scholarly journals SAT0595 INTERSTITIAL LUNG DISEASE IN SOUTH AFRICAN ADULTS PATIENTS WITH AUTOIMMUNE RHEUMATIC DISEASES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1256.2-1256
Author(s):  
E. Palalane ◽  
D. Alpizar-Rodriguez ◽  
S. Botha ◽  
Q. M. S. H. Said-Hartley ◽  
G. Calligaro ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Rheumatic Diseases ◽  
Pulmonary Function Tests ◽  
Multivariable Analysis ◽  
Connective Tissue Diseases ◽  
Radiological Features ◽  
Autoimmune Rheumatic Diseases

Background:Interstitial lung disease (ILD) is prevalent in patients with autoimmune rheumatic diseases (ARD), leads to significant morbidity and mortality, and is poorly characterized in South Africa.Objectives:To describe the clinical, serological and radiological features of ILD associated with ARD in a tertiary referral hospital.Methods:A cross-sectional study of outpatients attending the rheumatology and respiratory clinics of Groote Schuur Hospital between October 2018 and September 2019. Clinical, serological and radiological features were documented. We compared features of 3 groups of patients: rheumatoid arthritis (RA), systemic sclerosis (SSc) and “Other” autoimmune rheumatic diseases (OARD) which included Idiopathic Inflammatory Myopathies, Mixed Connective Tissue Disease, Systemic Lupus Erythematous, ANCA-associated vasculitis, Sjogren’s Syndrome and overlap syndromes. Factors associated with Usual Interstitial Pneumonia (UIP) were sought by univariate and multivariate analysis. P-values ≤ 0.05 were considered statistically significant. Analyses was performed with STATA 14.0 (Stata Corp LP, USA).Results:Of 124 patients, 29.8 % had RA, 25,8 % SSc and 44.4 % OARD. Most patients were female (86.3%), of mixed racial ancestry (75.0%), and the median (IQR) age was 55 (46-66). Over one-third were smokers, 22.6% had emphysema, and one third had previous pulmonary tuberculosis (PTB) infection. Smoking, emphysema, and previous PTB were higher in RA group but the difference was not statistically significant. All SSc patients and more than two-thirds of RA and OARD patients had gastroesophageal reflux disease (GORD).Similar to reports elsewhere, Nonspecific interstitial pneumonia (NSIP) was the commonest ILD (63.7 %), followed by UIP (26.6%) and other patterns (9.7%). Contrary to other reports, we found similar frequencies of NSIP and UIP patterns in patients with RA. RA patients were significantly older (median (IQR)) age at ILD onset 62 (55-68) years, compared to SSc (49 (38-56) and OARD (42 (33-56) (p < 0.001). The percentage of predicted Forced Vital Capacity (FVC) were significantly worse in SSc and OARD groups and DLCO in OARD. RA diagnosis (OR 3.8, 95% CI 1.5-9.5), older age (0R 1.1, 95% CI 1.0-1.1), COPD (OR 3.2, 95% CI 1.4-8.0), longer ARD-ILD interval, higher FVC (OR 1.0, 95% CI 1.0-1.1) and previous Methotrexate (MTX) use (OR 2.6, 95% CI 1.1-6.0) were significantly associated with UIP. Multivariable analysis showed that only COPD and previous MTX use was associated with UIP (OR 2.8 (95% CI 1.0 – 8.0) and 1.0 (95% CI 1.0 – 1.0) respectively).Regarding MTX exposure, 37.1% of patients were prescribed MTX before ILD diagnosis, and 33.9% continued, started or restarted after ILD diagnosis. No case of acute pneumonitis was documented.Conclusion:ILD was most commonly diagnosed in RA and SSc, with NSIP seen most frequently overall. RA patients presented better Pulmonary function tests despite higher frequency of UIP. The use of MTX seems to not be associated with the development of acute pneumonitis in patients with ILDReferences:[1]Wallace, B., D. Vummidi, and D. Khanna,Management of connective tissue diseases associated interstitial lung disease: a review of the published literature.Current Opinion in Rheumatology, 2016.28(3): p. 236-245.[2]Dellaripa, P.F.,Interstitial lung disease in the connective tissue diseases; a paradigm shift in diagnosis and treatment.Clinical Immunology, 2018.186: p. 71-73.Disclosure of Interests:None declared

scholarly journals 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

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

scholarly journals A study of respiratory system involvement in autoimmune connective tissue diseases: a marker of morbidity

2020 ◽  
Vol 6 (4) ◽  
pp. 532
Author(s):  
Neha T. Solanki ◽  
Sahana P. Raju ◽  
Deepmala Budhrani ◽  
Bharti K. Patel
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Tertiary Care ◽  
Connective Tissue Diseases ◽  
Cutaneous Manifestations ◽  
Respiratory Involvement

