scholarly journals Pictorial review: Thoracic involvement in connective tissue diseases: radiological patterns and follow-up

2015 ◽  
Vol 98 (1) ◽  
pp. 3
Author(s):  
G. Serra ◽  
A. L. Brun ◽  
P. Ialongo ◽  
M. L. Chabi ◽  
Philippe A. Grenier
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Pictorial Review ◽  
Radiological Patterns

FRI0099 Pulmonary fibrosis and connective tissue diseases: Follow-up of lung involvement in rheumatoid arthritis and systemic sclerosis

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 343.1-343
Author(s):  
A. Soriano ◽  
D. Margiotta ◽  
B. Marigliano ◽  
P. Alemanno ◽  
M. Vadacca ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Connective Tissue Diseases ◽  
Lung Involvement

scholarly journals Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Nicola Ciancio ◽  
Mauro Pavone ◽  
Sebastiano Emanuele Torrisi ◽  
Ada Vancheri ◽  
Domenico Sambataro ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Connective Tissue Diseases ◽  
Function Tests

scholarly journals O34 Predicting autoimmune connective tissue diseases: three year follow up of an at-risk cohort identifies late progression and predicts need for therapy

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Sabih Ul-Hassan ◽  
Katherine Dutton ◽  
Zoe Wigston ◽  
Ade Alase ◽  
Yuzaiful Md Yusof ◽  
...  
Keyword(s):  
At Risk ◽  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Clinical Feature ◽  
Clinical Criteria ◽  
Score Analysis ◽  
Clinically Significant ◽  
Future Work ◽  
Significant Disease

Abstract Background Autoimmune connective tissue diseases (AI-CTDs: SLE, pSS, IIM) are preceded by asymptomatic ANA positivity. We previously recruited a cohort of new ANA-positive referrals without AI-CTD. 17% met criteria by AI-CTD at 12 months, and this was predicted an IFN Score. This study includes 3 year follow up of that cohort with more detailed analysis of the non-progressor group and predictors. Methods Patients were recruited if they had: (i) ANA; (ii) did not meet criteria for AI-CTD; (iii) symptoms less than 12 months. Diagnostic criteria for AI-CTDs and therapies were assessed at baseline then 12-monthly for 3 years. We categorised progression as:1. Absolute-non-progressors (no clinical criteria at all time points: 0-36 months), 2. Undifferentiated-CTD ( > =1 clinical criterion at baseline and/or at follow up but not meeting criteria), 3. Year-1-progressor (meeting criteria for AI-CTD within 12 months), 4. Late-progressor (meeting criteria for AI-CTD later than 12 months). A 2-score analysis of IFN Status was performed as previously described (Yusof, ARD 2018). Results 3-year follow up was available in 146/150. Proportions in the above categories were: Absolute-non-progressors: 33/146 (23%); Undifferentiated-CTD: 86/146 (59%); Year-1-progressors: 21/146 (14%); Late-progressors: 5/146 (3%). No patient progressed or required immunosuppression after 2 years. 6/86 patients with Undifferentiated-CTD received an immunosuppressant. The present work therefore defines a larger group of 32/146 (22%) with clinically significant disease including 21 Year-1-progressors, 5 late-progressors, and 6 undifferentiated-CTD who needed an immunosuppressant. Clinical features had limited utility in predicting these outcomes. Of 31 patients with no clinical criteria at baseline, 1 progressed to meet criteria within 1 year, 2 progressed at 1-2 years, 3 were prescribed hydroxychloroquine and 1 was prescribed an immunosuppressant. The 108 patients with at least 1 criterion at baseline had the highest risk: 20 progressed to meet criteria within 1 year, 2 progressed at 1-2 years, the others all had U-CTD. 35 were prescribed hydroxychloroquine and 13 were prescribed an immunosuppressant. There was also no association between ENA, C3 or C4 and clinical outcome. The association between Interferon Score B and progression was stronger when comparing Year 1 progressors with absolute non-progressors (p = 0.007). Late progression was not predicted by baseline IFN Scores. However, within U-CTD, patients who required an immunosuppressant had higher expression of IFN Score A (p = 0.011) and IFN Score B (p < 0.001) than those who did not. Conclusion Among ANA-positive referrals, no clinical feature or routine laboratory test could rule out development of clinically significant disease, which included AI-CTD or undifferentiated CTD needing therapy. However, IFN Scores had a unique value in predicting these outcomes. At-risk individuals who ultimately developed clinically significant disease are therefore immunologically but not clinically distinctive. Future work will incorporate biomarkers into clinically applicable risk models to allow earlier exclusion of AI-CTD or trials of preventative treatment. Disclosures S. Ul-Hassan None. K. Dutton None. Z. Wigston None. A. Alase None. M. Md Yusof None. E.M. Vital None.

