Welcome to the 2021 LUPUS CORA Meeting Program Scheduling
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CORA Topics || ASL23 POLYMYOSITIS AND DERMATOMYOSITIS, CORA Topics || ASL26 PULMONARY ARTERIAL HYPERTENSION & SYSTEMIC SCLEROSIS, No Topic Needed
- Gabriella Szucs (Hungary)
Introduction by Chairperson
- Gabriella Szucs (Hungary)
Can we always distinguish PAH (group I) from group 3 PH in SSc patients? YES
- Elisabetta Zanatta (Italy)
Can we always distinguish PAH (group I) from group 3 PH in SSc patients? NO
- Cosimo Bruni (Italy)
Live rebuttal
- Elisabetta Zanatta (Italy)
Live rebuttal
- Cosimo Bruni (Italy)
Panel discussion - Live
FREQUENCY OF DISEASE PROGRESSION AFTER IMMUNOSUPPRESSIVE WITHDRAWAL IN A RETROSPECTIVE COHORT OF SYSTEMIC SCLEROSIS PATENTS
- Francesco Benvenuti (Italy)
Abstract
Background and Aims
Systemic sclerosis (SSc) is an autoimmune disease mainly affecting skin, lung, and joints. Immunosuppressants (IS) may halt disease progression. No data are available concerning the risk of disease progression after IS withdrawal.
Methods
Among the 650 SSc patients enrolled in Padova cohort (2001-2020), 24 withdrew IS due to disease stabilization (according to expert clinician) or intolerance/poor adherence. 58% patients have diffused (dcSSc) and 42% limited SSc. We recorded IS therapies and categorized disease progression as cutaneous (mRSS increase of >=5 points or >=25%), respiratory (decline in FVC>=10%, DLCO>=15%, or extension of radiographic ILD), articular (DAS28 increase >=0.6), or renal (scleroderma renal crisis). We analyzed FVC, DLCO, mRSS and radiographic ILD extent (>= or < 20%) at IS withdrawal, at disease progression, and last follow-up.
Results
The median duration of IS therapy was 42.3 (SD±31) months. After IS withdrawal ten patients (42%) experienced disease progression: 4 respiratory, 2 cutaneous, 3 articular and 1 renal. The average survival time without progression was 5 years. The risk for disease progression for any causes tends to be greater in patients with dcSSc (3.388 CI0.972-11.81, p=0.0554) and lower in patients who withdrew IS for disease stabilization (0.18 CI 0.04-1.2, p=0.0543). No associations were observed between the risk of progression and disease or IS duration, mRSS, and ILD extent.
Conclusions
The IS withdrawal leads to a huge risk of disease progression which tends to be higher in dcSSc patients and lower in those who withdrew IS for disease stabilization (according to expert clinician).
Live Q&A
EPIDEMIOLOGY OF IDIOPATHIC INFLAMMATORY MYOPATHIES IN AFRICA: A CONTEMPORARY SYSTEMATIC REVIEW
- Mickael Essouma (Cameroon)
Abstract
Background and Aims
The epidemiology of idiopathic inflammatory myopathies (IIMs) has been extensively studied out of Africa, but remains unclear in Africa. This is the first systematic review extensively addressing the epidemiology of IIMs in Africa.
Methods
We searched MEDLINE, EMBASE and African Journals Online for relevant studies published through December 30, 2020 . Data were combined via narrative synthesis. The review protocol was registered with PROSPERO, CRD42020186781.
Results
Of 9918 records, 39 were included, reporting 683 cases (71.7% adults). The incidence of dermatomyositis (DM) was estimated at ~7.5/1,000,000 person years and 1.2/1,000,000 person years, and that of polymyositis (PM) was estimated at 8.8/1,000,000 person years. Prevalence rates of IIMs and PM subtype were estimated at 11.49/100,000 and 11/100,000 (95% confidence interval: 0-32), respectively. Mean age at diagnosis ranged from 7.9 to 57.2 years, and the female proportion from 50% to 100%. Main subtypes of IIMs in adults were DM (21%-93%) and PM (12%-79%), whereas DM (5.8%-9%) was the commonest juvenile IIM. Skeletal muscle involvement (56%-100%) was the main disease feature, and oesophagus the most commonly affected internal organ (6%-65.2%). Anti-Jo1/histidyl tRNA synthetase (7%-100%) and anti-Mi2 (17%-45%) antibodies were the commonest myositis specific antibodies. Early mortality was high (7.8%-45%); main death causes being infections, cancers and damage accrual in respiratory and cardiovascular domains.
Conclusions
The epidemiology of IIMs in Africa seems similar to that from other continents, except for a potential much younger age at onset of adult IIMs in Africa. Further high-quality studies are needed.
Live Q&A
DIAGNOSTIC PERFORMANCE OF THE ACR/EULAR 2013 CLASSIFICATION CRITERIA FOR SYSTEMIC SCLEROSIS IN A ROUTINE CARE SETTING
- María Teresa Schiaffino (Spain)
Abstract
Background and Aims
An ACR/EULAR task force released new criteria in 2013 to classify patients with systemic sclerosis (SSc). This study evaluates the diagnostic performance of these criteria in a multidisciplinary care setting.
Methods
Patients with an active follow-up in a Systemic Autoimmune Diseases Unit with clinical diagnosis of SSc (SSc-cases) were matched by age and gender with consecutive patients referred to a capillaroscopy clinic (controls). The classification criteria were tested on discrimination and diagnostic accuracy between both groups of patients. Receiver operating characteristic (ROC) curve and the area under the curve (AUC) was calculated for the global score to define the best cut off to classify the SSc patients.
Results
130 patients with SSc and 130 matched-controls were included in this analysis, 90% women, with a mean age of 61.5. Main diagnosis for the control group were primary Raynaud´s phenomenon (34.6%), undifferentiated connective tissue disease (13.1%), and mixed connective tissue disease (9.2%). The 92% and 8% of patients in the SSc-cases and control groups met the 2013 ACR/ EULAR SSc classification criteria respectively. Sensitivity and specificity of the criteria were 81.5% and 93.7%, respectively. The best cut offs for the score were 8 and 9, and the AUC (95%CI) was 0.962 (0.939-0.985). The individual items with a better discriminatory capacity were abnormal capillaroscopy, telangiectasia and anticentromere antibody positivity.
Conclusions
The ACR/EULAR 2013 criteria showed good diagnostic properties in this cohort reflecting daily practice. Individual items showing the highest discriminatory capacity were abnormal capillaroscopy, telangiectasia and anticentromere antibody positivity.
Live Q&A
Live Session summary by Chairperson
- Gabriella Szucs (Hungary)