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Objective: Behçet's syndrome (BS) is a multisystem variable vessel vasculitis characterized by skinmucosal lesions. It can also involve the eyes, blood vessels, joints, gastrointestinal system, urogenital system, and central nervous system. BS starts in the third or fourth decade and affects both genders equally. The disease is more severe in young men. Although the sensitivity of the pathergy test (PT) is decreasing today, it is still an important clue in the diagnosis of BS. We describe the characteristics of BS in our region, retrospectively. We also analyzed the effect of gender, age, family history, and skin PT positivity status on the difference of clinical involvement. Methods A total of 777 BS patients (391 women and 386 men; 40.0 ± 11.6 years old) who applied to our Rheumatology Department between January 2010 and June 2020 were included in the study. Results: Of the 777 patients, 391 were female (50.3%) and 386 were male (49.7%). The mean age at diagnosis was 30.3 ± 9.8 years. The proportion of patients with BS in their family was 10.2%. Of the 777 patients, 310 (39.9%) had only mucocutaneous symptoms. Other 467 patients (60.1%) had at least one of the ocular, musculoskeletal, vascular, neurologic, intestinal, or genitourinary involvement. Serious involvements such as eye, cardiovascular, and neurologic involvement were more common in male patients. Conclusion: BS has the different clinical phenotypes according to gender, age of onset, and skin PT positivity status. Gender influences on the major organ involvements such as eye, neurologic, and vascular. [ABSTRACT FROM AUTHOR]

Objective: In the familial Mediterranean fever (FMF) clinic, arthritis is among the most common symptoms, and it generally responds well to colchicine treatment. However, cases of patients with chronic prolonged colchicine-resistant arthritis have been reported, and there are inadequate studies on the treatments to be used for such patients. Methods: This study included 18 patients diagnosed with FMF who had colchicine-resistant chronic arthritis and received anti-interleukin (IL)-1 treatment for at least 1 year. The clinical and laboratory data of the patients were retrospectively retrieved from the database of our hospital. Results: Remission was achieved in arthritis attacks in 16 of 18 patients who started anti-IL-1 therapy because of colchicine-resistant chronic arthritis. The clinical and laboratory values of the other 2 patients improved, but complete remission could not be achieved. The treatment dose of colchicine was reduced with anti-IL-1 therapy. In addition to the improvement in arthritis symptoms, remission was achieved in other clinical findings of FMF by anti-IL-1 therapy. In this study, with an average follow-up time of 33 months, no adverse effects requiring discontinuation were observed in any patient. Conclusion: Anti-IL-1 therapy is effective and reliable in the treatment of colchicine-resistant chronic FMF arthritis. The efficacy of anti-IL-1 therapy was realized without concomitant disease-modifying antirheumatic drug therapy, despite the reduction in colchicine dose. [ABSTRACT FROM AUTHOR]

Objective: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by recurrent serositis attacks and fever. The discovery of the Mediterranean fever (MEFV) gene has been a milestone in FMF etiopathogenesis. Our knowledge about the relationship between the MEFV gene and FMF phenotype increases each day. This study aims to investigate the relationship between MEFV gene mutations and the FMF clinical findings of a single-center FMF cohort. Methods: Gender, age, age at symptom onset, age at diagnosis, clinical characteristics, and MEFV gene analysis of the patients were recorded. Results: A total of 837 FMF patients were included in this study. There were 515 females and 322 males. The age at symptom onset was 18.3±10.9 years, while the age at diagnosis was 24.4±10.9 years. The most common symptom that accompanied fever was peritonitis (91.1%), while the other common clinical findings were pleuritis (45%), myalgia (44%), and arthritis (36%). A total of 47 patients developed amyloidosis. A total of 553 (66%) FMF patients had M694V mutation, 221 (26%) of which were homozygous, while 332 (40%) were heterozygous. Exon 10 mutation frequency was 759 (91%), while the non-exon 10 mutation frequency was 78 (9%). There was no wild type among the patients. Conclusion: In conclusion, the fact that a vast majority of the disease burden was constituted by the exon 10, especially M694V mutations and that none of the 837 patients from our cohort had a wildtype FMF proved the significance of MEFV gene mutation analysis. Therefore, we speculate that it is necessary to examine the MEFV gene mutations in each FMF suspected case. It seems plausible to re-evaluate the FMF diagnosis for cases in which a wild type MEFV gene mutation occurs. [ABSTRACT FROM AUTHOR]

