SAT0476 Incidence, prevalence, mortality and performance of the acr/eular 2013 classification criteria in physician diagnosed systemic sclerosis: results from a population based us cohort (1980–2016)

Background:Systemic sclerosis (SSc) is a complex immune-mediated disease with heterogeneous manifestations, which is characterized by vasculopathy and fibrosis of the skin and visceral organs. Mortality associated with SSc exceeds that of other rheumatic diseases, though population-based studies assessing recent trends in survival are lacking.Objectives:We aimed to determine the incidence and prevalence of physician-diagnosed SSc in a population-based cohort over a 39-year time period, and assess for trends in survival over time.Methods:Medical records of patients with a diagnosis or suspicion of SSc in a geographically well-defined area from Jan 1, 1980 to Dec 31, 2018 were reviewed to identify incident cases of SSc. Cases were defined by physician diagnosis of SSc, and fulfillment of the 2013 ACR/EULAR classification criteria was ascertained. Prevalent cases of SSc on Jan 1, 2015 were also identified. Incidence and prevalence rates were age- and sex-adjusted to the 2010 U.S. white population. Survival rates were compared with expected rates in the general population.Results:85 incident cases of SSc (91% female, mean age 55.4 ± 16 y) and 49 prevalent cases on Jan 1, 2015 were identified. Patients had a mean 11.7 (SD 9.4) years of follow-up available. The overall age and sex adjusted annual incidence for 1980-2018 was 2.5 (95% CI: 2.0-3.1) per 100,000 population, with no change in incidence over time (p=0.32). The age-adjusted incidence was 4.4 (95% CI: 3.4-5.4) for females, and 0.56 (95% CI: 0.16-0.96) for males per 100,000 population. The age- and sex-adjusted prevalence on Jan 1, 2015 was 43.6 (95% CI: 31.3-55.8) per 100,000 population.77 (91%) patients fulfilled the 2013 classification criteria; 38 (45%) fulfilled 1980 criteria. 70 (82%) had limited cutaneous involvement, 12 (14%) had diffuse cutaneous involvement, and 3 (4%) had sine scleroderma. At SSc diagnosis, 80 (94%) patients had Raynaud’s, 43 (51%) had sclerodactyly, 39 (46%) had telangiectasias, 14/48 (29%) had abnormal nailfold capillaries, 16/35 (46%) had digital ulcers or fingertip scarring, 8 (9%) had interstitial lung disease (ILD), and 7 (8%) had pulmonary arterial hypertension (PAH). 77/82 patients (91%) had a positive antinuclear antibody. 44 (52%) had a known SSc-related autoantibody: 32 (73%) with anti-centromere, 9 (20%) with anti-Scl-70, and 4 (9%) with anti-RNA-polymerase III.Survival was 77% (95% CI: 69-87) at 5 years, 66% (95% CI: 56-78) at 10 years, and 42% (95% CI: 30-57) at 20 years, with no evidence of improved survival over time (p=0.46). Age (Hazard ratio [HR]: 1.49 per 10 year increase; 95% CI 1.19-1.88), smoking at time of diagnosis (HR: 2.37; 95% CI: 1.05-5.34), digital ischemia (HR: 2.54; 95% CI: 1.33-4.87), ILD (HR: 4.00; 95% CI: 2.11-7.59), and PAH (HR: 4.30; 95% CI: 2.24-8.25) had significant associations with mortality. Survival of patients with SSc was poorer than the general population (standardized mortality ratio: 2.48; 95% CI: 1.76-3.39).Conclusion:The average incidence of SSc in this population-based cohort spanning 39 years was 2.5 per 100,000 population, with no change in incidence over time. Age, smoking, digital ischemia, ILD and PAH were risk factors for poorer survival. Overall survival for patients with SSc is worse than that of the general population and shows no improvement over time, suggesting continued need for improved diagnostic and treatment measures.Figure 1.Survival of 85 Olmsted County residents with SSc compared with expected survival rates from Minnesota lifetables (observed: solid line, expected: dashed line).Disclosure of Interests:None declared

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Epidemiology and trends in survival of systemic sclerosis in Olmsted County (1980–2018): A population-based study

Journal of Scleroderma and Related Disorders ◽

10.1177/23971983211026853 ◽

2021 ◽

pp. 239719832110268

Author(s):

Caitrin M Coffey ◽

Yasser A Radwan ◽

Avneek S Sandhu ◽

Cynthia S Crowson ◽

Philippe R Bauer ◽

...

