scholarly journals POS0789 APPLICATION OF THE NEW ACR/EULAR 2019 CLASSIFICATION CRITERIA OF SYSTEMIC LUPUS ERYTHEMATOSUS TO A INCEPTION, MONOCENTRIC COHORT OF UNDIFFERENTIATED CONNECTIVE TISSUE DISEASES POPULATION AT ONSET OF THE DISEASE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 647.1-647
Author(s):  
E. Elefante ◽  
A. Parma ◽  
V. Nannipieri ◽  
V. Signorini ◽  
C. Stagnaro ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Classification Criteria ◽  
Inception Cohort ◽  
Systemic Lupus ◽  
New Classification ◽  
Undifferentiated Connective Tissue Diseases ◽  
Over Time

Background:undifferentiated connective tissue diseases (UCTDs) are a group of systemic autoimmune diseases that share clinical and serological manifestations with definite connective tissue diseases (CTDs), but do not satisfy existing classification criteria1. Within this group, it is possible to identify very heterogeneous conditions: transitory and self-limiting forms, stable conditions over time and forms that will evolve towards definite CTDs, in particular Systemic Lupus Erythematosus (SLE). The availability of new classification criteria for CTDs could be useful in identifying major CTDs from their onset and help in the differentiation from stable UCTDs, which will maintain their undifferentiated profile over time.Objectives:the aim of this study was to apply the new ACR/EULAR 2019 classification criteria of SLE2 to patients included, at the onset of UCTD, to evaluate how many patients could be re-classified as SLE.Methods:this is a retrospective observational study that enrolls patients who received the diagnosis of UCTD at the Rheumatology Unit of Pisa, according to the classification criteria proposed by Mosca et al1, and were then regularly followed in the same clinic (inception cohort). For each patient, demographics, comorbidities, treatment, clinical and serological data were collected, at baseline and during follow-up. The new ACR/EULAR 2019 classification criteria of SLE were applied to the inception cohort at the onset of the disease. The characteristics of patients re-classified as SLE according to the new classification criteria and those of patients who “remained” classified as UCTD were compared.Results:we enrolled 202 patients with a diagnosis of UCTD, mainly female (F:M 193:9) and of Caucasian ethnicity (97.5%), mean age at the diagnosis 38,5 ± 13,2 years and a median follow-up of 5 years (IQR 2-10).During the follow-up, 10 patients (4.9%) in our cohort developed clinical and serological characteristics that led to a diagnosis of a definite CTD, in particular: 7 SLE, 2 Mixed Connective Tissue Disease (MCTD) and 1 Sjogren’s syndrome.Applying the ACR/EULAR 2019 classification criteria of SLE to patients enrolled at the onset of the disease, 38/202 (18.8%) would have been classified as SLE (with a median score of 12 (IQR 11-15)) on the basis of the presenting clinical and serological manifestations. Interestingly, 6/7 patients who received a diagnosis of SLE during the follow-up were among these 38 patients.Among the items of the new classification criteria of SLE, the most frequently satisfied by patients “re-classified” were arthritis (63.2%), hematological (44.7%) and skin (23.7%) manifestations; as for the “immunological” items, hypocomplementemia (71%), SLE-specific (52.6%) and antiphospholipid (35.1%) autoantibodies.Comparing the subgroup of patients “re-classified” as SLE with that of patients who “remained” UCTD, we found that the first group presented more frequently hematological manifestations, hypocomplementemia and anti-dsDNA, anti-Sm, anti-RNP, anti-beta2GPI positivity at the onset of the disease (p<0.01). Moreover, during the follow-up, the subgroup of patients “re-classified” as SLE developed more frequently malar (p<0.05) and discoid rash and arthritis (p<0.01) compared to patients who “remained” UCTD.Conclusion:The development of increasingly sensible and specific classification criteria for definite CTDs may guide in the identification of stable UCTDs since their early stages and consequently in better definition of these conditions that can be considered as a distinct clinical entity.The early identification of stable UCTD is of great importance not only for clinical management (follow-up schedules as well as therapeutic protocols) but also for scientific implications.References:[1]Mosca M. et al., Clin Exp Rheumatol. Sep-Oct 1999;17(5):615-20; 2. Fanouriakis A. et al., Ann Rheum Dis. 2020 Jun;79(6):713-723.Disclosure of Interests:None declared

