scholarly journals The Association between MUC5B Rs35705950 and Risks of Idiopathic Interstitial Pneumonia, Systemic Sclerosis Interstitial Lung Disease, and Familial Interstitial Pneumonia: A Meta-Analysis

2020 ◽  
Author(s):  
Huai-Qing LOU ◽  
Chun-Xia HUANG ◽  
Guang-Yi LI ◽  
Ping LI ◽  
Shou-Ming ZHANG ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Meta Analysis ◽  
Biomedical Literature ◽  
Idiopathic Interstitial Pneumonia ◽  
Normal Lung ◽  
Cochrane Central Register

Background: Interstitial lung disease (ILD) is a category of chronic lung diseases with more than 200 subtypes. Idiopathic interstitial pneumonia (IIP), systemic sclerosis (SSc) ILD, and familial interstitial pneumonia (FIP) are three major groups of lung diseases with different causes or with unknown causes. Mucin5B (MUC5B) belongs to the mucin family, which contribute to the lubricating and viscoelastic properties of the whole saliva, normal lung mucus, and cervical mucus. The association between MUC5B rs35705950 and ILDs risks has been widely studied. However, the results were inconclusive and inconsistent. Methods: In the present meta-analysis, the database PubMed, Embase, Cochrane Central Register of Controlled Trials, CNKI and Chinese Biomedical Literature Database were searched till Aug 20th, 2018. Overall 16 publications with 28 studies, 76345 cases and 18402 controls were included. Results: The results indicated a significant increase of overall IIP risk for TT genotype and T allele of the rs35705950 in all genetic models (TT vs GG, OR=9.11; TT vs GT+TT, OR=5.80; GT+TT vs GG, OR=4.34; T vs G, OR=4.03. P<0.0001). Subgroup analysis by subtypes of IIP revealed higher risks of TT genotype and T allele for IPF and iNSIP (P<0.05). A significant increase of FIP risk was also found for the TT genotype and T allele of the rs35705950 (TT vs GG, OR=17.08; GT+TT vs GG, OR=6.02; T vs G, OR=1.64.P<0.05). Conclusion: No significant relations existed between the rs35705950 and SSc-ILD risks. MUC5B rs35705950 might be a predictor for the susceptibility of IIP and FIP.

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 Radiological diagnosis of interstitial lung diseases

2013 ◽  
Vol 66 (suppl. 1) ◽  
pp. 29-33
Author(s):  
Ruza Stevic ◽  
Vucinic Mihailovic ◽  
Dragana Jovanovic ◽  
Nada Vasic
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Usual Interstitial Pneumonia ◽  
Interstitial Lung Diseases ◽  
Ground Glass ◽  
Cryptogenic Organizing Pneumonia ◽  
Acute Interstitial Pneumonia ◽  
Nonspecific Interstitial Pneumonia

Introduction. Interstitial lung diseases include the entities of idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. Recent introduction of high-resolution computed tomography has made the diagnosis of interstitial lung disease much easier. Usual interstitial pneumonia A predominantly subpleural reticulation and honeycombing at the basal posterior part of the lung with a progression to anterior and superior parts are characteristic of usual interstitial pneumonia/ idiopathic pulmonary fibrosis. Nonspecific interstitial pneumonia Typical findings of nonspecific interstitial pneumonia are bilateral, relatively symmetrical subpleural ground glass opacifications and irregular linear opacities. Desquamative interstitial pneumonia is characterized by diffuse symmetrical ground glass opacifications. Respiratory bronchiolitisassociated interstitial lung disease Centrilobular nodules and irregular ground glass opacifications are present. Cryptogenic organizing pneumonia Subpleural and peribronchial consolidations are prominent findings that are not present in other idiopathic interstitial pneumonias. Acute interstitial pneumonia. Bilateral ground-glass opacifications are present and areas of peripheral consolidations may also be seen in acute interstitial pneumonia. Lymphocytic interstitial pneumonia. Diffuse or patchy areas of ground glass opacification with centrilobular nodules and occasionally well-defined cysts are seen. Conclusion. Imaging plays a crucial role in identifying interstitial lung diseases but precise diagnosis requires a dynamic interdisciplinary approach that correlates clinical, radiological and pathologic features.