Keyword(s):

Systemic Sclerosis ◽

General Population ◽

Confidence Interval ◽

Population Based ◽

Classification Criteria ◽

Standardized Mortality Ratio ◽

Olmsted County ◽

Incident Cases ◽

Over Time ◽

Age And Sex

Background/Purpose: To update the epidemiology of systemic sclerosis and evaluate the performance of the American College of Rheumatology/European League Against Rheumatism 2013 versus 1980 American Rheumatism Association classification criteria in a US population-based cohort. Methods: An inception cohort of patients with incident systemic sclerosis from 1 January 1980 through 31 December 2018, in Olmsted County, Minnesota, was identified based on comprehensive individual medical record review. Incidence and prevalence rates were age- and sex-adjusted to the 2010 US white population. Survival rates were compared with expected rates in the general population. Fulfillment of 1980 and 2013 classification criteria was ascertained. Results: A total of 85 incident cases of systemic sclerosis (91% female, mean age = 55.4 ± 16 years) and 49 prevalent cases on 1 January 2015 were identified. The overall age- and sex-adjusted annual incidence was 25 (95% confidence interval = 20–31) per million population, with no change in incidence over time. The age- and sex-adjusted prevalence was 436 (95% confidence interval = 313–558) per 1,000,000 population. 77 (91%) patients fulfilled the 2013 classification criteria, and 38 (45%) fulfilled the 1980 criteria. Mortality among patients with systemic sclerosis was significantly higher in comparison to the general population, with a standardized mortality ratio of 2.48 (95% confidence interval = 1.76–3.39) and no evidence of improvement over time. Conclusion: Systemic sclerosis developed in 25 persons/million/year with no change over the 39-year study period. The 2013 classification criteria perform significantly better than the 1980 criteria but failed to classify 9% of patients. Systemic sclerosis portends a 2.5-fold higher risk of mortality than the general population, with no evidence of improved survival over time.

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Faculty Opinions recommendation of Changes in the incidence, prevalence and mortality of bronchiectasis in the UK from 2004 to 2013: a population-based cohort study.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.725917838.793530591 ◽

2017 ◽

Author(s):

Adam Hill

Keyword(s):

Cohort Study ◽

Population Based ◽

The Uk ◽

Incidence Prevalence

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FRI0526 THE INCIDENCE, PREVALENCE AND MEDICATION USE OF RHEUMATOID ARTHRITIS AMONG KOREAN WOMEN IN CHILDBEARING YEARS: A NATIONWIDE POPULATION-BASED STUDY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4249 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 862.2-863

Author(s):

M. K. Chung ◽

J. S. Park ◽

H. S. Lim ◽

C. H. Lee ◽

J. Lee

Keyword(s):

