Long-term results of kidney transplantation in patients with familial mediterranean fever.

INTRODUCTION: Long-term kidney transplantation (KT) results in patients with familial Mediterranean fever (FMF)-related amyloidosis are not well studied. This study reviewed the long-term survival outcomes of FMF patients who underwent KT. METHODS: We compared the outcomes of 31 patients who underwent (KT) for biopsy-proven amyloidosis secondary to FMF with 31 control patients (five with diabetes mellitus and 26 with nondiabetic kidney disease) undergoing KT between 1994 and 2021 at Baskent University Hospital. All data were recorded retrospectively from patients' files. RESULTS: THE MEDIAN AGE (QUARTILE DEVIATION: QD) at the time of KT in the FMF and control group were 31 (6.7) and 33 (11), respectively. The median follow-up period (QD) after KT was 108 (57) months in the FMF and 132 (72) months in the control group. In the FMF group, graft and patient survivals were 71% and 84% at 5 years and 45% and 48% at 10 years, respectively. In the control group, graft and patient survivals were 79% and 100% at 5 years and 63% and 71% at 10 years, respectively. Patient survival in the FMF group at 5 years was significantly lower than in the control group (p = .045). There was no statistically significant difference between the FMF and control groups in terms of graft and patient survival, and serum creatinine levels at 10 years. All patients were given triple immunosuppressive treatment with cyclosporine, mycophenolate mofetil, and prednisolone. Three patients received anakinra and one received canakinumab in addition to colchicine treatment. One FMF patient also underwent heart transplantation due to AA amyloidosis. Of the FMF patients, 11 died during follow-up. CONCLUSION: We have found that the long-term outcome of KT in patients with FMF amyloidosis is numerically worse but not statistically different from the control group. However, short- and long-term complications still need to be resolved.

Factors affecting relapse in patients with Granulomatosis Polyangiitis: a single-center retrospective cohort study

Background and aim: This study aimed to determine the frequency of relapse, the risk factors for relapse, and the correlation of relapse with immunosuppressive regimens in patients with granulomatosis polyangiitis (GPA). Materials and methods: The demographic characteristics, the clinical, laboratory, and radiological findings, the immunosuppressive treatment regimens, and the remission and relapse rates of 50 patients with GPA were obtained retrospectively from medical records. Results: The mean relapse-free survival rates at years 1, 3, and 5 were 82%, 60%, and 50%, respectively. Increased relapse rates were observed in patients who had cavitary lung lesions (52.2% vs. 22.2%, p = 0.04) and in those who had elevated serum creatinine levels (1.8 vs. 0.9, p = 0.00). The patients received two different types of remission induction therapies; 36% of them received the combination therapy involving cyclophosphamide (CYC) and rituximab (RTX), and 62% received CYC alone. Relapse was observed in 22.3% of the patients who received the combination remission induction therapy and in 61.3% of the patients who received CYC alone (P = 0.003). Conclusion: An increased risk of relapse was observed in patients who had cavitary lung lesions and in those who had elevated serum creatinine levels. The combined use of RTX and CYC for the remission therapy in GPA reduced the relapse rates compared with the use of CYC alone.

Correlation between IL-17A/F, IL-23, IL-35 and IL-12/-23 (p40) levels in peripheral blood lymphocyte cultures and disease activity in Behcet's patients.

Behcet's disease is a chronic multisystemic disease with remissions and relapses. Several studies have shown that immune mechanisms play an important role in the development of the disease. In order to assess the association of disease activity with IL-17A/F, IL-23, IL-12/23 (p40) and IL-35 expression, we aimed to investigate production of these cytokines in peripheral blood mononuclear cells (PBMCs) from Behcet's patients and normal controls. Furthermore, we included Systemic Lupus Erythematosus (SLE) as disease control to evaluate the specificity of our data for immunopathogenesis of BD. Totally 15 active, 15 inactive Behcet's patients, 12 active and 12 inactive SLE patients and 12 healthy volunteers were enrolled in the study. Peripheral blood mononuclear cells were separated, lymphocyte cultures were performed and IL-17A/F, IL-12/23 p(40), IL-23, IL-35 cytokine levels were measured by ELISA in culture supernatants in the presence or absence of phytohemagglutinin (PHA) on time-dependent manner. IL-17 A/F levels increased parallel to IL-23 levels in Behcet's and SLE patients. Compared to healthy controls, IL-17 A/F levels were higher in active Behcet's and SLE patients; on the contrary, levels of IL-35 were lower. IL-17A/F, IL-12/23 (p40) and IL-23 levels were detectable most frequently in active Behcet's patients followed by active SLE patients. Our results indicate that IL-17 A/F, IL-23 and IL-12/23 (p40) may play role in the immunopathogenesis of BD so as Th17 and Th1 cell responses. Since IL-35 levels were lower in active Behcet's patients compared to inactive patients and healthy controls, there may be a plasticity between Th17 and Treg cells according to the state of disease activity.

