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 April 5, 2025.« 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/