Clinical associations of the positive anti Ro52 without Ro60 autoantibodies: undifferentiated connective tissue diseases

2017 ◽  
Vol 71 (1) ◽  
pp. 12-19 ◽  
Author(s):  
Sai H K Murng ◽  
Moira Thomas
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Sjogren's Syndrome ◽  
Systemic Lupus ◽  
Autoimmune Conditions ◽  
Clinical Conditions ◽  
Undifferentiated Connective Tissue Diseases

AimsAutoantibodies targeting Ro52 and Ro60 antigens are historically reported as anti SSA/Ro. In general anti SSA/Ro results are either anti Ro52+Ro60+ or anti Ro52−Ro60+ antibodies. Anti Ro52 without anti Ro60 (Ro52+ Ro60−) antibodies are often not reported routinely. This study intends to review the potential significance of these autoantibodies in the management of connective tissue diseases.MethodA retrospective survey of Ro52+Ro60− was carried out as part of the service evaluation of extractable nuclear antigen antibodies (ENA) reporting from the immunology laboratory, the NHS Greater Glasgow and Clyde (GGC), UK. The clinical documents and laboratory results of 97 patients with Ro52+Ro60− and 100 patients with Ro52+Ro60+ were reviewed.ResultsSeventy-one patients (73%) with anti Ro52+Ro60− antibodies have been diagnosed with autoimmune conditions including undifferentiated connective tissue diseases (n=14, 14%), systemic lupus erythematosus (n=10, 10%), Sjögren’s syndrome (n=10, 10%) and rheumatoid arthritis (n=13, 13%). Twenty-three patients (24%) with anti Ro52+Ro60− antibodies have no autoimmune features but were found to have significant clinical conditions including malignancies. In contrast, 87 patients (87%) with anti Ro52+Ro60+ antibodies have autoimmune conditions including Sjögren’s syndrome (n=34, 34%), systemic lupus erythematosus (SLE; n=23, 23%), undifferentiated connective tissue diseases (n=12, 12%) and rheumatoid arthritis (n=6, 6%).ConclusionAnti Ro52 without anti Ro60 (Ro52+Ro60−) antibodies should be reported. In the majority of patients these autoantibodies were associated with various autoimmune diseases. Anti Ro52+Ro60− antibodies were also found in patients with significant clinical conditions including malignancies even though there was no suggestion of autoimmunity at the time of testing.

Application of SLICC classification criteria in undifferentiated connective tissue disease and evolution in systemic lupus erythematosus: analysis of a large monocentric cohort with a long-term follow-up

Lupus ◽  
2016 ◽  
Vol 26 (6) ◽  
pp. 616-622 ◽  
Author(s):  
A Bortoluzzi ◽  
F Furini ◽  
F Campanaro ◽  
M Govoni
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Classification Criteria ◽  
Undifferentiated Connective Tissue Disease ◽  
Systemic Lupus ◽  
Acr Criteria ◽  
Long Term Follow Up

Objectives The objectives of this study were to analyse the performance of the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria for systemic lupus erythematosus (SLE) in a large cohort of undifferentiated connective tissue disease (UCTD) population at onset of the disease and during a long-term follow-up of 15 years (1999–2013) and to evaluate the transition from UCTD to SLE, according to American College of Rheumatology (ACR) 1997 and SLICC 2012 classification criteria. Methods A cohort of patients who met the classification criteria proposed by Mosca et al. for UCTD, were analysed. The SLICC 2012 classification criteria for SLE were retrospectively applied to each patient at the time of the diagnosis (T0) and also periodically re-applied and compared to ACR 1997 criteria at three different time points in the follow-up. Results 329 patients were enrolled. According to inclusion criteria at T0 no patient met the SLE/ACR criteria, whilst, retrospectively applying the SLE/SLICC criteria, 44 patients already satisfied this set of criteria for SLE. During the follow-up 23 new patients reached the SLE/SLICC criteria and 14 patients met the ACR criteria with a stable rate of progression to SLE over time. Acute or subacute skin rash, antiphospholipid antibody (aPL) positivity and serositis were the variables correlated to the evolution to SLE. Conclusions In our UCTD population, the application of SLICC classification criteria for SLE at disease onset allowed identification of a proportion of otherwise missed SLE cases; during follow-up, and compared with ACR criteria, SLICC criteria expanded the number of patients classifiable as SLE otherwise classified as UCTD.