scholarly journals POS0863 DIFFERENTIATING THE DOMINANCE OF PULMONARY VASCULAR DISEASE OR INTERSTITIAL LUNG DISEASE ON HEMODYNAMIC ABNORMALITIES IN SYSTEMIC SCLEROSIS AND CLARIFYING EACH CHARACTERISTIC BY USING QUANTITATIVE EVALUATION OF CHEST CT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 687.1-687
Author(s):  
K. Ninagawa ◽  
M. Kato ◽  
M. Kono ◽  
Y. Fujieda ◽  
K. Oku ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Quantitative Evaluation ◽  
Lung Volume ◽  
Chest Ct ◽  
Normal Lung ◽  
Pulmonary Vascular Disease ◽  
Group 1

Background:Group 1 and 3 pulmonary hypertension (PH) develop through different pathological mechanisms but have similar hemodynamic abnormalities. Systemic sclerosis (SSc) is associated with both pulmonary vascular disease (PVD) and interstitial lung disease (ILD), making it challenging to differentiate group 1 and 3 PH in those patients. A previous study using quantitative evaluation of chest computed tomography (CT) demonstrated that normal lung volume was inversely correlated with mean pulmonary arterial pressure (mPAP) in patients with group 3 PH (1).Objectives:In this study, we aimed to assess the dominance of PVD or ILD in SSc patients by quantitative evaluation of chest CT and to evaluate each characteristic.Methods:A total of 76 SSc patients who underwent right heart catheterization (RHC) were included. Chest CT was evaluated by using a software (Synapse Vincent Ver.3.0, Fujifilm) which quantified normal and total area of the lung. Then, we calculated abnormal area by drawing normal area from total area in the lung (%). Pulmonary function test (PFT) and serum biomarkers, such as KL-6 and LDH, were also evaluated. The dominance of PVD or ILD was defined as divergent or parallel change between the first and last assessments, respectively, in mPAP and abnormal area in the lung calculated using the software. Increase or decrease by over 10% in the last assessment compared to the first assessment was considered as a significant change in mPAP or abnormal area in the lung. P values were calculated by Mann-Whitney U test, and correlation coefficients were calculated by direct regression variance.Results:The median [range] values of mPAP and abnormal area in the lung at baseline were 23 [9-65] mmHg and 30.2 [0-100] %, respectively. Of 37 SSc and PH patients, 18 were defined as having PVD dominance while 19 as ILD dominance. Abnormal area in the lung at baseline was greater in patients with ILD dominance compared to those with PVD dominance (39.1 [16.3-98.3] v.s. 14.0 [0-99] %, p=0.002), whereas mPAP was higher in patients with PVD dominance than those with ILD dominance (42.5 [23.0-65.0] v.s. 26.0 [16.0-42.0] mmHg, p=0.002). PFT parameters including forced vital capacity were not different between the two groups. The ratio of mPAP/KL-6 showed a great difference between the two groups with its significant elevation in patients with PVD dominance (p=0.007).Conclusion:Quantitative evaluation of chest CT showed great efficiency in differentiating the dominance of PVD or ILD in patients with SSc and PH. In addition, the ratio of mPAP/KL-6 may easily be used as a parameter for dominance evaluation.References:[1]Iwasawa T, Kato S, Ogura T, Kusakawa Y, Iso S, Baba T, et al. Low-normal lung volume correlates with pulmonary hypertension in fibrotic idiopathic interstitial pneumonia: computer-aided 3D quantitative analysis of chest CT. AJR Am J Roentgenol. 2014;203(2):W166-73.Disclosure of Interests:None declared

Treatment of systemic sclerosis-associated interstitial lung disease: A systematic literature review and meta-analysis

2020 ◽  
Vol 27 ◽  
pp. 146-169
Author(s):  
Jorge Bruce Flórez-Suárez ◽  
Kevin Maldonado-Cañón ◽  
Jenny Londoño ◽  
Paul Méndez-Patarroyo ◽  
Gerardo Quintana-López
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Literature Review ◽  
Lung Disease ◽  
Systematic Literature Review ◽  
Meta Analysis
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