Session Information
Date: Wednesday, November 11, 2015
Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud's - Clinical Aspects and Therapeutics III
Session Type: ACR Concurrent Abstract Session
Session Time: 11:00AM-12:30PM
Background/Purpose:
Microangiopathy in systemic sclerosis (SSc), as visualized by nailfold videocapillaroscopy (NVC) is a dynamic and sequential process1. In time, NVC patterns progress from early, to active and finally late SSc pattern. A relation between improvement of NVC pattern and treatment with immunosuppressive drugs has been suggested2,3. We aimed to evaluate 1. change of NVC patterns in SSc patients, and 2. influence of treatment with cyclophosphamide (CYC) and autologous hemopoetic stem cell transplantation (HSCT) on change of NVC patterns.
Methods:
NVC was performed annually in SSc patients participating in a care program. For the current analysis, patients were included if at least two NVCs were performed and qualitatively assessed (normal, borderline changes, or SSc pattern [early, active or late]). Change of NVC pattern was classified as: 1. stable (same pattern at all evaluations), 2. progressive (change from early to active/ late or from active to late) or 3. reverse transition (change from late to early/active, or from active to early) . In order to asses the association between CYC and HSCT with relative rates of transition of NVC patterns a maximum likelihood estimation in a multistate model was used, which, additionally to correction for age, gender and disease duration, corrects for time to transition in relation to drug exposure time.
Results: NVC was performed twice in n=98 patients, 3 times in n= 35 patients and 4 times in n=5 patients. Of these 138 patients, 82% was female, mean age was 51 years, and median disease duration was 3 years. At baseline 6 patients had a normal pattern, n=4 borderline changes, n=13 an early pattern, n=54 an active pattern, and n=61 a late pattern. Over time, NVC pattern was stable in 53%, progressive in 21% and 26% showed reverse transition (table 1). Treatment with HSCT was significantly associated with higher rate of reverse transition (relative transition rates [95% CI] 5.4 [1.0-28.8]). No association between CYC and rate and direction of change of NVC pattern was found.
Conclusion:
Change of NVC pattern was observed in 47% of SSc patients; 26% showed reverse transition. Rapid reverse transition was associated with treatment with HSCT but not with CYC, indicating that more aggressive immunosuppressive treatment can influence microangiopathy in SSc.
(1) Arthritis Rheum 2012;64:821.
(2) Ann Rheum Dis 2008 ;67:1057.
(3) PLoS One 2008;3:e1452.
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Table 1. Baseline characteristics of SSc patients stratified according to change of NVC pattern |
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* |
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Total |
Stable |
Progressive |
Reverse |
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N =138 |
N=65 |
N=31 |
N=42 |
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Sociodemographics |
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|
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Age, years, mean (SD) |
51 (13.6) |
50.7 (12.6) |
48.4 (17.5) |
53.6 (11.6) |
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Female, N (%) |
113 (82) |
55 (84.6) |
29 (93.5) |
29 (69) |
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Caucasian origin, N (%) |
98 (71) |
44 (67.7) |
23 (74.2) |
31 (73.8) |
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Disease characteristics |
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|
|
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Disease duration, years, median (IQR) |
3 (1-10.3) |
3 (1-11) |
1 (0-8) |
4 (1-10) |
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Onset of RP, years, median (IQR)§ |
11.5 (4.8-19.3) |
13 (5-21.3) |
8 (3-15) |
10 (5-17) |
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Onset of non-RP, years, median (IQR)† |
6 (3-12) |
8 (3-13) |
3 (1.3-9.5) |
5 (3-10) |
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DU |
38 (27.5) |
20 (30.8) |
10 (32.3) |
8 (19) |
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Pitting scars |
64 (46.4) |
37 (56.9) |
13 (41.9) |
14 (33.3) |
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Synovitis |
16 (11.6) |
8 (12.3) |
5 (16.1) |
3 (7.1) |
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Friction rubs |
3 (2.2) |
1 (1.5) |
2 (6.5) |
0 (0) |
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Proximal muscular weakness |