Calcium pyrophosphate deposition disease (CPPD) is a crystal arthropathy, and may present with various clinical manifestations such as asymptomatic CPPD, osteoarthritis with CPPD, acute CPPD crystal arthritis (formerly pseudogout), and chronic CPPD crystal inflammatory arthritis. It is known that aging, trauma and osteoarthritis are major risk factors for CPPD. Acute CPP arthritis may occur as monoarticular or oligoarticular and usually involves large peripheral joints such as the knees, wrists and ankles. CPPD is characterized by sudden onset of severe pain, swelling and periarticular erythema, and systemic symptoms such as fever, chills, and weakness may occur. On the other hand, axial CPPD has been reported rarely and most cases appear with symptoms related to a mass effect such as foramen magnum syndrome, spinal stenosis, radiculopathy, myelopathy, synovial cyst or cauda equina syndrome. In addition, there are fewer reported cases of spinal CPPD that cause neck and back pain. This clinical condition should be considered in the differential diagnosis of acute neck and back pain. [ABSTRACT FROM AUTHOR]

Objective: Chronic inflammatory diseases are associated with altered body composition. Ghrelin has anti-inflammatory effects, and its level is altered in obesity and inflammatory diseases. The aim of the study was to evaluate the prevalence of obesity and ghrelin and obestatin levels in patients with Behçet's disease (BD). Material and Methods: One hundred and forty-three (143) patients with BD and 112 healthy controls (HC) were enrolled. Participants were subdivided according to the body mass index (BMI) as lean (<18.5 kg/m²), normal weight (18.5-24.9 kg/m²), overweight (25-29.9 kg/m²) and obese (≥30 kg/m²). In addition to the routine evaluations (fasting blood glucose, lipid profile, and kidney and liver function tests), serum acylated-ghrelin (AG), unacylated-ghrelin (UAG), total ghrelin (TG) and obestatin levels were analyzed. Student's t-test and chi-square test were used for statistical analysis. Results: The prevalence of obesity was relatively lower in the BD group than in the HC group (12.6% vs. 20.5%, p=0.089). Serum ghrelin levels were similar in the BD and HC groups (p>0.05 for all) although the obestatin level was higher in the BD group com- pared to the HC group (p<0.001). Serum UAG, TG and obestatin levels were lower in obese BD patients (n=18) than non-obese BD patients (p=0.027, p=0.014 and p=0.001, respectively). Conclusion: The obestatin level was high and the prevalence of obesity was low in the BD group. Moreover, obese BD patients had low obestatin levels. These results suggest that obestatin may protect BD patients from obesity. [ABSTRACT FROM AUTHOR]

Objective: Behçet's disease (BD) is a chronic, relapsing type of vasculitis of unknown etiology and is characterized by oral and urogenital ulcers and ocular inflammation with cutaneous, musculoskeletal, vascular, and nervous system manifestations. Few cases involving the nasal mucosa have been reported in the literature, and the true prevalence of BD remains unknown. Neurological involvement associated with BD might play a more important role in causing olfactory dysfunction than mucosal involvement, but sufficient clinical data are not available on the effect of BD on olfaction in adults. We therefore evaluated the olfactory function of patients diagnosed with BD. Material and Methods: Patients were chosen from among a consecutive patient group population who visited the internal medicine rheumatology polyclinic and otolaryngology departments of Ondokuz Mayıs University Hospital. A total of 50 patients (both males and females) aged 18 to 60 years with a diagnosis of BD and 46 healthy controls (matched to the study group in terms of age and gender) were included. BD was diagnosed based on the criteria defined by the International Study Group for BD. A complete clinical history was taken for and a physical examination was performed in all participants. Patients with other rheumatic diseases; obstructive nasal pathologies leading to conductive-type olfactory dysfunction (e.g., septum deviation or nasal polyp); advanced systemic disease (e.g., hypertension or malignancy); a history of antithyroid, antihistamine, antidepressant, or steroid medication use within the past month; or who were current smokers, had an active upper respiratory infection, or had a history of otolaryngologic operations were excluded. The results of the "Sniffin' Sticks" (SS) olfactory test were compared between the two groups. Results: The mean age of the 50 BD patients was 35.3±10 years; that of the 46 health controls was 36.9±11 years. There was no significant group difference in age or gender distribution (p>0.05). Odor identification and overall scores were significantly lower in the BD group than in the control group. There were no significant differences in odor discrimination scores between the BD and control groups (p>0.05). Conclusion: To our knowledge, this is the first study to evaluate olfactory function in patients diagnosed with BD using the SS test. Odor identification was more impaired in BD patients than in healthy controls, but there was no group difference in odor discrimination. BD patients should also be assessed for the involvement of olfactory function and may require treatment due to a malfunction of the olfactory system that affects the quality of life. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 47-year-old woman with a history of hand pain and swelling who was diagnosed with heterotopic ossification through the single-photon emission computed tomography (SPECT).