Corneal, Scleral, Choroidal, and Foveal Thickness in Patients with Rheumatoid Arthritis.

OBJECTIVES: To investigate corneal, scleral, choroidal, and foveal thicknesses in female patients with rheumatoid arthritis (RA) and compare them with healthy subjects. MATERIALS AND METHODS: This prospective study included consecutive female patients diagnosed with RA and healthy subjects. Corneal, scleral, choroidal, and retinal (foveal) thicknesses were obtained by using optical coherence tomography and a comparison was performed between groups for all outcome measures. RESULTS: Thirty-six eyes of 36 female patients diagnosed with RA (group 1) and 36 eyes of 36 healthy female volunteers (group 2) were included. Mean corneal, scleral, choroidal thicknesses and retinal thickness at the fovea of group 1 were 543.3±33.7 µm, 343.7±42.2 µm, 214.6±50, and 213.5±18.9 µm, respectively; in group 2, these values were 549.9±29.6 µm, 420.9±42.4 µm, 206.4±41.9 µm, and 222±15.5 µm, respectively. The comparison between group 1 and 2 with respect to corneal, choroidal, and foveal thicknesses did not reveal statistical significant differences (p>0.05). On the contrary, there was a statistically significant difference with respect to scleral thickness between the groups, with the RA patients demonstrating a thinner scleral layer (p<0.001). CONCLUSION: Female patients with RA seem to demonstrate statistically significant scleral thinning when compared with healthy subjects, while there was no difference concerning corneal, choroidal, and foveal thickness.

The prevalence of spondyloarthropathy in fibromyalgia patients.

OBJECTIVE: To document the prevalence of Spondyloarthropathy (SpA) with an evaluation of patients previously diagnosed with fibromyalgia syndrome (FMS). METHODS: The patients diagnosed with FMS before telephoned and asked three questions to determine for inflammatory back pain. American College of Rheumatology (ACR) Fibromyalgia criteria 1990 and ACR 2010 and for diagnosing patients with SpA; criteria from the European Spondyloarthropathy Study Group (ESSG), and Amor were applied. RESULTS: FMS was diagnosed according to 1990 ACR criteria in 14 (60.8%) SpA patients who were diagnosed with SpA according to the Amor criteria alone and in 10 (43.4%) patients who were diagnosed according to ESSG criteria alone, while it was diagnosed in 9 (33.3%) patients who were diagnosed with SpA according to Amor and ESSG criteria together and in 15 (65.2%) patients diagnosed with SpA according to Amor and/or ESSG criteria. The most tenderness was experienced in the bilateral shoulder supraspinatus tendon insertion region (57.7%) and in the sacroiliac joint (40.8%). Fourteen (60.8%) patients diagnosed according to ACR 1990 and 17 (51.6%) patients diagnosed according to ACR 2010 had plantar fasciitis and/or Achilles enthesopathy on foot radiography. CONCLUSIONS: There is a meaningful section of patients who are SpA or FMS and SpA are together in the patients thought to be FMS or the patients diagnosed with FMS according to ACR's criteria can be said.

High Levels of Circulating Endothelial Progenitor Cells Are Associated with Acrotism in Patients with Takayasu Arteritis.

OBJECTIVES: To investigate the association between endothelial progenitor cells (EPCs) and Takayasu arteritis (TA). Subjects andMethods: A total of 39 subjects were included in this study: 12 subjects had been diagnosed with active TA, 11 had active Behçet disease (BD), and 16 were healthy controls. The EPCs, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) levels of all the subjects were measured. MedCalc 15.8 software (MedCalc, Belgium) was used for all statistical analyses. RESULTS: The level of EPCs was higher in TA patients (4.25 ± 2.56) than in the BD group (2.27 ± 2.0) and the healthy controls (2.12 ± 1.2) (p = 0.015). TA patients with acrotism (n = 4) had higher levels of EPCs compared to TA patients without acrotism (n = 8) (6.50 ± 1.73 vs. 3.12 ± 2.16, p = 0.02). A positive correlation was found between EPCs and the ESR (r = 0.723, p = 0.0079) and between EPCs and CRP in patients with TA (r = 0.769, p < 0.0034). CONCLUSION: High levels of circulating EPCs were correlated with the CRP level and the ESR in patients with TA. These cells could be a marker for acrotism and inflammation in patients with TA.

