Şükran Erten

Low plasma vitamin D levels in patients with familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an autosomal recessive, inherited autoinflammatory disease characterized by recurrent, self-limited attacks of fever and inflammation of serosal surfaces. There is an explosion of the data regarding inflammatory markers in FMF and clinical effects of chronic inflammation on the disease presentation. Vitamin D (vit D) is the common denomination of a group of sterols with a crucial role in phospho-calcium metabolism. There are some data about the importance of vit D in the initiation and propogation of a range of autoimmune diseases. The aim of the present study was to determine whether vit D deficiency is present in patients with FMF compared with healthy individuals. The study group included 99 patients with diagnosis of FMF attended to our outpatient Rheumatology and Nephrology Clinics of Atatürk Education and Research Hospital. The control group comprised 51 age- and sex-matched healthy people selected from hospital staff. Serum baseline 25-hydroxy vit D levels were measured by HPLC method using an Agilent 1100 Liquid Chromatograph. We found significantly lower serum 25-hydroxy vit D levels among FMF patients compared with matched controls and a high prevalence of vit D deficiency. This study demonstrated that vit D deficiency is frequent in patients with FMF than the healthy controls. It is convenient to look for vit D deficiency and to correct vit D nutritional status in FMF patients.

Thinning of Choroidal Thickness in Patients with Rheumatoid Arthritis Unrelated to Disease Activity

Abstract Purpose: To investigate subfoveal and perifoveal choroidal thickness (CT) in patients with rheumatoid arthritis (RA). Methods: A study group of 117 patients with RA and a control group of 46 age-matched healthy individuals were enrolled in the study. Subfoveal and perifoveal CTs were measured using enhanced depth imaging optical coherence tomography (EDI-OCT). Perifoveal CT was measured 1,500 µm nasally and 1,500 µm temporally apart from the foveal center. Relationship between the disease activity score 28 (DAS-28) and subfoveal CT was also evaluated. Results: The mean subfoveal CT values in the study and control groups were 226.90 ± 43.61 μm and 299.74 ± 48.06 μm, respectively, which made for a statistically significant difference (p < 0.001). Likewise, perifoveal CT values were significantly thinner in the study group when compared with control group. DAS-28 wasn’t correlated with subfoveal CT in the study group. Conclusions: CT was significantly thinner in patients with RA when compared with healthy controls.

Choroidal Thickness in Patients with Systemic Lupus Erythematosus Analyzed by Spectral-domain Optical Coherence Tomography

Abstract Purpose: The aim of this study is to investigate the effect of systemic lupus erythematosus (SLE) on choroidal thickness (CT) in the subfoveal and perifoveal area as measured by enhanced depth imaging optical coherence tomography (EDI-OCT) in patients with SLE. Materials and methods: Fifty-eight patients with SLE (study group) and 58 healthy individuals (control group) were enrolled in the study. The subfoveal and perifoveal CT were measured by EDI-OCT. Results: The mean CT (μm) subfoveal, nasal, and temporal was 231.2 ± 57.6, 190.56 ± 30.64, and 222.65 ± 37.45 in the study group and 297.5 ± 45.1, 248.34 ± 39.67, and 286.54 ± 49.65 in the control group, respectively (p < 0.001, for all). Conclusion: The mean subfoveal, nasal, and temporal CT values were statistically significantly lower in patients with SLE when compared with healthy controls.

Phenotype 2 familial mediterranean fever: evaluation of 22 case series and review of the literature on phenotype 2 FMF.

Familial Mediterranean fever (FMF) is an autosomal recessive autoimmune disorder characterized by recurrent bouts of fever and serosal inflammation. FMF may be complicated by AA-type amyloidosis, worsening the prognosis, with associated renal failure in some patients. Complication rate varies with race, being as high as 60% in Turks and as low as 2% in Armenians. In a few cases of patients with FMF (phenotype 2), amyloid nephropathy may be the presenting manifestation. This study included 420 patients who were admitted to the Nephrology and Rheumatology Departments of Atatürk Education and Research Hospital with unexplained proteinuria/nephrotic syndrome. The initial screening test for amyloidosis was the presence of significant proteinuria (300 mg/24 h). All MEFV gene exons were screened for causative mutations by direct DNA sequencing to check for any mutations. There were 22 phenotype 2 FMF patients with 27 allelic variants. The most prevalent allelic variants were M694V (10/27, 37%) and E148Q (7/27, 26%). Phenotype 2 FMF is not as rare as it was thought before; this should be kept in mind for all patients with unexplained proteinuria and/or acute phase response in high-risk ethnic groups for FMF.

