Clinical aspects
Clinical features of systemic sclerosis patients with anti-RNA polymerase III antibody in a single centre in Spain
E.L. Callejas-Moraga1, A. Guillén-Del-Castillo2, A.M. Marín-Sánchez3, M. Roca-Herrera4, E. Balada5, C. Tolosa-Vilella6, V. Fonollosa-Pla7, C.P. Simeón-Aznar8
- Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Parc Taulí, Universitat Autònoma de Barcelona, Sabadell, Barcelona, Spain.
- Unit of Systemic Autoimmune Diseases, Institut de Recerca Vall d’Hebron, Hospital Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Spain. alguille@vhebron.net
- Department of Inmunology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.
- Unit of Systemic Autoimmune Diseases, Institut de Recerca Vall d’Hebron, Hospital Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Spain.
- Unit of Systemic Autoimmune Diseases, Institut de Recerca Vall d’Hebron, Hospital Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Spain.
- Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Parc Taulí, Universitat Autònoma de Barcelona, Sabadell, Barcelona, Spain.
- Unit of Systemic Autoimmune Diseases, Institut de Recerca Vall d’Hebron, Hospital Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Spain.
- Unit of Systemic Autoimmune Diseases, Institut de Recerca Vall d’Hebron, Hospital Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Spain.
CER11630
2019 Vol.37, N°4 ,Suppl.119
PI 0041, PF 0048
Clinical aspects
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PMID: 30767873 [PubMed]
Received: 19/08/2018
Accepted : 30/10/2018
In Press: 11/02/2019
Published: 03/10/2019
Abstract
OBJECTIVES:
To evaluate the clinical features and survival of patients with positive anti-RNA polymerase III (anti-RNAP III) in a Spanish single centre.
METHODS:
We analysed 221 patients with SSc according to LeRoy and Medsger criteria. Twenty-six patients with positivity for anti-RNAP III antibodies were compared with 195 negative patients. Epidemiological, clinical, immunological features and survival were analysed.
RESULTS:
In patients with anti-RNAP III positivity diffuse cutaneous SSc (dcSSc) subset was the most prevalent (20, 76.9% vs. 35, 17.9%, p < 0.001), with shorter diagnosis delay (4.11 ± 7.34 years vs. 6.77 ± 9.22 years, p = 0.005). Patients with anti-RNAP III antibodies had higher frequency of arterial hypertension (13, 50% vs. 55, 28.2%, p = 0.024), scleroderma renal crisis (SRC) (3, 11.5% vs. 3, 1.5%, p = 0.023), arthritis (9, 34.6% vs. 35, 17.9%, p = 0.046), tendon friction rubs (4, 15.4% vs. 1, 0.5%, p = 0.001) and contractures (5, 19.2% vs. 10, 5.1%, p = 0.02). There were no differences found in the presence of cancer or in global survival. In the multivariate survival analysis, severe interstitial lung disease (ILD) (HR: 8.61, 95%CI 3.40 – 21.81), pulmonary arterial hypertension (PAH) (HR: 4.05, 95%CI 1.42 – 11.61) and SRC (HR: 17.27, 95%CI 3.36 – 88.97) were the only factors associated with poor prognosis.
CONCLUSIONS:
In this cohort anti-RNAP III antibodies are related with dcSSc subset, shorter diagnostic delay and higher prevalence of musculoskeletal involvement, arterial hypertension and SRC. ILD, PAH and SRC were independent prognostic factors.