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Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):98-105. Epub 2017 Sep 21.

Influence of antibody profile in clinical features and prognosis in a cohort of Spanish patients with systemic sclerosis.

Author information

1
Department of Autoimmune Diseases, Institut Clinic de Medicina i Dermatología, Hospital Clínic, Barcelona, Spain.
2
Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
3
Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain.
4
Department of Internal Medicine, Hospital Universitario de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain.
5
Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain.
6
Department of Internal Medicine, Hospital Universitario Virgen del Rocío, Sevilla, Spain.
7
Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain.
8
Department of Internal Medicine, Hospital Universitario Cruces, Barakaldo, Spain.
9
Systemic Autoimmune Diseases Unit, Hospital Campus de la Salud, Complejo Universitario de Granada, Spain.
10
Thrombosis and Vasculitis Unit, Department of Internal Medicine, Complexo Hospitalario Universitario de Vigo, Spain.
11
Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Sabadell, Barcelona, Spain.
12
Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada, Spain.
13
Department of Internal Medicine, Hospital Universitario La Paz, Madrid, Spain.
14
Department of Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
15
Department of Internal Medicine, Hospital Universitari Mútua Terrassa, Barcelona, Spain.
16
Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.
17
Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Spain.
18
Department of Internal Medicine, Consorci Hospitalari de Vic, Barcelona, Spain.
19
Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain.
20
Department of Internal Medicine, Hospital Universitario Fundación Alcorcón, Madrid, Spain.
21
Department of Autoimmune Diseases, Institut Clinic de Medicina i Dermatología, Hospital Clínic, Barcelona, Spain. gespino@clinic.cat.

Abstract

OBJECTIVES:

To assess the clinical manifestations and prognosis of Spanish patients with systemic sclerosis (SSc) according to their immunological profile.

METHODS:

From the Spanish Scleroderma Study Group or RESCLE (Registro de ESCLErodermia as Spanish nomenclature) Registry we selected those patients in which anti-centromere (ACA), anti-topoisomerase I (ATA), and anti-RNA polymerase III (ARA) antibodies had been determined, and a single positivity for each SSc specific antibody was detected. Demographic, clinical, laboratory, and survival data were compared according to the serologic status of these antibodies.

RESULTS:

Overall, 209 SSc patients were included. In 128 (61%) patients ACA was the only positive antibody, 46 (22%) were only positive for ATA, and 35 (17%) for ARA. Of note, the three groups were mutually exclusive. In univariate analysis, patients with ACA presented more frequently limited cutaneous SSc (lcSSc) (p<0.001), whereas diffuse cutaneous SSc (dcSSc) was the most frequent subtype in patients with ATA (54%) and ARA (62%) (both p<0.001). Positive patients for ARA showed the highest prevalence of joint involvement (p<0.001) and those from ATA group had a higher prevalence of interstitial lung disease (ILD) (p<0.001). Scleroderma renal crisis was more frequent in the ARA group (p<0.001). In multivariate analysis, ACA were associated with female gender and were protective for dcSSc and ILD. ATA were found to be protective for lcSSc and they were independently associated with interstitial reticular pattern. ARA positivity was independently associated with dcSSc. We did not find differences in mortality between the three groups.

CONCLUSIONS:

In Spanish SSc patients, the presence of SSc specific antibodies conferred a distinctive clinical profile.

PMID:
28980912
[Indexed for MEDLINE]
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