Clinical features of systemic sclerosis patients with anti-RNA polymerase III antibody in a single centre in Spain.

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.

• Authors: Eduardo Luis Callejas-Moraga, Alfredo Guillén-Del-Castillo, Ana María Marín-Sánchez, María Roca-Herrera, Eva Balada, Carles Tolosa-Vilella, Vicente Fonollosa-Pla, Carmen Pilar Simeón-Aznar
• Journal: Clinical and experimental rheumatology (Clin Exp Rheumatol) 2019 Jul-Aug Vol. 37 Suppl 119 Issue 4 Pg. 41-48 ISSN: 0392-856X [Print] Italy
• PMID: 30767873 (Publication Type: Journal Article)

Chemical References

• Autoantibodies
• RNA Polymerase III

Topics

• Adult
• Autoantibodies (blood, immunology)
• Delayed Diagnosis
• Female
• Humans
• Lung Diseases, Interstitial (diagnosis, immunology, metabolism)
• Male
• Middle Aged
• RNA Polymerase III (immunology)
• Scleroderma, Systemic (diagnosis, immunology, metabolism)
• Spain