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by several immunological abnormalities. We wish to communicate the case of a patient with SLE and lupus nephritis (LN) who developed pseudothrombocytopenia. Pseudothrombocytopenia can occur in patients with SLE and LN and should be considered when diagnosing patients with thrombocytopenia without bleeding. [ABSTRACT FROM AUTHOR]

Sjögren's syndrome, an autoimmune disease characterized by lymphocytic infiltration of the lacrimal and salivary glands, leads to dryness of the mouth and eyes. Herein, we present a case of Sjögren's syndrome that developed after silicone breast implantation. A cause-effect relationship between breast implantation and Sjögren's syndrome has not been established. However, the possibility of such an association should be considered when a patient with silicone implants is admitted to the hospital for treatment of Sjögren's syndrome. [ABSTRACT FROM AUTHOR]

Objective: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by recurrent serositis attacks and fever. The discovery of the Mediterranean fever (MEFV) gene has been a milestone in FMF etiopathogenesis. Our knowledge about the relationship between the MEFV gene and FMF phenotype increases each day. This study aims to investigate the relationship between MEFV gene mutations and the FMF clinical findings of a single-center FMF cohort., Methods: Gender, age, age at symptom onset, age at diagnosis, clinical characteristics, and MEFV gene analysis of the patients were recorded., Results: A total of 837 FMF patients were included in this study. There were 515 females and 322 males. The age at symptom onset was 18.3±10.9 years, while the age at diagnosis was 24.4±10.9 years. The most common symptom that accompanied fever was peritonitis (91.1%), while the other common clinical findings were pleuritis (45%), myalgia (44%), and arthritis (36%). A total of 47 patients developed amyloidosis. A total of 553 (66%) FMF patients had M694V mutation, 221 (26%) of which were homozygous, while 332 (40%) were heterozygous. Exon 10 mutation frequency was 759 (91%), while the non-exon 10 mutation frequency was 78 (9%). There was no wild type among the patients., Conclusion: In conclusion, the fact that a vast majority of the disease burden was constituted by the exon 10, especially M694V mutations and that none of the 837 patients from our cohort had a wild-type FMF proved the significance of MEFV gene mutation analysis. Therefore, we speculate that it is necessary to examine the MEFV gene mutations in each FMF suspected case. It seems plausible to re-evaluate the FMF diagnosis for cases in which a wild type MEFV gene mutation occurs.

Objective: Systemic lupus erythematosus (SLE) and systemic sclerosis (SSc), chronic inflammatory diseases, demonstrate an increased incidence of cardiovascular manifestations and subclinical atherosclerotic disease. Salusin-α is a novel bioactive peptide that suppresses the formation of macrophage foam cells, and its serum level is significantly lower in patients with angiographically proven coronary artery disease. The aims of the study were to assess serum salusin-α level and its potential association with the predictors of atherosclerosis in SLE and SSc., Material and Methods: The study included 20 SLE and 22 SSc patients and 23 healthy controls (HC). All of the participants were female. Tumour necrosis factor-α (TNF-α), IL-6 and salusin-α levels, homeostasis model assessment for insulin resistance (HOMA-IR) index and common carotid intima-media thickness (IMT) were determined., Results: Salusin-α levels were lower and the IMTs were higher in the SLE and SSc groups than in the HC group. The salusin-α level was correlated with neither the disease activity scores nor cytokine levels and IMT in the SLE and SSc groups, although it was correlated with triglyceride level in the SLE group (r=-0.564, p=0.012), and with HOMA-IR index in the HC group (r=0.485, p=0.019)., Conclusion: The present preliminary study may support the idea that SSc leads to subclinical atherosclerosis, as in SLE. Moreover, it can be concluded that the decreased salusin-α levels in SLE and SSc may contribute to subclinical atherosclerosis. However, further studies with larger sample size are needed to demonstrate this contribution in SLE and SSc.