TY - JOUR
T1 - Adult-onset Still's disease in Western Australia
T2 - Epidemiology, comorbidity and long-term outcome
AU - Nossent, Johannes
AU - Raymond, Warren
AU - Keen, Helen
AU - Preen, David B.
AU - Inderjeeth, Charles A.
PY - 2022/11
Y1 - 2022/11
N2 - Aim: Adult-onset Still's disease (ASD) is a rare, potentially life-threatening autoinflammatory condition. As reported prevalence shows regional variation and long-term outcome data are scarce, we investigated epidemiology and long-term health outcomes of ASD in Western Australia (WA).Methods: Population-based cohort study using longitudinally linked administrative health data from all WA hospitals between 1999 and 2013 for ASD patients (ICD-10-AM M06.1) and controls matched for age, gender, and index year. Rate ratios and odds ratios (RR/OR) with 95% confidence intervals (CI) compared ASD patients with controls. Results: The average ASD incidence (n = 52) was 0.22/100000 with 2.4/100000 point-prevalence as of December 31, 2013. ASD patients (median age 41.5 years, 59.6% female) had higher odds of previous liver disease (OR 2.67, 95% CI 1.31-5.45), fever (OR 54.10, 95% CI 6.60-433.0), rash (OR 15.70, 95% CI 4.08-60.80), and serious infections (OR 4.36, 95% CI 2.11-22.80) than controls. Despite biological disease-modifying antirheumatic drugs in 27% of patients, ASD patients had higher odds for joint replacement (n = 7, 13.5%) (OR 45.5, 95% CI 4.57-93.70), osteoporosis (OR 31.3, 95% CI 3.43-97), and serious infections (RR 5.68; 95% CI 6.61-8.74) during follow up. However, crude mortality (11.5% vs 7.5%; P = 0.34), survival at 1 and 5 years (P=0.78), and last modified Charlson Comorbidity score (median 2 vs 2) were similar between groups. Conclusion: The epidemiology and demographics of ASD in Western Australia fall within the internationally reported range. ASD patients present increased rates of liver disease, rash, and serious infections before disease onset. Mortality following ASD was not increased for 5 years despite high rates of chronic arthritis requiring joint replacement, serious infections, and osteoporosis.
AB - Aim: Adult-onset Still's disease (ASD) is a rare, potentially life-threatening autoinflammatory condition. As reported prevalence shows regional variation and long-term outcome data are scarce, we investigated epidemiology and long-term health outcomes of ASD in Western Australia (WA).Methods: Population-based cohort study using longitudinally linked administrative health data from all WA hospitals between 1999 and 2013 for ASD patients (ICD-10-AM M06.1) and controls matched for age, gender, and index year. Rate ratios and odds ratios (RR/OR) with 95% confidence intervals (CI) compared ASD patients with controls. Results: The average ASD incidence (n = 52) was 0.22/100000 with 2.4/100000 point-prevalence as of December 31, 2013. ASD patients (median age 41.5 years, 59.6% female) had higher odds of previous liver disease (OR 2.67, 95% CI 1.31-5.45), fever (OR 54.10, 95% CI 6.60-433.0), rash (OR 15.70, 95% CI 4.08-60.80), and serious infections (OR 4.36, 95% CI 2.11-22.80) than controls. Despite biological disease-modifying antirheumatic drugs in 27% of patients, ASD patients had higher odds for joint replacement (n = 7, 13.5%) (OR 45.5, 95% CI 4.57-93.70), osteoporosis (OR 31.3, 95% CI 3.43-97), and serious infections (RR 5.68; 95% CI 6.61-8.74) during follow up. However, crude mortality (11.5% vs 7.5%; P = 0.34), survival at 1 and 5 years (P=0.78), and last modified Charlson Comorbidity score (median 2 vs 2) were similar between groups. Conclusion: The epidemiology and demographics of ASD in Western Australia fall within the internationally reported range. ASD patients present increased rates of liver disease, rash, and serious infections before disease onset. Mortality following ASD was not increased for 5 years despite high rates of chronic arthritis requiring joint replacement, serious infections, and osteoporosis.
KW - adults
KW - comorbidity
KW - epidemiology
KW - lookback
KW - Still's disease
KW - survival
KW - MANIFESTATIONS
UR - http://www.scopus.com/inward/record.url?scp=85136507979&partnerID=8YFLogxK
U2 - 10.1111/1756-185X.14424
DO - 10.1111/1756-185X.14424
M3 - Article
C2 - 36004429
SN - 1756-1841
VL - 25
SP - 1306
EP - 1314
JO - International Journal of Rheumatic Diseases
JF - International Journal of Rheumatic Diseases
IS - 11
ER -