inversely correlated with estimated glomerular filtration rate (eGFR) in SSc patients with renal dysfunction (eGFR, <60min/mL per 1.73m2 ), SSc patients with normal renal function (eGFR, ≥60min/mL per 1.73m2 ) were analyzed. Serum CTSS levels were significantly decreased in diffuse cutaneous SSc patients compared with limited cutaneous SSc patients and healthy controls. Among vascular and fibrotic clinical manifestations, Raynaud's phenomenon and interstitial lung disease (ILD) were relevant to a significant decrease in serum CTSS levels. Importantly, serum CTSS levels negatively correlated with serum levels of Krebs von den Lungen-6 and surfactant protein D in total SSc patients, while not correlating with modified Rodnan total skin thickness score and the percentage of predicted diffusion lung capacity for carbon monoxide and showing a positive trend with the percentage of predicted vital capacity. These results suggest a potential contribution of decreased CTSS expression to the development of ILD in patients with SSc.

FRI0531 AIR POLLUTANTS AND DEVELOPMENT OF INTERSTITIAL LUNG DISEASE IN PATIENTS WITH AUTOIMMUNE DISEASES

Background:Interstitial lung disease (ILD) is characterized by progressive inflammation and fibrosis, and accumulating evidence have shown that exposure to air pollutants was associated with the development of ILD. Autoimmune diseases are highly correlated with ILD, including connective tissue disease-associated ILD (CTD-ILD) as well as interstitial pneumonia with autoimmune features (IPAF), and the development of ILD is a crucial cause of morbidity and mortality in patients with autoimmune diseases. One recent Taiwanese study reported that exposure to air pollutants was associated with incident systemic lupus erythematosus (SLE). However, the impact of air pollutants on the development of ILD among patients with autoimmune diseases remains unknown.Objectives:The study aimed to address the impact of accumulating exposure to air pollutant above moderate level, defined by Air Quality Index (AQI) value higher than 50, on the development of ILD in patients with autoimmune diseases including SLE, rheumatoid arthritis (RA) and primary Sjögren’s syndrome (SS).Methods:We used a National Health Insurance Research Database in Taiwan to enroll patients with SLE (International Classification of Diseases (ICD)-9 code 710.0, n=13,211), RA (ICD-9 code 714.0 and 714.30–714.33, n=32,373), and primary SS (ICD-9 code, 710.0, n=15,246) between 2001 and 2013. We identified newly diagnosed ILD cases (ICD-code 515) between 2012 and 2013 and selected age, sex, disease duration and index-year matched (1:4) patients as non-ILD controls. The hourly levels of air pollutants one year prior to the index-date were obtained from 60 air quality monitoring stations across Taiwan, and the air pollutants in the present study consisted of particulate matter &lt;2.5 μm in size (PM2.5), particulate matter &lt;10 μm in size (PM10), nitrogen dioxide (NO2), carbon monoxide (CO), sulfur dioxide (SO2) and ozone (O3). We used a spatio-temporal model built by a deep-learning mechanism to estimate levels of air pollutants at 374 residential locations based on data of 3 air quality monitoring stations near the location (8). Notably, we used cumulative exposed hours to air pollutants higher than modest level, defined by AQI criteria, given that daily mean level of air pollutants might possibly underestimate the triggered inflammatory effect by a temporary exposure of high-level air pollutant. A conditional logistic regression was used to determine the association between exposure to air pollutant and the development of ILD, adjusting age, gender, Charlson Comorbidity Index (CCI), urbanization, family income, and medications for autoimmune diseases.Results:A total of 272 patients with newly diagnosed ILD were identified among patients with autoimmune diseases, including 39 with SLE, 135 with RA, and 98 with primary SS. We found that the duration of exposure to PM 2.5 higher than modest level was associated with the risk of ILD development in patients with SS (adjOR 1.07, 95% CI 1.01–1.13), and similar trends were also found in patients with SLE (adjOR 1.03, 95% CI 0.95–1.12) and RA (adjOR 1.03, 95% CI 0.99–1.07). Intriguingly, we observed an inverse correlation between the duration of exposure to O3 and the development of ILD in patients with SS (adjOR 0.83, 95% CI 0.70–0.99); however, the finding was not found in patients with SLE (adjOR 1.13, 95% CI 0.92–1.37) and RA (adjOR 0.98, 95% CI 0.87–1.11).Conclusion:In conclusion, we identified that longer exposure to PM2.5 higher than modest level tended to be associated with the development of ILD in patients with autoimmune diseases, mainly SS.