Assessment of the relationship between aortic stiffness and left ventricular functions with echocardiography in patients with Sjögren's syndrome.

Capable of multi‐organ involvement in Sjogrens syndrome (SS), cardiac findings of pulmonary effusion, left ventricular diastolic dysfunction and pulmonary hypertension are seen in patients with SS. Aortic stiffness (AS) reflects the mechanical tension and elasticity of the aorta. In this study, our aim is to determine if there is any differences in AS and left ventricular function between patients diagnosed as SS and healthy control groups.

The Prevalence of Dry Eye and Sjögren Syndrome in Patients with Migraine.

ABSTRACT Purpose: To evaluate the presence of dry eye and primary Sjögren syndrome (SS) in patients with migraine. Methods: In total, 46 eyes of 46 patients with migraine (group 1) and 50 eyes of 50 healthy subjects (group 2) were included in this study. Detailed ophthalmologic, neurologic and rheumatologic examination were performed on all participants. Ocular surface disease index questionnaire, tear function tests, visual analog scale for pain, serologic analysis were also performed. Results: Dry eye symptoms and findings were significantly higher and more severe in group 1 when compared with group 2. Primary SS was not found in any of the participants. The migraine lifetime duration was negatively correlated with the tear function tests while it was positively correlated with the ocular surface disease index scores. Conclusions: Dry eye symptoms and findings are higher in migraine patients when compared with the healthy subjects without the presence of Sjögren syndrome.

Concomitance of Gitelman syndrome and familial Mediterranean fever: a rare case presentation.

We report a case that has Gitelman syndrome (GS) and familial Mediterranean fever (FMF) presenting with recurrent arthritis of right knee and heel pain. Investigations showed hypokalemia and hypomagnesemia with urinary magnesium wasting. Genetic analysis revealed the presence of heterozygous E148Q mutation in the MEFV gene. Management with potassium, magnesium supplements, spironolactone for GS, and colchicine for FMF resulted in a significant improvement in symptoms. To the best of our knowledge, this is the first report of association between GS and FMF. Further studies are needed to identify if there is an association between these two diseases and the genes responsible for these diseases.

Assessment of local carotid stiffness in seronegative and seropositive rheumatoid arthritis

Abstract Objectives: Rheumatoid arthritis (RA) is a chronic, inflammatory disease associated with increased risk of cardiovascular (CV) disease. Arterial stiffness (AS) is an independent predictor of CV events. This study aimed to analyse local carotid AS parameters in seronegative and seropositive RA patients. Design: Of 347 consecutive RA patients, we selected specifically those who were free of established CV diseases and risk factors. As a result, 140 patients (126 women, 52.2 ± 10 years) and 140 healthy controls (122 women, 52.7 ± 8.0 years) were enrolled into this study. The common carotid AS was evaluated using radio frequency echo-tracking system to determine the local carotid pulse wave velocity (cPWV) and carotid intima-media thickness (cIMT). Based on rheumatoid factor (RF) and/or anti-citrullinated protein antibody (ACPA) positivity, RA patients were categorized into seronegative and seropositive subgroups. Results: Carotid PWV was determined to be significantly higher in all patients and subgroups than controls (p < .001 for all). Although cIMT was similar between the patients, controls and seropositive subgroup, seronegative patients had significantly higher cIMT compared to controls (p = .035) and seropositive group (p = .010). Moreover, a significant positive correlation was found between cPWV and age (r: 0.603, p < .001), ESR (r: 0.297, p = .004), ACPA (r: 0.346, p = .001) and cIMT (r: 0.290, p = .005) in seropositive patients. Conclusions: RA per se is sufficient to cause arteriosclerosis in the absence of classical CV risk factors. However, arterial hypertrophy is only increased in seronegative patients but not in seropositive group.

Serum cystatin C is not an appropriate marker for kidney involvement in patients with primary Sjögren's syndrome

We aimed to investigate serum cystatin C (cysC) levels in primary Sjögrens syndrome (pSS) patients, and evaluate its correlation with renal involment.

Incidence of sleep disturbances in patients with familial Mediterranean fever and the relation of sleep quality with disease activity

To evaluate the sleep quality and the relation of sleep quality with depression, anxiety, fatigue and disease activity in adult patients with familial Mediterranean fever (FMF).