<p class="abstract"><strong>Background:</strong> The auto-immune connective tissue diseases (AICTD) are polygenic clinical disorders having heterogeneous overlapping clinical features. Certain features like autoimmunity, vascular abnormalities, arthritis/arthralgia and cutaneous manifestations are common to them. Lung involvement can present in AICTDs in form of: pleurisy, acute/ chronic pneumonitis, pulmonary artery hypertension (PAH), shrinking lung syndrome, diffuse alveolar damage, pulmonary embolism (PE), bronchiolitis obliterans organizing pneumonia, pulmonary infections, cardiogenic pulmonary edema, etc. High-resolution computed tomography (HRCT) plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease.</p><p class="abstract"><strong>Methods:</strong> The present study is an observational study carried out on 170 patients of AICTD in department of Dermatology, Venereology and Leprosy at a tertiary care centre during a period of 2 years from October 2017 to August 2019. Detailed history, examination and relevant investigations like chest X-ray, pulmonary function test (PFT), HRCT thorax were done as indicated.<strong></strong></p><p class="abstract"><strong>Results:</strong> The overall incidence of respiratory involvement was 56.7% with maximum involvement in systemic sclerosis cases (82.8% of cases). 45.7% of patients of systemic lupus erythematosus had respiratory involvement, most common being pleural effusion in 11.5%. Impaired PFT’s were seen in 82.8% cases of systemic sclerosis (SSc)  and all cases of UCTD. Interstitial lung disease was seen in 34.7% and 25% cases of SSc and DM respectively. PAH was found in 15.2% cases of SSc and 9.8% cases of mixed connective tissue diseases.</p><p class="abstract"><strong>Conclusions:</strong> AICTD are multisystem disorders in which pulmonary involvement can be an important cause of morbidity to the patient and early detection is necessary for prevention of long-term respiratory complications.</p>

scholarly journals SAT0582 RISK OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC AUTOIMMUNE RHEUMATIC DISEASES: A NATIONWIDE, POPULATION-BASED COHORT STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1250.1-1250
Author(s):  
H. H. Chen ◽  
K. H. Ng ◽  
W. C. Chao ◽  
C. H. Lin
Keyword(s):  
Cohort Study ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Rheumatic Diseases ◽  
Population Based ◽  
Autoimmune Rheumatic Diseases ◽  
Comparison Groups

Background:To date, very few studies had investigated the epidemiology of interstitial lung disease (ILD) among patients with systemic autoimmune rheumatic disease (SARD).Objectives:To study the risk of interstitial lung disease (ILD) among patients with various systemic autoimmune rheumatic diseases (SARDs) including rheumatoid arthritis (RA), dermatomyositis (DMtis), polymyositis (PM), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and primary Sjögren’s syndrome (pSS).Methods:Using 1997–2013 claims data from the Taiwanese National Health Insurance Research Database, we identified 63,277 newly diagnosed patients with various SARDs after excluding those with overlapping SARD diagnoses from 2001-2013 and randomly selected 253,108 non-SARD subjects matching (1:4) SARD patients for SARD diagnosis, age, sex and the year of the index date. We calculated the incidence rates (IRs) of ILD (ICD-9 code 515) in various SARD groups and the corresponding non-SARD comparison groups and estimated the IR ratios (IRRs) with 95% confidence intervals (CI) of ILD development. Using multivariable cox regression analyses, we estimated hazard ratios (HRs) with 95% CIs of ILD in various SARD groups compared with their comparison groups after adjusting for age, sex, Charlson comorbidity index, amiodarone use and methotrexate use. Sensitivity analyses were conducted by using a narrow definition of ILD.Results:As shown in Table 1, the IRs of ILD were greatest in SSc patients (2,523 per 105years), followed by patients with DMtis (2,463 per 105years), PM (1,956 per 105years), SS (601 per 105years), RA (279 per 105years), and SLE (276 per 105years). Multivariable analyses showed that the risks of ILD were significantly increased in patients with SSc (HR, 66.01; 95% CI, 32.73—133.13), DMtis (128.74, 95% CI, 40.19—412.47), PM (HR, 30.39; 95% CI, 11.24—82.15), pSS (HR, 8.76; 95% CI, 7.03—10.90), RA (HR, 4.22; 95% CI, 3.51—5.08), and SLE (HR, 13.98; 95% CI, 9.25—21.14).Conclusion:This nationwide, population-based, matched cohort study demonstrated that the risks of ILD were significantly increased in patients with SARDs.References:[1]Su R, Bennett M, Jacobs S, Hunter T, Bailey C, Krishnan E, et al. An analysis of connective tissue disease-associated interstitial lung disease at a US Tertiary Care Center: better survival in patients with systemic sclerosis. The Journal of rheumatology. 2011;38(4):693-701.[2]Hu Y, Wang LS, Wei YR, Du SS, Du YK, He X, et al. Clinical Characteristics of Connective Tissue Disease-Associated Interstitial Lung Disease in 1,044 Chinese Patients. Chest. 2016;149(1):201-8.Araki T, Putman RK, Hatabu H, Gao W, Dupuis J, Latourelle JC, et al. Development and Progression of Interstitial Lung Abnormalities in the Framingham Heart Study. Am J Respir Crit Care Med 2016;194:1514-1522.Disclosure of Interests:None declared

scholarly journals Morphologic Aspects of Interstitial Pneumonia With Autoimmune Features