scholarly journals Efficiency in follow-up immunology testing for patients with connective tissue diseases and vasculitis

2011 ◽  
Vol 11 (6) ◽  
pp. 632-633
Author(s):  
Charlotte A Sharp ◽  
Ian N Bruce
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Diseases

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

FRI0510 Long-Term Post Partum Follow-Up of Undifferentiated Connective Tissue Diseases Newly Diagnosed during Pregnancy

2016 ◽  
Vol 75 (Suppl 2) ◽  
pp. 623.1-623
Author(s):  
V. Ramoni ◽  
M. Romano ◽  
C. Rocchetti ◽  
B. Vitolo ◽  
F. Beneventi ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Post Partum ◽  
Connective Tissue Diseases ◽  
Newly Diagnosed ◽  
Undifferentiated Connective Tissue Diseases

scholarly journals Patients with ANCA-Associated Glomerulonephritis and Connective Tissue Diseases: A Comparative Study from the Maine-Anjou AAV Registry

2019 ◽  
Vol 8 (8) ◽  
pp. 1218
Author(s):  
Fanny Guibert ◽  
Anne-Sophie Garnier ◽  
Samuel Wacrenier ◽  
Giorgina Piccoli ◽  
Assia Djema ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Thrombotic Event ◽  
Connective Tissue Diseases ◽  
Case Series ◽  
Rare Condition ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Control Group ◽  
Renal Relapse ◽  
Patients Experience

Background and objectives: The overlap between antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis (ANCA-GN) and connective tissue diseases (CTD) has been reported mainly as case series in the literature. Frequency of this association, as well as presentation and outcomes are unknown. Materials and Methods: Patients from the Maine-Anjou ANCA-associated vasculitides (AAV) registry with ANCA-GN diagnosed between 01/01/2000 and 01/01/2018, ANCA positivity, and at least six months of follow-up, were included. Results: 106 out of 142 patients fulfilled the inclusion criteria and were analyzed. CTD was present at ANCA-GN diagnosis in 16 (15.1%) patients. The most common CTD were rheumatoid arthritis, Sjogren syndrome and systemic sclerosis. Compared to the control group, females were more represented in the CTD group (75%, p = 0.001). Renal presentation was comparable between groups, including the pathological analysis of renal biopsies. Patients of CTD group presented a higher rate of non-renal relapse (25% versus 7.7%, p = 0.037), and experienced more frequently a venous thrombotic event (31.2% versus 10%, p = 0.021). No difference between groups was observed according to major outcomes. Conclusion: Association between CTD and ANCA-GN is not a rare condition and predominantly affects females. While AAV presentation is not significantly different, CTD patients experience more frequently non-renal relapse and venous thrombotic events.

SAT0435 Undiagnosed Connective Tissue Diseases in Pulmonary Arterial Hypertension Patients: Baseline and Follow-Up Results from a PAH Referral Centre

2015 ◽  
Vol 74 (Suppl 2) ◽  
pp. 817.2-817
Author(s):  
V. Codullo ◽  
L. Cavagna ◽  
S. Ghio ◽  
C.A. Scirè ◽  
E. Guzzafame ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Diseases ◽  
Referral Centre ◽  
Pulmonary Arterial

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

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