Methodology of a new inflammatory arthritis registry: TReasure

Background/aim: The TReasure registry, created in 2017, is an observational multicenter cohort that includes inflammatory arthritis patients. This article reviews the methodology and objectives of the TReasure registry established to collect data from rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients. Methodology: Fifteen rheumatology centers in Turkey will contribute data to the TReasure database. The actual proprietor of the database is the Hacettepe Rheumatology Association (HRD) and Hacettepe Financial Enterprises. Pharmaceutical companies that operate in Turkey (in alphabetical or er), Abbvie, Amgen, BMS, Celltrion Healthcare, Novartis, Pfizer, Roche, and UCB, support the TReasure registry. TReasure is a web-based database to which users connect through a URL (https://www.trials-network.org/treasure) with their unique identifier and passwords provided for data entry and access. TReasure records demographic and clinical features, comorbidities, radiology and laboratory results, measures of disease activity, and treatment data. Discussion: TReasure will provide us with various types of data, such as a cross-sectional view of the current nationwide status of the patients currently receiving these treatments, and retrospective data as much as allowed by the participating centers' records. Finally, a high-quality prospective dataset will be built over the ensuing years from patients with a new diagnosis of RA or SpA.

Amyloidosis and its related factors in Turkish patients with familial Mediterranean fever: a multicentre study.

OBJECTIVE: The primary aim of this study was to investigate the prevalence of amyloidosis and its related factors in a large number of FMF patients. METHODS: Fifteen centres from the different geographical regions of Turkey were included in the study. Detailed demographic and medical data based on a structured questionnaire and medical records were collected. The diagnosis of amyloidosis was based on histological proof of congophilic fibrillar deposits in tissue biopsy specimens. RESULTS: There were 2246 FMF patients. The male/female ratio was 0.87 (1049/1197). The mean age of the patients was 34.5 years (S.D. 11.9). Peritonitis was the most frequent clinical finding and it was present in 94.6% of patients. Genetic testing was available in 1719 patients (76.5%). The most frequently observed genotype was homozygous M694V mutation, which was present in 413 (24%) patients. Amyloidosis was present in 193 patients (8.6%). Male sex, arthritis, delay in diagnosis, M694V genotype, patients with end-stage renal disease (ESRD) and family history of amyloidosis and ESRD were significantly more prevalent in patients with amyloidosis compared with the amyloidosis-negative subjects. Patients with homozygous M694V mutations had a 6-fold higher risk of amyloidosis compared with the other genotypes (95% CI 4.29, 8.7, P < 0.001). CONCLUSION: In this nationwide study we found that 8.6% of our FMF patients had amyloidosis and homozygosity for M694V was the most common mutation in these patients. The latter finding confirms the association of homozygous M694V mutation with amyloidosis in Turkish FMF patients.

Psoriasis Symptom Inventory (PSI) as a patient-reported outcome in mild psoriasis: Real life data from a large psoriatic arthritis registry.

OBJECTIVE: Our aim is to test the validity of the Psoriasis Symptom Inventory (PSI), a patient-reported outcome, to assess the psoriasis severity within the scope of rheumatology. METHODS: Within the PsA international database (PSART-ID), 571 patients had PSI, while 322 of these also showed body surface area (BSA). Correlations between PSI, BSA, and other patient- and physician-reported outcomes were investigated. RESULTS: There was a good correlation between PSI and BSA (r=0.546, p<0.001), which was even higher for mild psoriasis (BSA<3 (n=164): r=0.608, p<0.001). PSI significantly correlated with fatigue, pain, and patient and physician global parameters (p<0.001). CONCLUSION: PSI has a good correlation with other patient- and physician-reported outcomes, and our findings support its use in rheumatology practice.

Exon 2: Is it the good police in familial mediterranean fever?

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF. METHODS: Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database. RESULTS: Patients having the MEFV mutations on exon 2 or 10 (n:1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group. CONCLUSION: Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome.

Serum P-selectin, soluble vascular cell adhesion molecule-I (s-VCAM-I) and soluble intercellular adhesion molecule-I (s-ICAM-I) levels in bladder carcinoma patients with different stages.

Adhesion molecules are thought to have a role in the host defense against carcinogenesis. In this study, we measured serum platelet-(P-) selectin, soluble vascular cell adhesion molecule-I (s-VCAM-I), and soluble intercellular adhesion molecule-I (s-ICAM-I) levels in 51 sequential bladder cancer patients at our urology clinic and 8 controls. Serum levels of P-selectin, s-VCAM-I and s-ICAM-I were significantly higher in all patients than controls. Serum P-selectin and s-ICAM-I levels did not differ based on tumor (T) stage and tumor grade, whereas serum levels of s-VCAM-I were significantly higher in patients with muscle invasive tumors than those with superficial tumors. Further, s-VCAM-I levels correlated with T stage. In conclusion, significantly increased P-selectin, s-VCAM-I, and s-ICAM-I levels were observed in patients with bladder cancer, and s-VCAM-I levels correlated with T stage. Thus, we suggest that the analysis of serum s-VCAM-I levels may provide a prognostic test for T stage and bladder cancer progression during and/or following therapy.

Spondylodiscitis and endocarditis caused by S. vestibularis.

Streptococcus vestibularis is a recently described member of the viridans group that was first isolated from the vestibular mucosa of the human oral cavity and described as a new species in 1988. It has been rarely associated with human infections. In few papers, it has been reported as a causal agent of systemic infection in immunosupressed adults and in those with other severe underlying diseases, like coronary valve diseases. A 65-year-old woman was admitted to the hospital with complaints of fever for three months, general malaise, effort dyspnea, weight loss, back pain and myalgia. Both native aortic valve endocarditis and spondylodiscitis due to Streptococcus vestibularis were detected. The patient was successfully treated with intravenous potassium penicillin G and gentamicin for six weeks, followed by oral amoxicillin for three months, in addition to aortic valve replacement. In all patients with spondylodiscitis, infective endocarditis should be considered, particularly in patients with heart valve disease history, since spondylodiscitis may be the presenting sign of an infective endocarditis. Cardiac valve replacement surgery should be performed if the course of fever and inflammatory syndrome is unfavorable after appropriate antibiotic treatment. We report the first case with both native aortic valve endocarditis and spondylodiscitis due to Streptococcus vestibularis.

Spondylodiscitis and endocarditis caused by S. vestibularis

Streptococcus vestibularis is a recently described member of the viridans group that was first isolated from the vestibular mucosa of the human oral cavity and described as a new species in 1988. It has been rarely associated with human infections. In few papers, it has been reported as a causal agent of systemic infection in immunosupressed adults and in those with other severe underlying diseases, like coronary valve diseases. A 65-year-old woman was admitted to the hospital with complaints of fever for three months, general malaise, effort dyspnea, weight loss, back pain and myalgia. Both native aortic valve endocarditis and spondylodiscitis due to Streptococcus vestibularis were detected. The patient was successfully treated with intravenous potassium penicillin G and gentamicin for six weeks, followed by oral amoxicillin for three months, in addition to aortic valve replacement. In all patients with spondylodiscitis, infective endocarditis should be considered, particularly in patients with heart valve disease history, since spondylodiscitis may be the presenting sign of an infective endocarditis. Cardiac valve replacement surgery should be performed if the course of fever and inflammatory syndrome is unfavorable after appropriate antibiotic treatment. We report the first case with both native aortic valve endocarditis and spondylodiscitis due to Streptococcus vestibularis.