Session Information
Date: Sunday, November 5, 2017
Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster I
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Serum antibodies are used to identify SSc patients who may be at higher risk for SSc-PH. The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry cohort followed SSc-PH patients. We assessed the validity of prior reports of association between serum antibodies and PH using data from PHAROS and the Northwestern Scleroderma Registry (NW).
Methods: Antibody data were compared between PHAROS and NW subjects (mPAP ≥25 mmHg on right heart catheterization). World Health Organization PH group (pulmonary arterial hypertension/PAH=Group 1, pulmonary venous hypertension/PVH=Group 2, and PH-interstitial lung disease/PH-ILD=Group 3) was determined. The association between antibodies and PH was studied using two approaches: 1) antibody distribution was compared between PHAROS and NW PH subjects; 2) antibody distribution was compared between combined PHAROS and NW PH (in total and by WHO group) and NW subjects without PH. Chi-square or Fisher’s exact tests were used for comparisons.
Results: Clinical characteristics were similar for 326 PHAROS subjects and 768 NW Registry subjects, 84 with PH (Table 1). There were significant differences in antibodies between PHAROS and NW subjects, p=0.005 (Table 1). When the PH groups were combined (n=410) and compared to NW subjects without PH (n=684), the antibodies between the groups were significantly different, p<0.0001 (Table 2). Subjects in the PH group had a higher percentage of isolated nucleolar antibodies (21%) compared to the NW subjects (8%). Similar percentages of ACA were found in both groups (28% for the PH group and 27% for the NW subjects without PH. When the subclassifications of PH groups were examined, the percentage of isolated nucleolar antibodies remained high in all 3 groups.
Conclusion: Isolated nucleolar serum antibodies were more prevalent in PH subjects compared to non-PH NW subjects. When the PH groups were examined separately, higher percentages of isolated nucleolar antibodies remained in all 3 groups. Similar percentages of ACA were found in both the PH group and NW subjects without PH, suggesting that isolated nucleolar antibodies may be a more specific marker for PH than ACA. Screening for isolated nucleolar antibodies may help to identify patients with SSc who are at higher risk for developing PH.
Mean (SD) or n, % PHAROS (All WHO groups) n=326 Age, years 58 (11) Sex, women 262, 80% Race/Ethnicity, Caucasian 236, 72% SSc Subtype, lcSSc 198, 61% mRSS 9 (9) SSc disease duration, years 10 (9) Negative 19, 6% Anticentromere (ACA) 95, 29% RNA Polymerase III 14, 4% Anti-topoisomerase I (Scl-70) 44, 14% U1RNP 13, 4% Isolated nucleolar antinuclear 78, 24% Other 63, 19%
Table 2: Distribution of autoantibodies in PHAROS and Northwestern PH (combined, all WHO groups) compared to Northwestern no PH Autoantibodies n, % NW no PH n=684 p-value Negative 21, 5% 19, 3% <0.0001 Anticentromere (ACA) 115, 28% 188, 27% RNA Polymerase III 23, 6% 130, 19% Anti-topoisomerase I (Scl-70) 62, 15% 151, 22% U1RNP 15, 4% 29, 4% Isolated nucleolar pattern 87, 21% 54, 8% Other 87, 21% 113, 17% Other=includes multiple antibodies, +ANA (homogenous, speckled, or multiple patterns) Table 3: Autoantibodies in the Northwestern no PH group compared to PHAROS and Northwestern PH (combined), by WHO group Autoantibodies n, % PHAROS and NW PH PHAROS and NW PH-ILD (Group 3) N=70 Negative 19, 3% 11, 4% 6, 8% 4, 6% Anticentromere (ACA) 188, 27% 99, 37% 12, 16% 4, 6% RNA Polymerase III 130, 19% 16, 6% 6, 8% 1, 1% Anti-topoisomerase I (Scl-70) 151, 22% 22, 8% 13, 18% 27, 39% U1RNP 29, 4% 9, 3% 3, 4% 3, 4% Isolated nucleolar pattern 54, 8% 55, 21% 19, 26% 13, 19% Other 113, 17% 54, 20% 15, 20% 18, 26% Other= includes multiple antibodies, +ANA (homogenous, speckled, or multiple patterns) When comparing distribution of autoantibodies between NW no PH and each PH group, p<0.0001.
To cite this abstract in AMA style:
Aren K, Carns MA, Cuttica M, Lee J, Steen VD, Hinchcliff M. The Association of Pulmonary Hypertension with Isolated Nucleolar Serum Autoantibodies in Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/the-association-of-pulmonary-hypertension-with-isolated-nucleolar-serum-autoantibodies-in-systemic-sclerosis/. Accessed .« Back to 2017 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-association-of-pulmonary-hypertension-with-isolated-nucleolar-serum-autoantibodies-in-systemic-sclerosis/