Digital ulcers and cutaneous subsets of systemic sclerosis: Clinical, immunological, nailfold capillaroscopy, and survival differences in the Spanish RESCLE Registry

Fecha de publicación: 01/10/2016

Autores de IIS La Fe

José Antonio Todolí Parra

Autor

Participantes ajenos a IIS La Fe

Tolosa-Vilella, C
Morera-Morales, ML
Simeon-Aznar, CP
Mari-Alfonso, B
Colunga-Arguelles, D
Rubio, JLC
Rubio-Rivas, M
Freire-Dapena, M
Guillen-del Castillo, A
Iniesta-Arandia, N
Castillo-Palma, MJ
Egurbide-Arberas, M
Trapiellla-Martinez, L
Vargas-Hitos, JA
Rodriguez-Carballeira, M
Marin-Ballve, A
Pla-Salas, X
Rios-Blanco, JJ
Fonollosa-Pla, V
RESCLE Investigators
Autoimmune Dis Study Grp GEAS

Grupos

Investigacion clínica en enfermedades crónicas e Infección por el VIH

Investigador establecido

Marta Montero Alonso

Abstract

Objective: Digital ulcers (DU) are the most common vascular complication of systemic sclerosis (SSc). We compared the characteristics between patients with prior or current DU with those never affected and evaluated whether a history of DU may be a predictor of vascular, organ involvement, and/or death in patients with SSc. Methods: Data from SSc patients with or without prior or current DU were collected by 19 referral centers in an ongoing registry of Spanish SSc patients, named Registro de ESCLErodermia (RESCLE). Demographics, organ involvement, autoimmunity features, nailfold capillary pattern, survival time, and causes of death were analyzed to identify DU related characteristics and survival of the entire series and according to the following cutaneous subsets diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc). Results: Out of 1326, 552 patients enrolled in the RESCLE registry had prior or current DU, 88% were women, the mean age was 50 +/- 16 years, and the mean disease duration from first SSc symptom was 7.6 +/- 9.6 years. Many significant differences were observed in the univariate analysis between patients with and without prior/current DU. Multivariate analysis identified that history of prior/current DU in patients with SSc was independently associated to younger age at SSc diagnosis, diffuse cutaneous SSc, peripheral vascular manifestations such Raynaud's phenomenon, telangiectasia, and acro-osteolysis but no other vascular features such as pulmonary arterial hypertension or scleroderma renal crisis. DU was also associated to calcinosis cutis, interstitial lung disease, as well as worse survival. Multivariate analysis performed in the cutaneous subsets showed that prior/current DU were independently associated: (1) in dcSSc, to younger age at SSc diagnosis, presence of telangiectasia and calcinosis and rarely a non-SSc pattern on nailfold capillaroscopy; (2) in lcSSc, to younger age at SSc diagnosis, presence of Raynaud's phenomenon as well as calcinosis cutis, interstitial lung disease, and higher incidence of death from all causes; and (3) in ssSSc, to younger age at first SSc symptom and greater incidence of death from all causes. Conclusions: Digital ulcers develop in patients with SSc younger at diagnosis, mainly in patients with dcSSc and IcSSc, and they are associated to other peripheral vascular manifestations such as Raynaud's phenomenon, telangiectasia, and acro-osteolysis but also to calcinosis, and interstitial lung disease. History of DU in SSc leads to worse survival, also noticeable for lcSSc and ssSSc subsets but not for dcSSc patients. (C) 2016 Elsevier Inc. All rights reserved.

Datos de la publicación

ISSN/ISSNe:: 0049-0172, 1532-866X
Tipo:: Review
Páginas:: 200-208
DOI:: 10.1016/j.semarthrit.2016.04.007
PubMed:: 27312381
Factor de Impacto:: 1,983 SCImago ℠
Cuartil:: Q1 SCImago ℠

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Keywords

Systemic sclerosis; Limited cutaneous SSc; Diffuse cutaneous SSc; SSc sine scleroderma; Digital ulcers; Anti-centromere antibodies; Anti-topoisomerase I antibodies; Nailfold capillaroscopy; Survival