High levels of endothelial progenitor cells can be associated with thrombosis in patients with Behçet's disease.

OBJECTIVES: Behçet's disease (BD) is a systemic disorder characterised by vasculitis. Endothelial progenitor cells are derived from the bone marrow and contribute to new vessel formation. The aim of this study was to investigate the level of endothelial progenitor cells in BD and BD-associated conditions. METHODS: A total of 74 subjects were included in this study, of whom 44 and 30 subjects were patients with BD or healthy subjects, respectively. Endothelial progenitor cells were defined and measured by flow cytometry according to the expression of CD146, CD31 and CD34. We separated BD patients according to the active disease, pathergy test results, thrombosis and gender. MedCalc 12.5 software programme was used for statistical analyses. RESULTS: The level of endothelial progenitor cells was comparable in patients with BD and healthy subjects (p=0.849). It was also comparable in patients with active or inactive BD (p=0.320). The level of endothelial progenitor cells was higher in patients with thrombosis (p=0.04). There was no statistical significant difference between pathergy positive and negative patients (p=0.969). The level of endothelial progenitor cells was not correlated with age, C-reactive protein, erythrocyte sedimentation rate, white blood cells and disease duration (p>0.05). CONCLUSIONS: The level of endothelial progenitor cells was significantly higher in BD patients with thrombosis. On the other hand, they were not associated with disease activity, pathergy test and other conditions. EPCs may be a useful marker for thrombosis in patients with BD. In our opinion, this is the most expected result in this study.

Effects of infliximab treatment in terms of cardiovascular risk and insulin resistance in ankylosing spondylitis patients.

OBJECTIVE: To assess the effects of infliximab treatment on insulin sensitivity and cardiovascular risk factors in patients with ankylosing spondylitis (AS). METHODS: In this prospective study, 30 consecutive AS patients (23 men and 7 women) fulfilling the modified 1984 New York criteria for AS were investigated. All patients were treated with intravenous infliximab. A complete biochemical profile and assesments were obtained before and after 12 weeks of infliximab therapy. The Homoeostasis Model Assessment of Insulin Resistance Index (HOMA-IR) was used to measure insulin resistance (IR). Framingham equation was used to assess cardiovascular risk factors. RESULTS: After 12 weeks of infliximab treatment, there was no statistically significant difference in fasting insulin, HOMA-IR, lipid parameters, body-mass index, waist circumference and waist-hip ratio, whereas fasting glucose levels (p = 0.001), triglycerides/high-density lipoprotein (HDL) ratio (p = 0.043) and total cholesterol/HDL (p = 0.041) ratio increased significantly from baseline. A significant decrease was observed for both systolic blood pressures (p < 0.001) and diastolic blood pressures (p = 0.003) in the 12th-week visit. A significant decrease was also found in terms of Framingham risk scores (p = 0.028) after treatment. CONCLUSIONS: Study results suggest that infliximab treatment may reduce cardiovascular risk and blood pressures without changing IR.

If Neurologists Establish The Diagnosis of Primary Sjogren's Syndrome?

INTRODUCTION: Neurological involvements were shown in 20% of patients with Primary Sjogren's Syndrome (pSS). Neurological symptoms may be the first signs of pSS in 57% of the cases. In addition, early diagnosis and treatment of neurological disorders may save or improve the quality of life of these cases. There have been reports about the neurologic manifestations of pSS but little is known about the details of neurologically presented cases. METHOD: In this study, we described 11 pSS patients who presented with neurological manifestations. RESULTS: Central nervous system (CNS) involvement was recorded in 7 (63.7%) and peripheric nervous system (PNS) involvement in 4 cases (36.4%). CONCLUSION: Our findings regarding the cases with neurological manifestations leading to the diagnosis of pSS suggest that: 1) The frequency of CNS involvement was higher than that of PNS, and the most frequent clinical pictures of CNS involvement are Multiple Sclerosis (MS)-like illnesses and optic neuritis, 2) Guillain Barre Syndrome (GBS) was the most frequent disease of PNS involvement; 3) Mononeuropathy multiplex (MM) might be the first sign of pSS; 4) Neurologists should consider pSS in the differential diagnosis of cases with MS, optic neuritis, GBS and neuropathies of unknown causes including MM; 5) There is an urgent need of therapeutical guidelines for the cases with neurological involvement associated with pSS.

