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 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 POS0755 PREVALENCE AND RELEVANCE OF ANTIBODIES AGAINST CITRULLINATED ALPHA ENOLASE (ANTI-CEP1) IN CONNECTIVE TISSUE DISEASES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 630.1-630
Author(s):  
A. Alunno ◽  
O. Bistoni ◽  
F. Carubbi ◽  
M. Antonucci ◽  
S. Calvacchi ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Bone Erosion ◽  
Disease Onset ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Igg Antibodies ◽  
Serum Samples ◽  
Healthy Donors

Background:Anti-citrullinated alpha enolase antibodies have been investigated in rheumatoid arthritis and associated with bone erosion and interstitial lung disease but little is known about their prevalence and role in connective tissue diseases (CTDs).Objectives:The aim of this study was to investigate the prevalence and relevance of anti-CEP1 antibodies in CTDs.Methods:Serum samples from five independent patient cohorts were assessed: 1) established (est) primary Sjogren’s syndrome (pSS) N=78, 2) est-systemic lupus erythematosus (SLE) N=52, 3) est-systemic sclerosis (SSc) N=71, 4) pSS at disease onset N=30, 5) SLE at disease onset N=46 (cohorts 4 and 5 had at least 3 years of follow-up). Samples from ninety sex and age matched healthy donors (HD) and 200 patients with est-RA (disease controls) were also tested. Anti-CEP1 IgG antibodies were measured with a commercially available ELISA kit (Euroimmun, Luebeck, Germany).Results:Anti-CEP1 titer was significantly higher in est-pSS, est-SLE and est-SSc compared to HD, significantly lower in est-pSS and est-SSc compared to est-RA and comparable in est-SLE versus est-RA. We divided patients in every CTD group based on whether their anti-CEP1 titer was below or above the 25th, 50th and 75th percentile. In est-SLE anti-CEP1 values over the 25th percentile were associated with articular involvement (odds ratio, OR (95% confidence interval, CI)=11.5; 1.9-70.6, p=0.008). In est-pSS, no relationship between anti-CEP1>25th percentile and articular involvement was found but rather an association with rheumatoid factor positivity (OR (95% CI)=4.8, 1.6-14.1, p=0.004) and salivary gland swelling (OR (95% CI)=6.2, 1.3-29.1, p=0.021). In est-SSc no difference could be detected across the 3 groups. Anti-CEP-1 titers in pSS and SLE at onset did not differ from each other, were comparable also to those of HD and significantly lower than those of est-pSS, est-SLE and est-RA patients (all p<0.0001).). Of interest, we could retrieve a serum sample collected at the time of diagnosis for 5 patients from the cohort of established pSS and we observed that anti-CEP1 titers were significantly lower at pSS onset than during follow up (at least 12 months after the diagnosis, p=0.0024). No difference was observed in the clinical presentation at disease onset according to different anti-CEP1 titer and they did not predict the development of new clinical manifestations during follow-up.Conclusion:Anti-CEP-1 antibodies can be detected in CTDs at different title during the disease course and may increase overtime, at least in pSS. Although anti-CEP1 antibodies are associated with specific clinical manifestation in est-CTDs, such as articular involvement in est-SLE, they seem to lack a predictive value for future manifestations when measured at disease onset.References:[1]Alunno A, Bistoni O, Pratesi F et al Rheumatology (Oxford) 2018.[2]Manca ML, Alunno A, D’Amato C et al. Joint Bone Spine 2018.Disclosure of Interests: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 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 Organizing pneumonia as the initial presentation in rheumatoid arthritis – A case report

2021 ◽  
Vol 5 (1) ◽  
pp. 051-053
Author(s):  
Kaur Harveen ◽  
Singh Dilbag ◽  
Pandhi Naveen
Keyword(s):  
Rheumatoid Arthritis ◽  
Case Report ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Injury ◽  
Lung Disease ◽  
Connective Tissue Diseases ◽  
Pulmonary Involvement ◽  
Organizing Pneumonia ◽  
Initial Manifestation

Organizing pneumonia (OP), can be seen in association with lung injury, infection, drug intoxication, and connective tissue diseases. Patients of rheumatoid arthritis (RA) are prone to develop interstitial lung disease (ILD), but the pulmonary involvement usually occurs several years after the joint manifestations. Only in about 10% cases of RA, the initial manifestation of the disease can be in the form of interstitial lung disease. OP as the initial manifestation of RA is extremely uncommon occurrence. Here is presented a case of 52-year-old male who presented with OP as the initial manifestation of RA. On investigation, the RA factor and anti-CCP Antibodies were positive. Based on clinical, radiological and histopathological findings the diagnosis was established.

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 Pulmonary rehabilitation in patients with interstitial lung disease associated with rheumatoid arthritis or connective tissue disease: From mechanisms to practice

2021 ◽  
Vol 16 (3) ◽  
pp. 341-345
Author(s):  
Ioana RUSU ◽  
◽  
Laura DAMIAN ◽  
Daisy Ana-Maria VAIDA VOEVOD ◽  
Romana VULTURAR ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Exercise Training ◽  
Lung Disease ◽  
Lung Diseases ◽  
Neuromuscular Electrical Stimulation ◽  
Connective Tissue Diseases ◽  
Respiratory Muscles ◽  
Physical Strength

Interstitial lung diseases are a large group of lung conditions characterized by inflammation and fibrosis. Interstitial lung disease in connective tissue diseases and rheumatoid arthritis is a severe complication of these pathologies. Apart from the pharmacological treatment, exercise training could help reduce breathing difficulties and increase physical strength, lower the burden of disease, help improving self-esteem and fight against depression and anxiety. Exercise training can be done either alone, or enhanced by auxiliary methods like oxygen administration, neuromuscular electrical stimulation or improving the strength of respiratory muscles. Possible associated lung diseases should be always taken into account. Even if exercise training is generally considered safe for this category of patients, there are also risks like exercise-induced hypoxemia, arrhythmia or pulmonary hypertension. Exercise training in this setting should be performed by dedicated physiotherapists after specialist prescription and under surveillance in a safe environment.

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 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

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