scholarly journals AB0815 FIRST DEGREE RELATIVES OF PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS - CLINICAL, SEROLOGICAL AND IMMUNOLOGICAL CORRELATIONS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1432.2-1432
Author(s):  
B. Penev ◽  
G. Vasilev ◽  
D. Kyurkchiev ◽  
S. Monov
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Current Knowledge ◽  
Statistical Significance ◽  
Clinical Signs ◽  
Connective Tissue Diseases ◽  
Pharmaceutical Products ◽  
Systemic Lupus

Background:Antinuclear antibodies (ANA) have been unequivocally recognized as essential for diagnosis and play both pathogenic and diagnostic roles in systemic lupus erythematosus (SLE). SLE and ANA have also been found to be more often among relatives of SLE patients. ANA and other immunological changes are known to appear prior to the clinical onset of the disease and thus can be used as predictors. Studies have reported that relatives of SLE patients who later transitioned to SLE displayed more lupus-associated autoantibody specificities and had early clinical signs. They also displayed elevated baseline plasma levels of inflammatory mediators, including B-lymphocyte stimulator (BLyS) and interferon-associated chemokines, with concurrent decreases in levels of regulatory mediators, e.g. tumor growth factor (TGF)-β. Commonly recognized risk factors for SLE are signs of past Epstein-Barr (EBV) infection, use of estrogen drugs and current smoking. It seems that ANA, immunologic changes and risk factors have not been investigated together in relatives of SLE patients.Objectives:The aim of the study was to determine the relative prevalence of clinical signs of SLE or connective tissue disease (CTD), smoking, use of estrogen drugs and levels of circulating ANA, BLyS, IFN-α, TGF-β, anti-EBV viral capsid antigen (VCA) IgM and IgG antibodies among sera of FDR, non-FDR healthy individuals and SLE patients.Methods:Forty three FDRs of SLE patients were studied along with 15 SLE patients and 15 clinically healthy subjects as control groups. The FDRs and the healthy answered a questionnaire about early clinical signs of CTD, smoking and estrogen use history. The questionnaire was developed based on the existing Screening Questionnaire for Connective Tissue Diseases and current knowledge of most early signs of CTD. Blood samples were obtained and tested for ANA, both by indirect immunofluorescence and immunoblot, anti-dsDNA by ELISA. ELISA was also performed to measure levels of BLys, IFN-α, TGF-β, anti-EBV IgM and IgG.Results:More than half of the FDRs displayed ANA in titer 1:160 or more, with predominately AC-4 type of fluorescence according to International Classification on ANA Patterns (ICAP) compared to only AC-1 and AC-0 among patients and controls respectively. A correlation between the ANA titer and the number of complaints was found. This was particularly valid or reported skin complaints and oral ulcers which appeared more frequently when ANA was 1:320 or above (p=0,018 and 0,038 respectively). Furthermore, oral ulcerations showed positive correlation with the presence of anti-Ro60. No associations were found in the healthy group between reported complaints and ANA titers. Smoking and estrogen use did not differ across the three groups. Patients showed significant differences in levels of BLys (p=0,027), TGF-β (p=0,019) and anti-EBV IgG (p=0.041) compared to both FDRs and controls. Without reaching statistical significance, levels of TGF-β tend to split the FDR group into “healthy-like” and “SLE-like”.Conclusion:Our results show that FDR ANA levels are between those of SLE patients and healthy subject groups. This is consistent with previous studies. The data also suggest that ANA positivity correlates with reported complaints, some of which could be interpreted as very early clinical signs of SLE. Of note, anti-Ro60 is known to be among the earliest ANA that appear in “future” SLE patients and in this study they are related to oral complaints that could be caused by early sicca phenomena. Immunologically, our data support previous findings [1] that the FDRs are a heterogenic group with different “lupus-developing” potential.References:[1]Munroe МE. et al, Soluble Mediators and Clinical Features Discern Risk of Transitioning to Classified Disease in Relatives of Systemic Lupus Erythematosus Patients, Arthritis Rheumatol. 2017 March; 69(3): 630–642.Disclosure of Interests:Bogdan Penev: None declared, Georgi Vasilev: None declared, Dobroslav Kyurkchiev: None declared, Simeon Monov Speakers bureau: I have been paid for giving lectures on statistical data on efficacy of many pharmaceutical products on various companies

