Alina-Mihaela Soare, Simona Pintilie, Ana Maria Gherghe, Alexandra Radu, Rucsandra Dobrota, Mariana Sasu, Mihai Bojinca, Victor Stoica and Carina Mihai

ABSTRACT

Background and objectives. Systemic sclerosis (SSc) is a multisystem connective tissue disease (CTD), being one of the most heterogeneous diseases of the spectrum of CTDs. It may associate other autoimmune diseases (AIDs), therefore in this study we aimed to evaluate the prevalence of other AIDs in a cohort of patients with SSc and to evaluate their prognosis in comparison to patients with SSc without this association.

Patients and methods. We performed a retrospective study in patients with SSc satisfying the ACR 1980 or the ACR/EULAR 2013 criteria, who were evaluated between January 2005 and May 2014 in our SSc center. These patients were investigated according to the EUSTAR recommendations, including modified Rodnan Skin Score (mRSS), lung function tests (LFT), echocardiography etc. As a control group for evaluating the prevalence of the AIDs we used all patients with Rheumatoid Arthritis (RA) visiting our clinic between January-December 2005. Data of all RA patients were collected from the electronic database of the hospital. All AIDs mentioned in the discharge diagnoses were recorded. Between-group comparisons were made with the chi-square test for nominal variables and with the independent-sample t-test for numeric variables.

Results. 144 patients with SSc were included: 88.8% females, 66.6% with the limited cutaneous subset of disease (lcSSc), mean age 53.9 ± 12.8 years, mean disease duration at study enrollment 5.1±10.5 years and a mean follow-up time of 3.7 ± 2.6 years. Prevalence of the AID in the SSc cohort was 19.4%, patients with lcSSc being more prone to associate other AID (p = 0.001). We noted a tendency towards less interstitial lung disease (p = 0.056) and less digital ulcers (p = 0.081) in patients with SSc and AIDs. Comparing skin involvement for each year of follow-up in patients with SSc with and without and AIDs we observed that the first tended to have lower and more stable values of the mRSS.

Conclusion. Patients with SSc and AIDs tend to have a better outcome than the ones without this association. mRSS remains stable across the years in patients with SSc and AID, with lower values than the SSc patients.

Keywords: systemic sclerosis, autoimmune disease, polyautoimmunity

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Marilena Gorga, Alina-Mihaela Soare, Ana-Maria Gherghe, Rucsandra Dobrota, Cristina Mambet and Carina Mihai

ABSTRACT

Background. Wnt signaling is involved in fibrosis, but the mechanisms of cross-talk with other pathways, like TGF-β, are not fully understood. (Pro)renin receptor (PRR) functions as an accessory protein to a V-ATP-ase responsible for acidic pH maintenance in intracellular compartments, and recent data indicate that PRR could be a regulator of Wnt signaling. A circulating fragment (sPRR) is generated by enzymatic cleavage of the extracellular domain and can be quantified in plasma, serum and urine.

Objectives. This is a pilot study, to explore the serum concentrations of the soluble (pro)renin receptor (sPRR) in patients with systemic sclerosis (SSc).

Patients and methods. Serum samples from 29 subjects with a confirmed diagnosis of SSc have been tested using an ELISA method. Clinical and laboratory parameters have been analysed for associations with sPRR serum levels.

Results and conclusion. Serum levels of sPRR were higher in patients with a history of digital ulcers (p 0.041, Mann-Whitney U-test) and those with digital pitting scars (p 0.037, Mann-Whitney U-test). Serum levels of sPRR correlated with serum creatinine (r 0.424, Spearman test), in line with previously reported data. Our results indicate the need for a larger study to assess the sPRR changes in patients with SSc, including more cases with severe disease and excluding a possible impact of angiotensin-receptor blockers.

Keywords: systemic sclerosis, scleroderma, soluble (pro)renin receptor, renin-angiotensin system

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