Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: Severe nailfold videocapillaroscopy (NVC) patterns in patients with systemic sclerosis (SSc) are associated with a high risk of organ involvement. SSc-specific autoantibodies seem to be associated with different NVC scleroderma patterns. Whether the combination of autoantibodies and NVC pattern contributes to better discrimination of patients at risk for organ involvement remains unclear. The aim of this study is to explore the association between clinical phenotype and the combination of autoantibodies and NVC pattern, in order to compose individualized screening programs for organ involvement.
Methods: Data on NVC patterns and anti-centromere (ACA), anti-topoisomerase (anti-Scl70) and anti-ribonucleoprotein (anti-RNP) autoantibodies was investigated in 167 patients of the Leiden Systemic Sclerosis Cohort. The prevalence of autoantibodies and NVC scleroderma patterns (early, active, late) was described. Clinical phenotypes were evaluated for different combinations of autoantibodies and NVC pattern.
Results: 148/167 patients (89%) had a NVC scleroderma pattern: early in 16 patients (11%), active in 77 patients (52%), late in 55 patients (37%). Of these 148 patients, 82 (55%) were ACA+, 52 (35%) were anti-Scl-70+ and 17 (11%) were anti-RNP+. In 19 patients with a non-scleroderma NVC pattern, 11 (58%) were ACA+, 6 (32%) were anti-Scl-70+ and 2 (11%) were anti-RNP+.
Similar distributions of NVC patterns were found in ACA+ and ACA‒ patients (p=0.185), as also in anti-Scl-70+ and anti-Scl-70‒ patients (p=0.293). None of 17 anti-RNP+ patients had an early pattern, compared to 16/131 anti-RNP‒ patients (12%) (p=0.127). Overall, and probably due to small numbers, no significant difference in distribution of NVC patterns between anti-RNP+ and anti-RNP‒ patients was shown (p=0.178).
ACA+ patients with an early pattern tend to have less organ involvement than patients with an active or late pattern: less skin involvement, higher gas transfer for CO (DLCO), less vascular lesions (table). Early patterns in anti-Scl-70+ (n=3) and anti-RNP+ patients (n=0) were too rare to evaluate. In anti-Scl-70+ patients with a late pattern, more interstitial lung disease, lower DLCO, less vascular lesions and a higher NT-proBNP were seen, compared to an active pattern (table). Similar trends were seen in anti-RNP+ patients (table). ACA+, anti-Scl-70+ and anti-RNP+ patients with a late NVC pattern, seem to have more often a DLCO <70% of predicted and a higher NT-proBNP.
Conclusion: Early NVC patterns in ACA+ SSc patients seem to correlate with less severe organ involvement, while a late pattern in ACA+, anti-Scl-70+ or anti-RNP+ patients seems to correlate with more severe organ involvement. These data indicate that the combination of NVC pattern and antibodies can contribute to discrimination of SSc patients that need extensive organ screening.
|
Table: Clinical phenotypes stratified for systemic sclerosis specific autoantibodies and nailfold capillaroscopy pattern (anti-Scl-70+ patients and anti-RNP+ patients with an early pattern were left out because of low numbers, n=3 and n=0, respectively). |