Análisis del índice masa corporal y factores de riesgo de neumopatía intersticial en pacientes con esclerosis sistémica

ObjectiveTo determine the risk factors and the potential relationship between body mass index (BMI) and the development of interstitial lung disease (ILD) in patients with systemic sclerosis. MethodsA cross-sectional, correlational, study with a non-probabilistic sample was conducted on consecutive patients with scleroderma seen in the Rheumatology Division at Hospital Universitario de Caracas during the period April to September 2018. Patients underwent clinical and laboratory assessments, anthropometric measurements, pulmonary function tests, and high-resolution computed axial tomography scan of the chest. ResultsOf 48 patients, 95.8% were female, with a high prevalence of limited cutaneous systemic sclerosis (lcSSc) (64.6%). Underweight (BMI <18.5kg/m2) was a risk factor for the development of ILD (OR 4.60; 95% CI 1.12-18.86). There was directly proportional relationship between BMI and forced vital capacity, with no statistical significance (CC 0.23; P=.1180). However, other predictors of ILD included diffuse systemic sclerosis (OR 6.91, 95% CI; 1.78-26.85), modified Rodnan Skin Score (mRSS) (OR 1.25; 95% CI; 1.04-1.30), and a speckled pattern of antinuclear antibody (OR 5.62; 95% CI; 1.33-23.62). Multivariate analysis showed that mRSS (OR 1.34; 95% CI; 1.04-1.72) and the presence of a speckled antinuclear antibody (OR 25.99; 95% CI; 1.02-16.02) were independent predictors of ILD. The percentage of BMI lost in a period of time of 2008-2018 was 14.3% (P=.0021), which was higher than rheumatoid arthritis (P=.0000), systemic lupus erythematosus (P=.0025), and healthy patients (P=.0331). ConclusionBMI and ILD are inversely correlated. Age, underweight, higher mRSS, and speckled pattern of antinuclear antibody were risk factors for development of ILD in patients with systemic sclerosis. The mRSS could be used as a clinical predictive tool for ILD in systemic sclerosis.

Analysis of body mass index and risk factors of interstitial lung disease in systemic sclerosis patients

ObjectiveTo determine the risk factors and the potential relationship between body mass index (BMI) and the development of interstitial lung disease (ILD) in patients with systemic sclerosis. MethodsA cross-sectional, correlational, study with a non-probabilistic sample was conducted on consecutive patients with scleroderma seen in the Rheumatology Division at Hospital Universitario de Caracas during the period April to September 2018. Patients underwent clinical and laboratory assessments, anthropometric measurements, pulmonary function tests, and high-resolution computed axial tomography scan of the chest. ResultsOf 48 patients, 95.8% were female, with a high prevalence of limited cutaneous systemic sclerosis (lcSSc) (64.6%). Underweight (BMI<18.5kg/m2) was a risk factor for the development of ILD (OR 4.60; 95% CI 1.12–18.86). There was directly proportional relationship between BMI and forced vital capacity, with no statistical significance (CC 0.23; p=0.1180). However, other predictors of ILD included diffuse systemic sclerosis (OR 6.91, 95% CI; 1.78–26.85), modified Rodnan skin score (mRSS) (OR 1.25; 95% CI; 1.04–1.30), and a speckled pattern of antinuclear antibody (OR 5.62; 95% CI; 1.33–23.62). Multivariate analysis showed that mRSS (OR 1.34; 95% CI; 1.04–1.72) and the presence of a speckled antinuclear antibody (OR 25.99; 95% CI; 1.02–16.02) were independent predictors of ILD. The percentage of BMI lost in a period of time of 2008–2018 was 14.3% (p=0.0021), which was higher than rheumatoid arthritis (p=0.0000), systemic lupus erythematosus (p=0.0025), and healthy patients (p=0.0331). ConclusionBMI and ILD are inversely correlated. Age, underweight, higher mRSS, and speckled pattern of antinuclear antibody were risk factors for development of ILD in patients with systemic sclerosis. The mRSS could be used as a clinical predictive tool for ILD in systemic sclerosis.