Rheumatoid Arthritis ◽

Lupus Erythematosus ◽

Medication Use ◽

Population Based ◽

Korean Women ◽

Childbearing Age ◽

Disease Modifying Antirheumatic Drugs ◽

Antirheumatic Drugs ◽

Disease Modifying ◽

Incidence Prevalence

Background:Rheumatoid arthritis (RA) predominantly affects women and has a significant impact on childbearing. Several population-based studies identifying incidence, prevalence, and medication use of RA have been reported, yet epidemiological studies focusing on women with RA in childbearing years are missing.Objectives:We aimed to identify the incidence, prevalence and medication use of RA among Korean women in childbearing years.Methods:From National Health Insurance Service (NHIS) data (2009-2016), containing inpatient and outpatient claim information for approximately 97% of the Korean population, we identified 9,217,139 women aged between 20-44 years. Incidence and prevalence of RA in the specific sociodemographic group of women in childbearing age were analyzed, and the prevalence of medication prescription were compared between women with RA and controls without rheumatic diseases such as RA, systemic lupus erythematosus, and ankylosing spondylitis. Individuals with RA were defined by the presence of International Classification of Disease, 10th revision code, M05. The medication use was defined as receiving > 90days prescriptions of NSAIDs, corticosteroids (CSs), and conventional synthetic (cs) disease modifying antirheumatic drugs (DMARDs) or > 1day prescription of biologic (b) DMARDs.Results:Total 24,590 women with RA were identified. The average incidence of RA during 2011-2016 among women in childbearing years was 24.1/100,000 person-years (PYs) (95% CI 20.91-27.31) with a yearly increase from 20.99/100,000 PYs in 2011 to 28.38/100,000 PYs in 2016. The average prevalence of RA during 2009-2016 among women in childbearing years was 105.2/100,000 PYs (95% CI 99.0-111.5) with a minimum of 95.7/100,000 PYs in 2009 and a maximum of 110.5/100,000 PYs in 2016. There were increasing trends in both incidence and prevalence of RA according to age among women in childbearing years peaking in the age group of 40-44 years. The prescriptions of NSAIDs, CSs, csDMARDs and bDMARDs were more frequent in women with RA than controls (NSAIDs; 94.21% vs 21.79%, CSs; 83.65% vs 4.28%, csDMARDs; 91.23% vs 0.41%, bDMARDs; 0.11% vs 0%, p<0.001).Conclusion:The incidence and prevalence of RA are high among Korean women in childbearing years, and medication use was significantly more frequent in this specific population than controls. High disease burden is imposed upon women in childbearing years.References:[1] Won S, Cho SK, Kim D, Han M, Lee J, Jang EJ, Sung YK, Bae SC: Update on the prevalence and incidence of rheumatoid arthritis in Korea and an analysis of medical care and drug utilization. Rheumatol Int 2018, 38(4):649-656.[2] Smeele HTW, Dolhain R: Current perspectives on fertility, pregnancy and childbirth in patients with Rheumatoid Arthritis. Seminars in arthritis and rheumatism 2019, 49(3s):S32-s35.Table 1.Medication use among women with RA and controls in childbearing age between 20-44 years during 2009-2016Control(n=155,486)RA(n=23,756)n(%)n(%)PNSAIDs33,887(21.79)22,380(94.21)<.0001Steroids6,653(4.28)19,871(83.65)<.0001csDMARDs634(0.41)21,673(91.23)<.0001bDMARDs0(0.00)27(0.11)<.0001RA, rheumatoid arthritis; NSAID, non-steroidal anti-inflammatory drug; cs, conventional synthetic; b, biologic; DMARDs, disease modifying antirheumatic drugsDisclosure of Interests:None declared

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AB0597 PULMONARY FUNCTION IN PATIENTS DIAGNOSED OF EARLY SYSTEMIC SCLEROSIS: A NEW TOOL FOR SYSTEMIC SCLEROSIS CLASIFFICATION?

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4423 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1595.1-1595

Author(s):

F. M. Ortiz Sanjuan ◽

C. Pávez Perales ◽

E. Vicens Bernabeu ◽

C. Alcañiz Escandell ◽

I. Cánovas Olmos ◽

...

Keyword(s):

Systemic Sclerosis ◽

Pulmonary Function ◽

Airway Obstruction ◽

Pulmonary Involvement ◽

Classification Criteria ◽

Small Airway ◽

University Hospital ◽

Ventilatory Pattern ◽

Small Airway Obstruction

Background:Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and is often progressive and has a poor prognosis. A restrictive ventilatory defect could suggest ILD either alone or in combination with pulmonary arterial hypertension.Nowadays, Early-SSc is well defined as preliminary stage of SSc. Patients who meet criteria for Early-SSc could benefit from an early diagnosis of pulmonary involvement.Objectives:Our aim was to assess the pulmonary function in patients diagnosed of Early SSc.Methods:Retrospective observational study of a wide and unselected series of patients diagnosed as Early-SSc from a single university hospital from 2012 to 2019. Patients were classified as Early-SSc following Le Roy criteria. Despite this, patients already did not meet 2013 ACR/EULAR classification criteria for SSc. We reviewed pulmonary function through conventional spirometry and diffusing capacity of lung for carbon monoxide (DLCO).Results:We included 56 patients with a mean age of 52.3±12.1 years (96.4% women; 3.6% men).At the diagnosis of Early-SSc, no one of our patients evidenced a restrictive ventilatory pattern. DLCO was below normal limits in 18 patients (32.1%). Small airway obstruction expressed according decreased maximal (mid-) expiratory flow (MMEF) 25-75 was present in 24 patients (42.8%).After a mean follow-up period of 38.3±2.4 months, 29 (51.8%) patients fulfilled 2013 ACR/EULAR criteria. The average time between diagnosis of Early-SSc and achieve SSc classification was 24.4±1.8 months. The remaining 27 patients continued classified as Early-SSc.An analysis of the subgroup of patients which progressed to SSc showed that DLCO was decreased in 15 of those 29 patients (51.7%) and 18 of 29 patients (62.1%) presented decreased MMEF 25-75. Comparing with the subgroup of patients which not progressed to SSc were significant differences (Decreased DLCO: 51.7% vs 11.1%; p=0.02 and decreased MMEF 25-75: 42.8% vs 22.2%; p=0.05).The analysis of pulmonary function of the subgroup of patients continued classified as Early-SSc after follow-up period did not show significative changes after follow-up.Conclusion:In our study, a third of the patients classified as Early-SSc presented at diagnosis abnormal values of DLCO and/or signs of small airway obstruction without the presence of a restrictive ventilatory pattern. Moreover, this pulmonary disfunction was significantly more frequent in patients who progressed to definitive SSc. Patients which remains classified as Early-SSc did not experience significative changes.Our results support the concept that pulmonary function was impaired in Early-SSc and that I should probably be considered for future Early-SSc classification criteria.Disclosure of Interests:None declared