|||||||
|
|
ACA+
|
anti-Scl70+
|
anti-RNP+
|
||||
|
|
Early
|
Active
|
Late
|
Active
|
Late
|
Active
|
Late
|
|
n=12 |
n=43 |
n=27 |
n=27 |
n=22 |
n=8 |
n=9 |
|
|
Patient characteristics
|
|
|
|
|
|
|
|
|
Age in years, mean (SD) |
59 (15) |
52 (14) |
62 (12) |
45 (14) |
55 (13) |
44 (7) |
46 (14) |
|
Female, n (%) |
11 (92) |
39 (91) |
24 (89) |
22 (82) |
18 (82) |
7 (88) |
7 (78) |
|
Skin
|
|
|
|
|
|
|
|
|
SSc type, n (%) |
|
|
|
|
|
||
|
lcSSc
|
9 (75) |
26 (61) |
20 (74) |
13 (48) |
7 (32) |
6 (75) |
8 (89) |
|
dcSSc
|
3 (25) |
17 (40) |
2 (7) |
13 (48) |
15 (68) |
1 (13) |
1 (11) |
|
lSSc
|
0 |
0 |
5 (19) |
1 (4) |
0 |
1 (13) |
0 |
|
mRSS, median (IQR) |
2 (0.5 – 4) |
2 (0 – 4) |
4 (2 – 6) |
4 (2 – 7) |
7 (4 – 13) |
2 (0.5 – 4) |
4 (2 – 8) |
|
Lung
|
|
|
|
|
|
|
|
|
Interstitial lung disease, n (%) |
1 (8) |
14 (33) |
6 (22) |
20 (74) |
20 (91) |
4 (50) |
6 (67) |
|
DLCO % of predicted, mean (SD) |
73 (10) |
71 (18) |
61 (13) |
64 (17) |
57 (14) |
60 (12) |
49 (9) |
|
DLCO <70% of predicted, n (%) |
3 (25) |
20 (47) |
17 (63) |
16 (59) |
18 (82) |
5 (63) |
9 (100) |
|
Kidney
|
|
|
|
|
|
|
|
|
eGFR, median (IQR) |
71 (66 – 110) |
96 (76 – 101) |
82 (66 – 86) |
92 (82 – 115) |
91 (71 – 105) |
98 (91 – 148) |
103 (81 – 144) |
|
eGFR ≤60 ml/min, n (%) |
2 (17) |
3 (7) |
5 (19) |
3 (11) |
2 (9) |
0 |
1 (11) |
|
Vascular
|
|
|
|
|
|
|
|
|
Digital ulcers, n (%) |
2 (17) |
14 (33) |
13 (48) |
7 (26) |
10 (46) |
2 (25) |
5 (56) |
|
Pitting scars, n (%) |
1 (8) |
16 (37) |
15 (56) |
8 (30) |
14 (64) |
4 (50) |
9 (100) |
|
Heart
|
|
|
|
|
|
|
|
|
NT-proBNP, median (IQR)
|
93 (61 – 124) |
68 (49 – 114) |
159 (42 – 382) |
60 (48 – 99) |
83 (42 – 369) |
147 (136 – 273) |
197 (54 – 618) |
|
Muscle
|
|
|
|
|
|
|
|
|
CPK, mean (SD) |
76 (61 – 143) |
77 (53 – 106) |
92 (66 – 105) |
72 (54 – 181) |
85 (63 – 132) |
60 (40 – 83) |
71 (54 – 125) |
|
CPK >145 U/L, n (%) |
3 (25) |
1 (2) |
1 (4) |
8 (30) |
5 (23) |
0 |
1 (11) |
|
Proximal muscle weakness, n |
0 |
1 |
0 |
1 |
1 |
1 |
2 |
|
Note: ‘Early’, ‘active’ and ‘late’ refer to the systemic sclerosis specific nailfold capillaroscopy patterns. |
|||||||
|
ACA, anti-centromere; anti-Scl70, anti-topoisomerase; anti-RNP, anti-ribonucleoprotein; SD, standard deviation; SSc, systemic sclerosis; lcSSc, limited cutaneous systemic sclerosis; dcSSc, diffuse cutaneous systemic sclerosis; lSSc, limited cutaneous systemic sclerosis; mRSS, modified Rodnan skin score; IQR, interquartile range; DLCO, diffusion capacity of the lung for carbon monoxide; eGFR, estimated glomerular filtration rate, NT-proBNP, NT-pro-brain-natriuretic peptide; CPK, creatinine phosphokinase. |
|||||||
Disclosure:
I. M. Markusse,
None;
J. Meijs,
Actelion Pharmaceuticals US,
2;
B. de Boer,
None;
A. A. Schouffoer,
None;
N. Ajmone Marsan,
None;
L. J. M. Kroft,
None;
M. K. Ninaber,
None;
T. W. J. Huizinga,
None;
J. K. de Vries-Bouwstra,
None.
« Back to 2014 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-additive-value-of-nailfold-videocapillaroscopy-patterns-to-disease-specific-autoantibodies-in-discrimination-of-patients-with-systemic-sclerosis-at-risk-for-severe-organ-involvement/