Comprehensive review of current diagnostic and treatment approaches to interstitial lung disease associated with rheumatoid arthritis.

Interstitial lung disease (ILD) is one of the extra-articular involvement forms of rheumatoid arthritis (RA), and it is associated with increased mortality. The presence of genetic susceptibility, smoking, rheumatoid factor positivity, and the presence of anticitrulline peptide antibodies are factors contributing to the development of ILD in patients with RA. Early diagnosis and treatment of ILD contribute to the reduction of morbidity and mortality. We herein evaluated the current literature for the diagnosis and treatment of RA-associated ILD.

Diagnosis and Treatment of Connective Tissue Disease-Associated Interstitial Lung Disease

Interstitial lung disease (ILD) is one of the most serious pulmonary complications associated with connective tissue diseases (CTDs), resulting in significant morbidity and mortality. Although the various CTDs associated with ILD often are considered together because of their shared autoimmune nature, there are substantial differences in the clinical presentations and management of ILD in each specific CTD. This heterogeneity and the cross-disciplinary nature of care have complicated the conduct of prospective multicenter treatment trials and hindered our understanding of the development of ILD in patients with CTD. In this update, we present new information regarding the diagnosis and treatment of patients with ILD secondary to systemic sclerosis, rheumatoid arthritis, dermatomyositis and polymyositis, and Sjögren syndrome. We review information on risk factors for the development of ILD in the setting of CTD. Diagnostic criteria for CTD are presented as well as elements of the clinical evaluation that increase suspicion for CTD-ILD. We review the use of medications in the treatment of CTD-ILD. Although a large, randomized study has examined the impact of immunosuppressive therapy for ILD secondary to systemic sclerosis, additional studies are needed to determine optimal treatment strategies for each distinct form of CTD-ILD. Finally, we review new information regarding the subgroup of patients with ILD who meet some, but not all, diagnostic criteria for a CTD. A careful and systematic approach to diagnosis in patients with ILD may reveal an unrecognized CTD or evidence of autoimmunity in those previously believed to have idiopathic ILD.

Pulmonary involvement in polymyositis and dermatomyositis

Pulmonary complications play an important role in causing morbidity and mortality in myositis, and interstitial lung disease (ILD) has been reported in up to 65% of myositis patients. Clinical symptoms including cough and dyspnoea are common, but they are not reliable for ILD detection. The diagnosis should be based on pulmonary function tests, chest radiographs and high resolution computed tomography, which should all be regarded as standard procedures for the initial evaluation of all myositis patients. Biomarkers, in particular anti-aminoacyl tRNA synthetase antibodies, have been demonstrated to be useful in predicting the risk of ILD in patients with myositis. Glucocorticoids remain the mainstay of therapy in adult polymyositis and dermatomyositis patients with ILD, but most patients fail to fully respond to this treatment and require additional immunosuppressives such as methotrexate, cyclophosphamide, azathioprine and ciclosporin A. Other treatment options include intravenous γ-globulins, tacrolimus and CD20 monoclonal antibodies. Myositis is closely related to the antisynthetase syndrome, which also includes manifestations such as ILDs, arthritis, Raynaud’s phenomenon, fever and mechanic’s hands. In this syndrome, ILD is often the first manifestation of a chronic inflammatory disease. There is now also accumulating data to support a role of a T-cell- and B-cell-mediated immune response in the development of ILD in patients with anti-tRNA synthetase autoantibodies.