2018 ◽  
Vol 142 (9) ◽  
pp. 1080-1089 ◽  
Author(s):  
Ellen Caroline Toledo do Nascimento ◽  
Bruno Guedes Baldi ◽  
Marcio Valente Yamada Sawamura ◽  
Marisa Dolhnikoff
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
Distinct Subgroup ◽  
Management Protocol ◽  
The Impact

Context.— Interstitial lung disease, a common complication observed in several connective tissue diseases, causes significant morbidity and mortality. Similar to individuals with connective tissue diseases, a significant subgroup of patients with clinical and serologic characteristics suggestive of autoimmunity but without confirmed specific connective tissue disease presents with associated interstitial lung disease. These patients have been classified using different controversial nomenclatures, such as undifferentiated connective tissue disease–associated interstitial lung disease, lung-dominant connective tissue disease, and autoimmune featured interstitial lung disease. The need for a better understanding and standardization of this entity, interstitial lung disease with autoimmune features, and the need for an adequate management protocol for patients resulted in the introduction of a new terminology in 2015: interstitial pneumonia with autoimmune features. This new classification requires a better comprehension of its diagnostic impact and the influence of its morphologic aspects on the prognosis of patients. Objective.— To review the diagnostic criteria for interstitial pneumonia with autoimmune features, with an emphasis on morphologic aspects. Data Sources.— The review is based on the available literature, and on pathologic, radiologic, and clinical experience. Conclusions.— The interstitial pneumonia with autoimmune features classification seems to identify a distinct subgroup of patients with different prognoses. Studies show that nonspecific interstitial pneumonia and usual interstitial pneumonia are the most prevalent morphologic patterns and show discrepant results on the impact of the usual interstitial pneumonia pattern on survival. Prospective investigations are necessary to better define this subgroup and to determine the prognosis and appropriate clinical management of these patients.

scholarly journals Interstitial Lung Disease in Rheumatoid Arthritis in the Era of Biologics

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
A. Picchianti Diamanti ◽  
V. Germano ◽  
E. Bizzi ◽  
B. Laganà ◽  
A. Migliore
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Pulmonary Function Tests ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
Causative Role ◽  
Pulmonary Damage ◽  
Definitive Statement

Interstitial lung disease (ILD) represents a severe manifestation in connective tissue diseases (CTD), with an overall incidence of 15%, and it is still a challenge for clinicians evaluation and management. ILD is the most common manifestation of lung involvement in Rheumatoid Arthritis (RA), observed in up to 80% of biopsies, 50% of chest Computed Tomography (CT) and only 5% of chest radiographs. Histopatological patterns of ILD in RA may present with different patterns, such as: usual interstitial pneumonia, non specific interstitial pneumonia, desquamative interstitial pneumonia, organizing pneumonia, and eosinophilic infiltration. The incidence of ILD in RA patients is not only related to the disease itself, many drugs may be in fact associated with the development of pulmonary damage. Some reports suggest a causative role for TNFαinhibitors in RA-ILD development/worsening, anyway, no definitive statement can be drawn thus data are incomplete and affected by several variables. A tight control (pulmonary function tests and/or HRCT) is mandatory in patients with preexisting ILD, but it should be also performed in those presenting risk factors for ILD and mild respiratory symptoms. Biologic therapy should be interrupted, and, after excluding triggering infections, corticosteroids should be administered.

scholarly journals Diagnosis, Clinical Features and Management of Interstitial Lung Diseases in Rheumatic Disorders: Still a Long Journey

2022 ◽  
Vol 11 (2) ◽  
pp. 410
Author(s):  
Marco Sebastiani ◽  
Caterina Vacchi ◽  
Giulia Cassone ◽  
Andreina Manfredi
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Clinical Features ◽  
Lung Diseases ◽  
Connective Tissue Diseases ◽  
Interstitial Lung Diseases ◽  
Pulmonary Complications ◽  
Autoimmune Rheumatic Diseases

Interstitial lung disease (ILD) is one of the most frequent pulmonary complications of autoimmune rheumatic diseases (ARDs), and it is mainly associated with connective tissue diseases (CTDs) and rheumatoid arthritis (RA) [...]