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FRI0226 OPTICAL COHERENCE TOMOGRAPHY OF THE SKIN DETECTS SCLERODERMA CHANGES IN CLINICALLY UNAFFECTED SKIN: AN OPPORTUNITY FOR EARLY DETECTION OF SYSTEMIC SCLEROSIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.6589 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 696.2-696

Author(s):

G. Abignano ◽

D. Temiz Karadağ ◽

O. Gundogdu ◽

G. Lettieri ◽

M. C. Padula ◽

...

Keyword(s):

At Risk ◽

Clinical Trials ◽

Optical Coherence Tomography ◽

Systemic Sclerosis ◽

Early Detection ◽

Classification Criteria ◽

Healthy Controls ◽

Optical Coherence ◽

Risk Patients ◽

Unaffected Skin

Background:The Very Early Diagnosis Of Systemic Sclerosis (VEDOSS) study has shown that 82% of patients with Raynaud’s Phenomenon, specific ANA positivity and scleroderma pattern at nail fold videocapillaroscopy will fulfil classification criteria within 5 years. This is suggesting that there is a subclinical window of opportunity to diagnose systemic sclerosis (SSc) before clinical manifestations occur. In this scenario, a non-invasive tool to diagnose SSc in clinically unaffected skin might improve the early detection of disease in at risk-patients. Optical coherence tomography (OCT) of the skin has been shown to be a sensitive and accurate biomarker of skin fibrosis in SSc.Objectives:Here we aimed to assess the ability of skin OCT to “detect” SSc in clinically unaffected skin from a multicentre cohort.Methods:Dorsal forearm skin of SSc patients and matched-healthy controls (HC) was evaluated using VivoSight scanner (Michelson Diagnostics). Mean A-scans (mean OCT signal plotted against depth-in-tissue) were derived as previously described. Minimum Optical Density (MinOD), Maximum OD (MaxOD) and OD at 300 micron-depth (OD300) were calculated. Clinical involvement was assessed by an operator blinded to OCT findings using the mRSS. Receiver-operating characteristic (ROC) curve analysis was carried out for MinOD, MaxOD, and OD300 to evaluate their ability to discriminate between SSc and HC. Statistical analysis was performed using GraphPad Prism software V.7.0.Results:One hundred seventy four OCT images were collected from 87 subjects [43 SSc (39 Female, mean age 49.7±9.1 years) and 44 gender/age-matched healthy controls (HC) (36 Female, mean age 50.2±8.3 years)] in two different SSc centres. All patients fulfilled classification criteria for SSc. OCT measures demonstrated discriminative ability in SSc skin detection with any clinical skin involvement (0-3 at site of analysis) with an AUC of 0.73 (MinOD, 95%CI 0.64-0.81), 0.77 (MaxOD, 95%CI 0.7-0.85) and 0.82 (OD300, 95%CI 0.76-0.89); p<0.0001 for all as previously indicated. Most importantly, all three measures showed comparable performance in detecting scleroderma also in clinically unaffected skin (mRss=0 at site of analysis), with an AUC of 0.7 (95%CI 0.6-0.81, p=0.001), 0.72 (95%CI 0.61-0.83, p=0.0003) and 0.72 (95%CI 0.61-0.83, p=0.0003) for MinOD, MaxOD and OD300 respectively.Conclusion:Virtual biopsy by OCT recognises clinically unaffected skin of SSc patients from the HC skin. This is consistent with gene array data showing that scleroderma specific signatures are consistent in affected and clinically unaffected skin. These results inform future studies on at risk patients with clinically unaffected skin which may define a role for OCT in detecting subclinical SSc.Disclosure of Interests:Giuseppina Abignano: None declared, Duygu Temiz Karadağ: None declared, Ozcan Gundogdu: None declared, Giovanni Lettieri: None declared, Maria Carmela Padula: None declared, Angela Padula: None declared, Paul Emery Grant/research support from: AbbVie, Bristol-Myers Squibb, Merck Sharp & Dohme, Pfizer, Roche (all paid to employer), Consultant of: AbbVie (consultant, clinical trials, advisor), Bristol-Myers Squibb (consultant, clinical trials, advisor), Lilly (clinical trials, advisor), Merck Sharp & Dohme (consultant, clinical trials, advisor), Novartis (consultant, clinical trials, advisor), Pfizer (consultant, clinical trials, advisor), Roche (consultant, clinical trials, advisor), Samsung (clinical trials, advisor), Sandoz (clinical trials, advisor), UCB (consultant, clinical trials, advisor), Salvatore D’Angelo: None declared, Francesco Del Galdo: None declared

