Diagnosis
Serodiscordant patients with systemic sclerosis: when antibody does not correspond to skin involvement
N. Iniesta Arandia1, G. Espinosa2, C. Tolosa Vilella3, A. Guillén Del Castillo4, M. Rubio Rivas5, M. Freire6, J.A. Vargas Hitos7, J.A. Todolí Parra8, M. Rodríguez Carballeira9, A. Marín Ballvé10, D. Colunga Argüelles11, C. González De Echávarri Pérez De Heredia12, N. Ortego-Centeno13, L. Trapiella Martínez14, X. Pla Salas15, A.J. Chamorro16, I. Perales Fraile17, M. Ruiz Muñoz18, R.Á. Fernández De La Puebla Giménez19, A.B. Madroñero Vuelta20, I. Pons Martín Del Campo21, I. Jiménez Pérez De Heredia22, A. González García23, V. Fonollosa Pla24, C.P. Simeón Aznar25
- Department of Autoimmune Diseases, Institut Clinic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Spain.
- Department of Autoimmune Diseases, Institut Clinic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Spain. gespino@clinic.cat
- Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
- Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Sabadell, Barcelona, Spain.
- Department of Internal Medicine, Hospital Universitario de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain.
- Thrombosis and Vasculitis Unit, Department of Internal Medicine, Complexo Hospitalario Universitario de Vigo, Spain.
- Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada, Spain.
- Department of Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
- Department of Internal Medicine, Hospital Universitari Mútua Terrassa, Barcelona, Spain.
- Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.
- Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain.
- Department of Internal Medicine, Hospital Universitario Cruces, Bizcaia, Barakaldo, Spain.
- Systemic Autoimmune Diseases Unit, Hospital Campus de la Salud, Complejo Universitario de Granada, Spain.
- Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain.
- Department of Internal Medicine, Consorci Hospitalari de Vic, Barcelona, Spain.
- Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Spain.
- Department of Internal Medicine, Hospital Universitario Rey Juan Carlos, Móstoles, Madrid, Spain.
- Department of Internal Medicine, Hospital Universitario Fundación Alcorcón, Madrid, Spain.
- Department of Internal Medicine, Hospital Universitario Reina Sofía, Córdoba, Spain.
- Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain.
- Department of Internal Medi- cine, Xarxa Assistencial Universitària de Manresa, Barcelona, Spain.
- Department of Internal Medicine, Hospital de Sagunto, Sagunto, Valencia, Spain.
- Department of Internal Medicine, Hospital Universitario Ramón y Cajal, Madrid, Spain.
- Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
- Autoimmune Unit, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
on behalf of RESCLE Investigators, Autoimmune Diseases Study Group (GEAS)
CER12469
2020 Vol.38, N°3 ,Suppl.125
PI 0106, PF 0114
Diagnosis
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PMID: 31969223 [PubMed]
Received: 31/05/2019
Accepted : 26/09/2019
In Press: 14/01/2020
Published: 26/08/2020
Abstract
OBJECTIVES:
Diffuse cutaneous systemic sclerosis (dcSSc) is associated with anti-topoisomerase (ATA) whereas limited cutaneous (lcSSc) and sine scleroderma (ssSSc) are mainly associated with anti-centromere antibody (ACA). Serodiscordant patients were defined as lcSSc subjects with ATA, dcSSc with ACA, and ssSSc with ATA. The aim of the present study was to compare the clinical manifestations and prognosis between serodiscordant patients and their counterparts (those with lcSSc with ACA, dcSSc with ATA and ssSSc with ACA, respectively).
METHODS:
From the Spanish Scleroderma Registry we selected those patients for whom skin involvement (dcSSc, lcSSc or ssSSc) was detailed at baseline and last visit and ACA and ATA had been determined. Demographic, clinical characteristics, and survival data were compared according to the antibody status.
RESULTS:
The whole cohort comprised 901 patients and six mutually exclusive groups were defined: lcSScACA in 511 (57%) patients, lcSScATA group in 87 (10%), dcSScATA group in 172 (19%), dcSScACA group in 21 (2%), ssSScACA group in 92 (10%), and ssSScATA group in 18 (2%) patients, respectively. Interstitial lung disease (ILD) and severe ILD were more frequent in patients with dcSScATA than in those with dcSScACA. Conversely, the prevalence of isolated pulmonary hypertension (without ILD) was higher in those with dcSScACA (15% vs. 2%; p=0.018). No differences were found regarding survival when comparing serodiscordant patients with the seroconcordants patients.
CONCLUSIONS:
In our cohort, the prevalence of serodiscordant SSc patients was low. They differed from their counterparts in some clinical manifestations. The management of patients with SSc should be guided by both serology and cutaneous subtype.