The effect of oral and parenteral vitamin D supplementation in the elderly: a prospective, double-blinded, randomized, placebo-controlled study.

Hypovitaminosis D in the elderly causes falls and fractures as a result of impaired neuromuscular functions and also may be a reason for nonspecific musculosceletal pain. The aim of this study is to investigate the benefits of a single dose per os or parenterally administrated vitamin D on increasing the quality of life and functional mobility and decreasing the pain in the elderly. The community-dwelling elderly subjects over 65 years age were included in the study. The subjects were given 300.000 IU Vitamin D via per os and parenteral route and assessed after 4 weeks. The serum creatinine, calcium, phosphorous, ALT, ALP, 24-h urine calcium excretion, PTH, and vitamin D levels, as well as VAS (visual analog scale) for pain assessment, functional mobility with TUG (timed up and go test) and quality of life with SF-36 before and after the treatment were evaluated. The serum vitamin D levels were measured by the RIA method. The subjects were divided into four groups each consisting of 30 subjects. The 1st group took i.m. vitamin D, the 2nd group took i.m. placebo, the 3rd group took p.o. vitamin D, and the 4th group took p.o. placebo. The mean age of all the participants was 70.1 ± 4.3 years. There was no difference in the age and gender between the groups (P > 0.05). After treatment, the PTH level of first group was decreased (P = 0.0001) and the vitamin D level increased (P = 0.0001) significantly. In the third group, the PTH level of first group was decreased (P = 0.0001) and the vitamin D level increased (P = 0.004) and the 24-h calcium excretion in urine (P = 0.015) increased significantly. When the pain, the functional mobility, and the quality of life were evaluated, in the first group, the TUG (P = 0.0001) and the VAS (P = 0.0001) decreased significantly, whereas the SF-36 subtitles: physical functioning (P = 0.0001), role physical (0.006), bodily pain (P = 0.0001), general health (P = 0.007), social functioning (P = 0.05), and mental health (P = 0.048) increased significantly. In group two, the VAS (P = 0.001) decreased, the role physical (P = 0.009), and role emotional (P = 0.034) increased significantly; In group three, the TUG (P = 0.0001) and the VAS (P = 0.002) decreased, whereas the physical function (P = 0.0001) and role physical (0.001) increased significantly; In group four, the VAS (P = 0.007) decreased significantly. The megadose vitamin D administration increases quality of life, decreases pain, and improves functional mobility via po or im route in the elderly.

Fibromyalgia: its prevalence in haemodialysis patients and its relationships with clinical and laboratory parameters.

OBJECTIVE: Our aim was to determine the prevalence of fibromyalgia syndrome (FS) in chronic haemodialysis (HD) patients and to identify possible links between FS and various clinical and laboratory parameters. METHODS: We studied 122 chronic HD patients and 89 healthy age- and sex-matched controls, classified according to the American College of Rheumatology (ACR) classification criteria for FS. Age, sex, causes of renal failure, length of time on dialysis and marital status were recorded, and questions were asked about symptoms related to FS. All subjects completed the Fibromyalgia Impact Questionnaire (FIQ). Laboratory data obtained in the preceding 6 months were re-evaluated. RESULTS: Nine (7.4%) of the 122 HD patients and four of the 89 control subjects (4.5%) fulfilled the ACR criteria for definite FS (P = 0.56). The mean ages of the subjects who had definite FS and those who did not were similar. Most of the subjects diagnosed with definite FS were female (11 out of 13). The HD patients had higher FIQ scores than the controls, regardless of FS diagnosis. Among the HD patients, those with definite FS had a significantly higher mean FIQ score than all the other HD patients combined. In the all HD patients group, fatigue, irritable bowel syndrome and personal histories of depression were correlated with FS, whereas duration of HD, aetiology of renal failure, laboratory parameters and hepatitis B or C virus infection were not. CONCLUSION: The prevalence of FS appeared to be similar in chronic HD patients and the general population; also, FS-related symptoms appear to be similar in HD patients and the general population who have FS. No laboratory parameter was correlated with frequency of FS.