scholarly journals Interstitial Pneumonia with Autoimmune Features: Why Rheumatologist-Pulmonologist Collaboration Is Essential

Biomedicines ◽  
2020 ◽  
Vol 9 (1) ◽  
pp. 17
Author(s):  
Marco Sebastiani ◽  
Paola Faverio ◽  
Andreina Manfredi ◽  
Giulia Cassone ◽  
Caterina Vacchi ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Task Force ◽  
Systemic Vasculitis ◽  
Pulmonary Function Tests ◽  
Classification Criteria ◽  
European Respiratory Society

In 2015 the European Respiratory Society (ERS) and the American Thoracic Society (ATS) “Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease” proposed classification criteria for a new research category defined as “Interstitial Pneumonia with Autoimmune Features” (IPAF), to uniformly define patients with interstitial lung disease (ILD) and features of autoimmunity, without a definite connective tissue disease. These classification criteria were based on a variable combination of features obtained from three domains: a clinical domain consisting of extra-thoracic features, a serologic domain with specific autoantibodies, and a morphologic domain with imaging patterns, histopathological findings, or multicompartment involvement. Features suggesting a systemic vasculitis were excluded. Since publication of ERS/ATS IPAF research criteria, various retrospective studies have been published focusing on prevalence; clinical, morphological, and serological features; and prognosis of these patients showing a broad heterogeneity in the results. Recently, two prospective, cohort studies were performed, confirming the existence of some peculiarities for this clinical entity and the possible progression of IPAF to a defined connective tissue disease (CTD) in about 15% of cases. Moreover, a non-specific interstitial pneumonia pattern, an anti-nuclear antibody positivity, and a Raynaud phenomenon were the most common findings. In comparison with idiopathic pulmonary fibrosis (IPF), IPAF patients showed a better performance in pulmonary function tests and less necessity of oxygen delivery. However, at this stage of our knowledge, we believe that further prospective studies, possibly derived from multicenter cohorts and through randomized control trials, to further validate the proposed classification criteria are needed.

scholarly journals INTERSTITIAL PULMONARY FIBROSIS ASSOCIATED CONNECTIVE TISSUE DISEASES – CLINICAL AND IMAGING ASPECTS

2017 ◽  
Vol 26 (2) ◽  
pp. 71-76
Author(s):  
Madalina Gheorghe ◽  
◽  
Violeta Claudia Bojinca ◽  
Ruxandra Ionescu ◽  
◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Lupus Erythematosus ◽  
Pulmonary Function Tests ◽  
Connective Tissue Diseases ◽  
Common Site ◽  
High Resolution Computed Tomography ◽  
Interstitial Pulmonary Fibrosis ◽  
Randomized Controlled

The lung is a common site of complications in systemic connective tissue diseases (CTD), and its involvement can present in several ways. Although it is generally thought that interstitial lung disease develops later on in CTD, it is often the initial presentation (“lung dominant” CTD). Interstitial lung disease (ILD) can be present in most types of CTD, including rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjögren’s syndrome and mixed connective tissue disease. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD. Interstitial lung disease must be detected early in the course of collagen disorders by performing high-resolution computed tomography and pulmonary function tests. The pattern described on HRCT is predictive for treatment response and disease progression. Immunosuppression is the mainstay of treatment for ILD, although data from randomized controlled trials (RCTs) to support specific treatments are lacking. The management of patients with CTD-associated ILD is optimized by multidisciplinary collaboration.

Feasibility, face, and content validity of quantitative computed tomography in interstitial lung disease related to connective tissue diseases

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Gianluca Sambataro ◽  
Domenico Sambataro ◽  
Martina Orlandi ◽  
Sofia Battisti ◽  
Lorenzo Cavagna ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Content Validity ◽  
Online Survey ◽  
Objective Assessment ◽  
Quantitative Computed Tomography ◽  
Connective Tissue Diseases ◽  
Face Validity

Abstract Objectives Quantitative computed tomography (QCT) is a promising tool for objective assessment of interstitial lung disease (ILD) related to connective tissue diseases (CTD). However, its validity was never investigated. The aim of this study was to assess QCT feasibility, face, and content validity evaluation concerning CTD-ILD. Methods A rheumatologist and a chest radiologist conceived an online survey with nine statements: Two about general issue involving CTD-ILD, one for the face validity, three both for content validity and feasibility. Each statement had to be rated with a score from 0 to 100, respectively, (complete disagreement and agreement). We considered a statement supported by the experts if the median score was ≥75.0. Results A panel of 14 experts (11 from Europe, three from America) with a nine years median experience was enrolled. All the statements about feasibility, face and content validity were supported, except for QCT capability to recognize elementary lesions. Conclusions The panel of experts supported feasibility, face, and content validity of QCT assessment concerning CTD-ILD. This may stimulate a greater use in clinical practice and further studies to confirm its discriminative properties and its construct validity.

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