6 (4.3) |
3 (4.6) |
1 (3.2) |
2 (4.9) |
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Renal crisis |
3 (2.2) |
2 (3.1) |
0 (0) |
1 (2.4) |
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MRSS, median (IQR) |
4 (0-6) |
4 (0-6) |
4 (2-6) |
2 (0-6) |
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Autoantibodies, N (%) |
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|
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ANA§ |
120 (87) |
58 (89.2) |
28 (90.3) |
34 (81) |
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Anti-Scl-70† |
35 (25) |
15 (23.1) |
10 (32.3) |
10 (23.8) |
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Anti-centromere† |
46 (33) |
22 (33.8) |
8 (25.8) |
16 (38.1) |
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Cardiopulmonary investigations |
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FVC % of predicted, mean (SD)§ |
101.5 (22.1) |
101.1 (23) |
103.8 (21.7) |
100.4 (21.5) |
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DLCO % of predicted, mean (SD)§ |
63.8 (16.2) |
62.8 (16.9) |
66.5 (16.7) |
63.2 (15) |
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ILD, N (%) |
77 (55.8) |
36 (55.4) |
15 (48.4) |
26 (61.9) |
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SPAP≥35mmHg, N (%) |
10 (7.2) |
6 (9.2) |
0 (0) |
4 (9.5) |
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LVEF %, mean (SD) |
59.6 (7.6) |
58.7 (7.3) |
62.8 (7.9) |
58.5 (7.5) |
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Pericardial fluid, N (%) |
4 (2.9) |
2 (3.1) |
1 (3.2) |
1 (2.4) |
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Nailfold videocapillaroscopy, N (%) |
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Normal |
6 (4.3) |
1 (1.5) |
5 (16.1) |
NA |
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Borderline changes |
4 (2.9) |
1 (1.5) |
2 (6.5) |
1 (2.4) |
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SSc pattern |
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|
|
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Early |
13 (9.4) |
1 (1.5) |
7 (22.6) |
5 (11.9) |
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Active |
54 (39.1) |
27 (41.5) |
17 (54.8) |
10 (23.8) |
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Late |
61 (44.2) |
35 (53.8) |
NA |
26 (61.9) |
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Immunosuppressive treatmentD |
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|
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Previous |
52 (37.7) |
21 (32.3) |
12 (38.7) |
19 (45.2) |
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Current |
38 (27.5) |
19 (29.2) |
10 (32.3) |
9 (21.4) |
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DcSSc: diffuse cutaneous SSc; LcSS: limited cutaneous SSc; LSSc: limited non |
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cutaneous SSc; RP: Raynaud’s Phenomenon; MRSS: modified Rodnan Skin Score; |
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ANA: antinuclear antibody; Anti-Scl-70: anti-topoisomerase; FVC: forced vital capacity; |
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DLCO: diffusing capacity for carbon monooxide; ILD: interstitial lung disease; SPAP: |
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systolic pulmonary arterial pressure; LVEF: left ventricle ejection fraction; IQR: interquartile |
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range; NA: not applicable. |
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*According to the last available NVC |
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D Any of the following: autologous hematopoeitic stem cell transplantation, cyclophosphamide, |
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mycophenolate mofetil, methotrexate, corticosteroids or rituximab. |
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§ <5% missing |
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† 20% missing |
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To cite this abstract in AMA style:
Meijs J, de Boer B, Schouffoer A, Huizinga TWJ, Putter H, de Vries-Bouwstra JK. The Influence of Immunosuppressive Therapy on Microangiopathy in Systemic Sclerosis As Measured with Nailfoldcapillaroscopy [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/the-influence-of-immunosuppressive-therapy-on-microangiopathy-in-systemic-sclerosis-as-measured-with-nailfoldcapillaroscopy/. Accessed .ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-influence-of-immunosuppressive-therapy-on-microangiopathy-in-systemic-sclerosis-as-measured-with-nailfoldcapillaroscopy/