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 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 Serum KL-6 as predictive and prognostic marker of interstitial lung disease in childhood connective tissue diseases: a pilot study

Reumatismo ◽  
2021 ◽  
Vol 73 (3) ◽  
Author(s):  
R. El-Beheidy ◽  
A.M. Domouky ◽  
H. Zidan ◽  
Y.A. Amer
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Control Group ◽  
Connective Tissue Disorders ◽  
Juvenile Systemic Lupus Erythematosus ◽  
Median Serum ◽  
Juvenile Systemic Sclerosis

This study was aimed to evaluate serum KL-6 levels to determine if this marker can be used for diagnosing and assessing severity of interstitial lung disease (ILD) in children with connective tissue disorders. In total, 40 patients [18 patients with juvenile systemic lupus erythematosus (JSLE), 10 patients with juvenile idiopathic arthritis (JIA), 8 patients with juvenile mixed connective tissue disease (JMCTD), 3 patients with juvenile systemic sclerosis (JSSc), and 1 patient with juvenile dermatomyositis (JDM)] and 20 healthy controls were included in this study. Age, sex, and duration of CTD and ILD (if any) were recorded. Blood samples from all the patients and controls were examined by ELISA. 20 of the 40 patients with CTD (50%) had ILD, 12 were mild and 8 were severe as assessed by spirometry. The median serum KL-6 level was 102.7 U/mL (76.1-180.8) in the CTD with severe ILD group, 72.2 U/mL (58.4- 100.5) in the CTD with mild ILD group, 56.7 U/mL (35.8-68.5) in the CTD without ILD group, and 52.3 U/mL (32.8-62.4) in the control group. KL-6 levels were significantly higher in the CTD with ILD (p<0.05), at a cutoff of 63.4 U/ml identified by ROC curve, serum KL-6 showed a sensitivity of 95.2% and specificity of 89.7%. KL-6 is a valuable biomarker for diagnostic purposes and to detect severity in ILD in childhood CTD.

scholarly journals Interstitial lung disease in mixed connective tissue disease

2018 ◽  
Vol 9 (1) ◽  
Author(s):  
Marshell Tendean ◽  
Sazkia Aziza Nuriawan ◽  
Pringgodigdo Nugroho
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
Connective Tissue Diseases ◽  
Severe Pneumonia ◽  
Interstitial Lung Diseases ◽  
Pulmonary Complications ◽  
Initial Symptoms ◽  
Screening And Prevention

Interstitial lung diseases (ILD) are known as a debilitating pulmonary complications that may be occured in almost all systemic connective tissue diseases (CTD), including mixed connective tissue disease (MCTD). ILD is usually found in more than half of MCTD patients after 2-4years after the diagnosis made. A-47-years-old female initially diagnosed as systemic lupus erythematosus (SLE) developed a severe progressive dyspnea. She has recently diagnosed as MCTD with ILD after 9 months of initial symptoms. She was giving with Cyclophosphamide 500 mg IV pulse dose. However, after 1 months she developed severe pneumonia andpronounced demise due to intractable septic shock. The debilitating course of ILD is commonly seen in most systemic CTD. Therefore, it is important to perform initial screening and prevention. Systemic corticosteroid with or without immunosupressor agent(s) are indicated inILD-MCTD. Patients with progressive diseases will have poor prognosis.Keywords : ILD, MCTD, Corticosteroid

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 FRI0565 PREVALENCE AND SIGNIFICANCE OF ANTIBODIES AGAINST CITRULLINATED ALPHA-ENOLASE (ANTI-CEP1) IN CONNECTIVE TISSUE DISEASES.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 885.2-885
Author(s):  
A. Alunno ◽  
F. Carubbi ◽  
O. Bistoni ◽  
M. Antonucci ◽  
E. Bartoloni Bocci ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Lupus Erythematosus ◽  
Bone Erosion ◽  
Connective Tissue Diseases ◽  
Serum Samples ◽  
Erosive Disease ◽  
Normal Controls

Background:Anti-cyclic citrullinated peptide (anti-CCP) auto-antibodies represent the current gold standard for the diagnosis of rheumatoid arthritis (RA). However, growing evidence suggests that a variety of other citrullinated self-proteins may act as autoantigens and lead to the production of autoantibodies (1). Furthermore, autoantibodies believed to be RA-specific have been detected also in patients with connective tissue diseases (CTDs). We recently demonstrated that antibodies against citrullinated alpha-enolase (anti-CEP1) are a biomarker of erosive disease and RA-associated interstitial lung disease (2).Objectives:The purpose of this study was to investigate the prevalence and possible prognostic value of anti-CEP-1 in patients with CTDs.Methods:Two hundred and twelve consecutive patients with CTDs (51 systemic lupus erythematosus (SLE), 85 primary Sjogren’s syndrome (pSS) and 76 systemic sclerosis (SSc)) were studied and compared to 97 sex and age matched normal controls (NC) and 267 patients with RA. Anti-CEP1 IgG were detected in serum samples with a commercial ELISA kit (Euroimmun).Results:The overall prevalence of anti-CEP1 in CTDs was 7% (15/212 patients). In detail, these antibodies were detectable in 4 out of 85 pSS (5%), 5 out of 51 SLE (10%) and 6/76 SSc (8%). The prevalence and the titer of anti-CEP1 in CTDs was significantly higher compared to NC and significantly lower compared to RA. Anti-CEP1 positive patients did not display a specific clinical and serological picture. Unlike in RA, anti-CEP1 did not correlate with CTD-associated ILD.Conclusion:This is the first study assessing anti-CEP1 in a large cohort of patients with CTDs. We demonstrated that the association of these autoantibodies with ILD is specific for RA since it is not observed in SLE, pSS and SSc. Furthermore, although being significantly more prevalent and at higher titer compared to NC, anti-CEP1 do not allow to discriminate different patient subsets displaying peculiar clinical or serological phenotypes. Based on our results, the application of anti-CEP1 in CTDs is not advisable, however larger studies may possibly identify correlations not evident in our cohort.References:[1] Bonifacio AF, Alunno A, La Paglia GMC, Valentini E, Leone MC, Bartoloni E, Gerli R. Novel autoantibodies in rheumatoid arthritis. Reumatismo 2019;71(1):1-12[2] Alunno A, Bistoni O, Pratesi F, La Paglia GMC, Puxeddu I, Migliorini P, Gerli R. Anti-citrullinated alpha enolase antibodies, interstitial lung disease and bone erosion in rheumatoid arthritis. Rheumatology (Oxford). 2018;57(5):850-855Disclosure of Interests:Alessia Alunno: None declared, Francesco Carubbi Speakers bureau: Francesco Carubbi received speaker honoraria from Abbvie and Celgene outside this work., Onelia Bistoni: None declared, Matteo Antonucci: None declared, Elena Bartoloni Bocci: None declared, Roberto Giacomelli Grant/research support from: Actelion, Pfizer, Speakers bureau: Abbvie, Roche, Actelion, BMS, MSD, Ely Lilly, SOBI, Pfizer, Roberto Gerli: None declared

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 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.

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.

Sign in / Sign up

Export Citation Format

Share Document