Gastroesophageal Reflux and Pulmonary Fibrosis in Scleroderma

Interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) is associated with increased morbidity and mortality. Gastroesophageal reflux (GER) is considered a contributing factor in the pathogenesis of ILD. To characterize GER (acid and nonacid) in patients with SSc with and without ILD. Patients with SSc underwent pulmonary high-resolution computer tomography (HRCT) scan and 24-hour impedance-pH monitoring off-proton pump inhibitor therapy. The presence of pulmonary fibrosis was assessed using validated HRCT-scores. Reflux monitoring parameters included number of acid and nonacid reflux episodes, proximal migration of the refluxate, and distal esophageal acid exposure. Unless otherwise specified, data are presented as median (25th-75th percentile). Forty consecutive patients with SSc (35 female; mean age, 53 yr; range, 24-71; 15 patients with diffuse and 25 with limited SSc) were investigated; 18 (45%) patients with SSc had pulmonary fibrosis (HRCT score >or= 7). Patients with SSc with ILD had higher (P < 0.01) esophageal acid exposure (10.3 [7.5-15] vs. 5.2 [1.5-11]), higher (P < 0.01) number of acid (41 [31-58] vs. 19 [10-23]) and nonacid (25 [20-35] vs. 17 [11-19]) reflux episodes, and higher (P < 0.01) number of reflux episodes reaching the proximal esophagus (42.5 [31-54] vs. 15 [8-22]) compared with patients with SSc with normal HRCT scores. Pulmonary fibrosis scores (HRCT score) correlated well with the number of reflux episodes in the distal (r(2) = 0.637) and proximal (r(2) = 0.644) esophagus. Patients with SSc with ILD have more severe reflux (i.e., more reflux episodes and more reflux reaching the proximal esophagus). Whether or not the development of ILD in patients with SSc can be prevented by reflux-reducing treatments needs to be investigated.

"Interstitial Lung disease (Ild) in Rheumatoid arthritis (Ra)"- a study of thirty cases

Objectives- The objective of this study is to evaluate the Interstitial Lung Disease (ILD) in the patients with Rheumatoid Arthritis (RA) and to correlate clinico - spirometric profile and High Resolution Computed Tomography (HRCT) findings with regards to early detection of possible lung involvement in the study group. Materials and Methods The present study was carried out at Shree Sayajirao General Hospital (SSGH), Baroda from January 2002 to December 2002. The patients satisfying the American College of Rheumatology (ACR) criteria [1] for RA were included in this study, without regards to the presence of pulmonary symptoms or chest x-ray changes. None of our patient was on Disease Modifying Anti-Rheumatic Drugs (DMARD) therapy. All thirty patients (seven males and twenty three females) - underwent clinical, spirometric, laboratory, x-ray chest and HRCT evaluation of lung. Diagnosis of ILD was confirmed by HRCT and supported by clinical and physiological findings in all patients. All the gathered information and investigations were subjected to statistical analysis. Results Majority of the patients had duration of illness between one and three years. The mean duration of illness was 2.9 years. RA factor was positive in 25 patients (83.3%). Six of the thirty patients had respiratory complaints (20%). Spirometric evidence of lung involvement was present in eight patients (26.6%). Only four of them had abnormalities detectable on chest radiograph (13.33%). HRCT was abnormal in eleven patients (36%). Conclusion In concluding the results of our study, the interstitial lung changes affects significant proportion (36%) of the patients affected with RA. Overall, HRCT is the most sensitive parameter to detect the early interstitial changes in patients with RA. HRCT can show evidence of interstitial lung changes even when clinical and pulmonary function tests parameters are normal. HRCT is superior to plain chest radiograph in the evaluation of early interstitial lung changes associated with RA. Coexisting subcutaneous rheumatoid nodules is a high-risk factor for development of ILD in patients with RA.