TY - JOUR
T1 - Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry
AU - The PHAROS Investigators
AU - Hinchcliff, Monique
AU - Khanna, Saira
AU - Hsu, Vivien M.
AU - Lee, Jungwha
AU - Almagor, Orit
AU - Chang, Rowland W.
AU - Steen, Virginia
AU - Chung, Lorinda
AU - Bolster, Marcy B.
AU - Csuka, Mary E.
AU - Derk, Chris T.
AU - Domsic, Robyn T.
AU - Fischer, Aryeh
AU - Frech, Tracy
AU - Furst, Daniel
AU - Goldberg, Avram Z.
AU - Gomberg-Maitland, Mardi
AU - Gordon, Jessica K.
AU - Hummers, Laura K.
AU - Kassab, Firas
AU - Molitor, Jerry A.
AU - Preston, Ioana
AU - Ann Saketkoo, Lesley
AU - Schiopu, Elena
AU - Silver, Rick
AU - Simms, Robert
AU - Varga, John
N1 - Publisher Copyright:
© 2015 Elsevier Inc.
PY - 2015/12
Y1 - 2015/12
N2 - Objective: To determine the association between serum autoantibodies and survival in patients with incident systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Methods: Patients with definite PAH diagnosed by right heart catheterization within 6 months of registry enrollment were studied. Serum autoantibodies were assayed at each participating institution's clinical laboratory. Mortality data were collected from electronic medical records and/or the Social Security Death Index. Kaplan-Meier survival estimates were reported for five autoantibody groups (anticentromere/AC, nucleolar ANA/NUC, anti-topoisomerase/Scl-70, overlapping or non-specific autoantibodies/other, and a combined group with similar survival consisting of RNA polymerase III, U1RNP, and autoantibody-negative patients). Cox proportional hazards models permitted examination of the association between autoantibody groups and overall survival, controlling for age, sex, race, and SSc disease duration. Results: In all, 162 subjects had PAH, and serum autoantibody and survival information; 60 (37%) had AC, 39 (24%) NUC, 11 (7%) Scl-70, 28 (17%) had other, 9 (6%) RNA pol, 8 (5%) U1RNP autoantibodies, and 7 (4%) had negative antibodies; 32 (20%) subjects died over a median follow-up time of 2.1 years (range: 0.01-6.8); 1- and 3-year survival estimates were, respectively, 94% and 78% for AC, 94% and 72% for NUC, 89% and 63% for Scl-70, 92% and 79% for the other group, and 100% and 93% for the combined group. Unadjusted and adjusted hazard ratios revealed no statistically significant association between risk of death and autoantibodies. Conclusion: Anticentromere and NUC autoantibodies are prevalent in SSc-PAH patients. An association between serum autoantibodies and survival in patients with SSc-PAH was not identified in the PHAROS cohort.
AB - Objective: To determine the association between serum autoantibodies and survival in patients with incident systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Methods: Patients with definite PAH diagnosed by right heart catheterization within 6 months of registry enrollment were studied. Serum autoantibodies were assayed at each participating institution's clinical laboratory. Mortality data were collected from electronic medical records and/or the Social Security Death Index. Kaplan-Meier survival estimates were reported for five autoantibody groups (anticentromere/AC, nucleolar ANA/NUC, anti-topoisomerase/Scl-70, overlapping or non-specific autoantibodies/other, and a combined group with similar survival consisting of RNA polymerase III, U1RNP, and autoantibody-negative patients). Cox proportional hazards models permitted examination of the association between autoantibody groups and overall survival, controlling for age, sex, race, and SSc disease duration. Results: In all, 162 subjects had PAH, and serum autoantibody and survival information; 60 (37%) had AC, 39 (24%) NUC, 11 (7%) Scl-70, 28 (17%) had other, 9 (6%) RNA pol, 8 (5%) U1RNP autoantibodies, and 7 (4%) had negative antibodies; 32 (20%) subjects died over a median follow-up time of 2.1 years (range: 0.01-6.8); 1- and 3-year survival estimates were, respectively, 94% and 78% for AC, 94% and 72% for NUC, 89% and 63% for Scl-70, 92% and 79% for the other group, and 100% and 93% for the combined group. Unadjusted and adjusted hazard ratios revealed no statistically significant association between risk of death and autoantibodies. Conclusion: Anticentromere and NUC autoantibodies are prevalent in SSc-PAH patients. An association between serum autoantibodies and survival in patients with SSc-PAH was not identified in the PHAROS cohort.
KW - Mortality
KW - Pulmonary arterial hypertension
KW - Pulmonary hypertension
KW - Risk factors
KW - Scleroderma
KW - Serum autoantibody
KW - Systemic sclerosis
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U2 - 10.1016/j.semarthrit.2015.06.011
DO - 10.1016/j.semarthrit.2015.06.011
M3 - Article
C2 - 26210782
AN - SCOPUS:84955693022
SN - 0049-0172
VL - 45
SP - 309
EP - 314
JO - Seminars in Arthritis and Rheumatism
JF - Seminars in Arthritis and Rheumatism
IS - 3
ER -