Mixed Connective Tissue Disease- Physician’s Dilemma: A case report

2021 ◽  
Vol 11 (Number 1) ◽  
pp. 60-65
Author(s):  
Abu Saleh Shimon ◽  
Mahjuba Umme Salam ◽  
Monharul Islam Bhuiyan ◽  
Mashuq Ahmad Jumma ◽  
Imran Hussain ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
High Titer ◽  
Connective Tissue Diseases ◽  
Systemic Lupus ◽  
Serological Tests ◽  
Normotensive Woman ◽  
New Onset

Mixed connective tissue disease is an entity of autoimmune disease with overlapping features of systemic lupus erythematosus, scleroderma, rheumatoid arthritis, dermatomyositis and with positive anti-U1 RNP antibody. We report here a 52 year old non-diabetic, normotensive woman presenting with new onset dysphagia for two months with variable features of multiple types of connective tissue diseases for two years. Clinical features and type specific serological tests for different connective tissue diseases showed puzzling results. However, finally a high titer of anti-U1RNP antibody led to the diagnosis of mixed connective tissue disease.

Lupus: the new epidemic

Lupus ◽  
2019 ◽  
Vol 28 (9) ◽  
pp. 1031-1050 ◽  
Author(s):  
M F Ugarte-Gil ◽  
L A González ◽  
G S Alarcón
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antinuclear Antibodies ◽  
Secular Trends ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
New Classification ◽  
Uniform Definition ◽  
Definition Of ◽  
Incomplete Lupus

Is systemic lupus erythematosus (SLE) is occurring more frequently now than in decades past? Despite improvements in the identification of patients with SLE, the development of new classification criteria, and the recognition of several biomarkers used alone or in combination, the diagnosis of SLE is still a challenge for clinicians, in particular early in the course of the disease, which makes the recognition of secular trends difficult to ascertain. Lacking a uniform definition of preclinical lupus or incomplete lupus, it is difficult to predict accurately which patients would go on to develop SLE. We will briefly review the classification criteria, early or preclinical SLE, the epidemiology of SLE, antinuclear antibodies-negative SLE, and biomarkers of the disease.

scholarly journals 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus

2019 ◽  
Vol 78 (9) ◽  
pp. 1151-1159 ◽  
Author(s):  
Martin Aringer ◽  
Karen Costenbader ◽  
David Daikh ◽  
Ralph Brinks ◽  
Marta Mosca ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Validation Cohort ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
New Classification ◽  
Entry Criterion ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
European League

ObjectiveTo develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR).MethodsThis international initiative had four phases. (1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort and a patient survey. (2) Criteria reduction by Delphi and nominal group technique exercises. (3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. (4) Refinement of weights and threshold scores in a new derivation cohort of 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 subjects.ResultsThe 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunological (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria.ConclusionThese new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered and weighted criteria reflect current thinking about SLE and provide an improved foundation for SLE research.

Plasma Platelet-derived Microparticles in Patients with Connective Tissue Diseases

2011 ◽  
Vol 38 (4) ◽  
pp. 680-684 ◽  
Author(s):  
CHINAMI OYABU ◽  
AKIO MORINOBU ◽  
DAISUKE SUGIYAMA ◽  
JUN SAEGUSA ◽  
SHINO TANAKA ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Regression Analysis ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Clinical Characteristics ◽  
Linear Regression Analysis ◽  
Connective Tissue Diseases ◽  
Multiple Linear Regression Analysis ◽  
Systemic Lupus

Objective.To clarify the role of platelet-derived microparticles (PDMP), which are small vesicles with thrombotic and immunological properties, in systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis/polymyositis (PM/DM), and mixed connective tissue disease (MCTD).Methods.Plasma levels of PDMP were measured by ELISA, and compared among patients with one of the 4 diseases. Association of PDMP levels with clinical characteristics and medication of the patients was also examined.Results.PDMP levels were higher in patients with MCTD and SSc than in controls. Multiple linear regression analysis revealed that patients with Raynaud’s phenomenon (RP) showed higher PDMP levels than those without. PDMP levels in individual patients did not fluctuate significantly over several months.Conclusion.PDMP level is associated with MCTD, SSc, and RP, and could be a novel marker for RP.

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