Systemic Sclerosis Mortality High in Asians - MedPage Today

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Systemic Sclerosis Mortality High in Asians - MedPage Today Posted: 24 Mar 2020 12:01 PM PDT Among patients with system sclerosis (SSc) in Northern California, death came sooner to those of Asian ethnicity than in other groups, a retrospective cohort study found. On a multivariable analysis that adjusted for multiple factors including demographics, disease subtype, and pulmonary or renal involvement, Asian patients with SSc had an increased risk of mortality (HR 1.80, 95% CI 0.99-1.68, P=0.047), according to Lorinda Chung, MD, of Stanford University in California, and colleagues. But all the included minority groups had lower 9-year survival rates than whites, with rates found to be 52.3% in Asians, 52.2% in blacks, and 68.2% in Hispanics compared with 75.8% for whites (P=0.057), the researchers reported online in ACR Open Rheumatology. Race and ethnicity are recognized as having significant impact on clinical manifestations, autoantibody patterns, and survival with SSc. For instance, numerous studies have found that black patients typically have a more severe phenotype and worse prognosis than whites, and studies of Asian patients residing in Asia also have found more severe clinical disease. However, little is known about disease characteristics and outcomes among patients of Asian heritage living in the U.S., so Chung and colleagues reviewed the electronic medical records of patients in the Kaiser Permanente Northern California healthcare system who were diagnosed with SSc from 2007 to 2016. The team obtained information on age, sex, SSc subtype (limited vs diffuse cutaneous disease), organ involvement, and the presence of autoantibodies including antinuclear antibody and anticentromere, anti-Scl-70, anti-SSA/Ro, anti-SSB/La, and anti-U1-RNP antibodies. The analysis included 609 adults with SSc, most of whom were women with limited cutaneous disease. Their mean age at the time of diagnosis was 55 and mean disease duration was 5 years; 51% were white, 25% were Hispanic, 16% were Asian, and 8% were black. The Asian group included Chinese, Filipinos, Japanese, Vietnamese, and Koreans. White patients were older than other groups, with a mean age at diagnosis of 59. Blacks more often had diffuse cutaneous disease than whites (44.9% vs 14.5%, P<0.001), while Asians and Hispanics were less likely to have ever smoked compared with whites (17.4% and 19.9% vs 43.3%, P<0.001). Anti-U1-RNP antibodies were detected more often in Asians than in whites (32.1% vs 11.9%, P=0.005), and Asians also had the highest prevalence of anti-Scl-70 (25.6%) and anti-SSA/Ro antibodies (22.2%). Organ involvement did not differ significantly among the groups. The cumulative mortality rate was 17.2% during a median follow-up of 5 years, but differed among the groups: 15.5% for whites, 14.9% for Hispanics, 22.9% for Asians, and 24.5% for blacks. Five-year survival rates were 85.3% for whites, 86.4% for Hispanics, 76.4% for Asians, and 76.2% for blacks. In an unadjusted analysis, increased mortality compared with whites was seen for Asians (HR 1.83, 95% CI 1.08-2.99, P=0.020) and blacks (HR 1.69, 95% CI 0.86-3.08, P=0.104). On a multivariate analysis that adjusted only for age, race, and smoking, increased risk of death was seen for both Asians (HR 2.42, 95% CI 1.38-4.14, P=0.001) and blacks (HR 2.1, 95% CI 1.05-3.89, P=0.026). After adjustment for additional variables such as disease subtype, pulmonary hypertension, interstitial lung disease, and scleroderma renal crisis, Asians still remained at elevated risk. Causes of death also differed among the groups. For whites, the most common cause was pulmonary fibrosis, while among Hispanics the most common cause was pulmonary hypertension. For blacks, multi-organ failure was the predominant cause, and for Asians, death most often resulted from pulmonary hypertension or infection. "The variability of disease expression among racial groups in our study suggests that multiple factors linked to race, including genetic and environmental factors, may influence clinical manifestations, disease course, autoantibody status, and mortality in SSc," Chung and colleagues wrote. Genetic factors might include the presence of different HLA antigen alleles and small nucleotide polymorphisms in the fibrillarin-1 gene, and further exploration of the genetic contributions should provide additional elucidation of the biologic underpinnings of SSc. "Understanding racial/ethnic variations in disease outcomes is critical because this will improve our ability to provide personalized medicine by informing patient counseling, appropriate baseline screening, and monitoring of disease progression," the investigators concluded. A limitation of the study, they noted, was its setting in a community-based healthcare organization, so the results may not be generalizable to the uninsured or to patients treated in specialized centers. Disclosures Funding for the study was provided by Kaiser Permanente Northern California Graduate Medical Education Research. The authors also reported support from the Training Program in Adult and Pediatric Rheumatology and the Scleroderma Research Foundation. One co-author was an investigator on an unrelated FDA-mandated safety study funded by Bayer Global. Secondary Source Source Reference: Additional Source Source Reference:

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