scholarly journals AB1136 THE DIAGNOSTIC VALUE OF SERUM KL-6 IN CONNECTIVE TISSUE DISEASE ASSOCIATED INTERSTITIAL LUNG DISEASE IN THE UYGUR POPULATION OF CHINA.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1858.2-1858
Author(s):  
X. Wu ◽  
L. Wu ◽  
C. N. Luo ◽  
Y. M. Shi
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Characteristic Curve ◽  
Connective Tissue Diseases ◽  
Nonsmall Cell Lung Cancer ◽  
Pulmonary Complications ◽  
Cross Sectional ◽  
Uygur Population

Background:Connective tissue diseases are a group of inflammatory, immune mediated disorders.Interstitial lung disease (ILD) is associated with significant morbidity and mortality.Currently,, scientists are still looking for serum markers to diagnose interstitial lung disease.Althought serum KL-6 level has been studied in ILD of various aetiologies and revealed to be an important serum marker for ILD,but differences in KL-6 expression related to ethnic and/or genetic variants may exist.Objectives:To evaluate the diagnosis of the serum Krebs von den Lungen-6 (KL-6) for CTD-ILD in the Uygur population of China.Methods:117 Patients with CTD-ILD (CTD-ILD group) and 182 patients with CTD (CTD group) who visited the department of rheumatology and immunology of People’s Hospital of Xinjiang Uygur Autonomous Region between January, 2015 and December, 2019 were included. Serum KL-6 levels were measured by chemiluminescent enzyme immunoassay kit.Results:The significantly higher levels of KL-6 were determined in the RA-ILD group than RA group [569(287.5,984)U/ml vs 194(152,266.5)U/ml](P<0.001)(figure 1).The optimal cutoff value of serum KL-6 for diagnosis of RA-ILD was 345.5 U/ml, and the sensitivity and specificity were71.8% and 90.1 %, respectively. Area Under the Curve (AUC) was 0.875.(figure 2)Figure 1.Comparison of serum KL-6 concentrations in CTD-ILD group and CTD group.Fig 2.Receiver-operating characteristic curve(ROC) of KL-6 for the diagnosis of CTD-ILDConclusion:The serum KL-6 is a important biomarker for the diagnosis of CTD-ILD and Serum KL-6 could be a clinically useful biomarker in screening CTD-ILD in the Uygur population of China.References:[1]Woodhead F, Wells A U, Desai S R. Pulmonary Complications of Connective Tissue Diseases[J]. Clinics in Chest Medicine, 2008, 29(29):149–164.Tanaka S, Hattori N, Ishikawa N, et al. Krebs von den Lungen-6 (KL-6) is a progn -ostic biomarker in patients with surgically resected nonsmall cell lung cancer. Int J Cancer 2012; 130:377–87.[2]Ogz E O, Kucuksahin O, Turgay M, et al. Association of serum KL-6 levels with interstitial lung disease in patients with connective tissue disease: a cross-sectional study. Clinical Rheumatology, 2016, 35(3):663-666.Disclosure of Interests:None declared

scholarly journals Therapeutic Options for the Treatment of Interstitial Lung Disease Related to Connective Tissue Diseases. A Narrative Review

2020 ◽  
Vol 9 (2) ◽  
pp. 407 ◽  
Author(s):  
Caterina Vacchi ◽  
Marco Sebastiani ◽  
Giulia Cassone ◽  
Stefania Cerri ◽  
Giovanni Della Casa ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Therapeutic Option ◽  
Inflammatory Myopathy ◽  
Connective Tissue Diseases ◽  
Immunosuppressive Drugs ◽  
Pulmonary Complications

Interstitial lung disease (ILD) is one of the most serious pulmonary complications of connective tissue diseases (CTDs) and it is characterized by a deep impact on morbidity and mortality. Due to the poor knowledge of CTD-ILD’s natural history and due to the difficulties related to design of randomized control trials, there is a lack of prospective data about the prevalence, follow-up, and therapeutic efficacy. For these reasons, the choice of therapy for CTD-ILD is currently very challenging and still largely based on experts’ opinion. Treatment is often based on steroids and conventional immunosuppressive drugs, but the recent publication of the encouraging results of the INBUILD trial has highlighted a possible effective and safe use of antifibrotic drugs as a new therapeutic option for these subjects. Aim of this review is to summarize the available data and recent advances about therapeutic strategies for ILD in the context of various CTD, such as systemic sclerosis, idiopathic inflammatory myopathy and Sjogren syndrome, systemic lupus erythematosus, mixed connective tissue disease and undifferentiated connective tissue disease, and interstitial pneumonia with autoimmune features, focusing also on ongoing clinical trials.

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.

Association of serum KL-6 levels with interstitial lung disease in patients with connective tissue disease: a cross-sectional study

2016 ◽  
Vol 35 (3) ◽  
pp. 663-666 ◽  
Author(s):  
Ekin Oktay Oguz ◽  
Orhan Kucuksahin ◽  
Murat Turgay ◽  
Mustafa Turgut Yildizgoren ◽  
Askin Ates ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cross Sectional

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) [...]

Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study

2012 ◽  
Vol 71 (12) ◽  
pp. 1966-1972 ◽  
Author(s):  
Ragnar Gunnarsson ◽  
Trond Mogens Aaløkken ◽  
Øyvind Molberg ◽  
May Brit Lund ◽  
Georg Karl Mynarek ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cross Sectional

scholarly journals FRI0571 THE DIAGNOSTIC VALUE OF KL-6 IN RHEUMATOID ARTHRITIS ASSOCIATED WITH INTERSTITIAL LUNG DISEASE IN XINJIANG OF CHINA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 887.2-888
Author(s):  
G. Chen ◽  
L. Wu
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Operating Characteristic ◽  
Characteristic Curve ◽  
Connective Tissue Diseases ◽  
Diagnostic Value ◽  
Operating Characteristic Curve ◽  
Xinjiang Of China

Background:Rheumatoid arthritis (RA) is a systemic inflammatory disease.Many researchers have observed that extra articular organs were highly involved in RA patients.The most common extra-articular manifestations were pulmonary involvement.Serum levels of KL-6 have been reported to be elevated in various ILD such as idiopathic pulmonary fibrosis, collagen vascular disease associated interstitial pneumonias, and other interstitial lung disorders.However, little is known regarding the usefulness of this biomarker in connective tissue diseases related interstitial lung diseases(CTD-ILD). Especially,the diagnostic value of KL-6 in interstitial lung disease associated with rheumatoid arthritis(RA-ILD) still has a dispute.Objectives:To assess the diagnosis of the serum Krebs von den Lungen-6(KL-6) for RA-ILD patients in Xinjiang of China.Methods:This retrospective study included 184 patients with RA in who visited the department of rheumatology and immunology of People’s Hospital of Xinjiang Uygur Autonomous Region between January, 2015 and December, 2019.The patients were divided into RA-ILD group(n=95) and RA group(n=89) according to the presence of ILD. Serum KL-6 concentration (U/mL) was measured using the chemiluminescent enzyme immunoassay kit.Results:The mean age(p < 0.001) and median value of CCP (p = 0.006) were significantly higher in the RA-ILD group.RA-ILD group had elevated serum KL-6 levels compared to RA group [447(281, 687)U/ml vs 195(151.5,265.5)U/ml](p < 0.001)(figure 1).According to the Receiver Operating Characteristic Curve (ROC) analysis, the area under the curve was of 0.879 and the optimal cut-off value of serum KL-6 to discriminate the presence of ILD was 277 U/ml, with sensitivity of 77.9%, specificity of 79.8%(figure 2).Figure 1.Comparison of serum KL-6 concentrations in RA-ILD group and RA group.Figure 2.Receiver-operating characteristic curve(ROC) of KL-6 for the diagnosis of RA-ILDConclusion:The present study confirms that KL-6 is a biological marker which is associated with RA-ILD. Furthermore, Patients with RA who are older and have a higher value of CCP are more likely to develop ILD.References:[1] Anaya JM, Diethelm L, Ortiz LA et al (1995) Pulmonary involvement in rheumatoid arthritis. Semin Arthritis Rheum 24:242–254[2] Bonella F, Costabel U (2014) Biomarkers in connective tissue disease-associated interstitial lung disease. Semin Respir Crit Care Med 35:181–200[3] Doishita S, Inokuma S, Asashima H et al (2011) Serum KL-6 level as an indicator of active or inactive interstitial pneumonitis associated with connective tissue diseases. Intern Med 50: 2889–2892Disclosure of Interests:None declared

scholarly journals Connective Tissue Disease, Interstitial Lung Disease, and Pulmonary Hypertension (CTD PH-ILD): A Distinct Entity and Potential Opportunity

2021 ◽  
Vol 20 (4) ◽  
pp. 109-118
Author(s):  
Denise Gabrielle A. Sese ◽  
Kristin B. Highland
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Treatment Options ◽  
Connective Tissue Diseases ◽  
Multisystem Disorder ◽  
Disease Pathogenesis ◽  
Increased Risk

Connective tissue diseases are a multisystem disorder that can cause impairments in quality of life, shorten life expectancy, and increase the risk of mortality at a younger age. These patients have an increased risk for the development of pulmonary hypertension through several mechanisms including pulmonary arterial hypertension and pulmonary hypertension associated with interstitial lung disease. This review aims to discuss the various presentations of connective tissue disease associated with pulmonary hypertension associated with interstitial lung disease, demographics, and survival. It gives an overview of accepted mechanisms of disease pathogenesis, discusses advances in diagnostics, and treatment options. Despite a deeper understanding of disease pathogenesis, treatment for this remains limited to prevention of disease progression. The identification of the primary disease driver requires careful evaluation of the disease phenotype and is a potential target for treatment and prevention of death.

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