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Systemic sclerosis portends increased risk of conduction and rhythm abnormalities at diagnosis and during disease course: A US population-based cohort

Journal of Scleroderma and Related Disorders ◽

10.1177/23971983211034074 ◽

2021 ◽

pp. 239719832110340

Author(s):

Yasser A Radwan ◽

Reto D Kurmann ◽

Avneek S Sandhu ◽

Edward A El-Am ◽

Cynthia S Crowson ◽

...

Keyword(s):

Risk Factors ◽

Pulmonary Hypertension ◽

Systemic Sclerosis ◽

Confidence Interval ◽

Cumulative Incidence ◽

Disease Onset ◽

Population Based ◽

Hazard Ratio ◽

Conduction Disorders ◽

Increased Risk

Objectives: To study the incidence, risk factors, and outcomes of conduction and rhythm disorders in a population-based cohort of patients with systemic sclerosis versus nonsystemic sclerosis comparators. Methods: An incident cohort of patients with systemic sclerosis (1980–2016) from Olmsted County, MN, was compared to age- and sex-matched nonsystemic sclerosis subjects (1:2). Electrocardiograms, Holter electrocardiograms, and a need for cardiac interventions were reviewed to determine the occurrence of any conduction or rhythm abnormalities. Results: Seventy-eight incident systemic sclerosis cases and 156 comparators were identified (mean age 56 years, 91% female). The prevalence of any conduction disorder before systemic sclerosis diagnosis compared to nonsystemic sclerosis subjects was 15% versus 7% ( p = 0.06), and any rhythm disorder was 18% versus 13% ( p = 0.33). During a median follow-up of 10.5 years in patients with systemic sclerosis and 13.0 years in nonsystemic sclerosis comparators, conduction disorders developed in 25 patients with systemic sclerosis with cumulative incidence of 20.5% (95% confidence interval: 12.4%–34.1%) versus 28 nonsystemic sclerosis patients with cumulative incidence of 10.4% (95% confidence interval: 6.2%–17.4%) (hazard ratio: 2.57; 95% confidence interval: 1.48–4.45), while rhythm disorders developed in 27 patients with systemic sclerosis with cumulative incidence of 27.3% (95% confidence interval: 17.9%–41.6%) versus 43 nonsystemic sclerosis patients with cumulative incidence of 18.0% (95% confidence interval: 12.3%–26.4%) (hazard ratio: 1.62; 95% confidence interval: 1.00–2.64). Age, pulmonary hypertension, and smoking were identified as risk factors. Conclusion: Patients with systemic sclerosis have an increased risk of conduction and rhythm disorders both at disease onset and over time, compared to nonsystemic sclerosis patients. These findings warrant increased vigilance and screening for electrocardiogram abnormalities in systemic sclerosis patients with pulmonary hypertension.

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