Investigation of the relationship between disease severity and development of amyloidosis and genetic mutation in FMF disease.

Background: Familial Mediterranean fever (FMF) is an autosomal recessive genetic disease. Amyloidosis is the most important complication of FMF that determines the prognosis of the disease.

Aims: In our study, we have investigated the relationship between the genetic mutations with the disease severity and the frequency of development of amyloidosis inpatients with FMF.

Budd-Chiari syndrome in Behçet's disease: a retrospective multicenter study.

Objective: To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS).

Methods: This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017.

Results: Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.

The distribution of MEFV mutations in Turkish FMF patients: multicenter study representing results of Anatolia.

Background/aim: The distribution of Mediterranean fever (MEFV) gene mutations in Turkish familial Mediterranean fever (FMF) patients varies according to geographic area of Turkey. There is a need for highly representative data for Turkish FMF patients. The aim of our study was to investigate the distribution of the common MEFV mutations in Turkish FMF patients in a nationwide, multicenter study.

Ptosis and Mass Like Lesions in Behçet's Disease: A Rare Presentation.

In this article, we describe a case of neuro-Behçet's disease presenting with unilateral ptosis and facial paresis due to an intracranial mass lesion. A 25-year-old male patient with a history of Behçet's disease presented with headache, vertigo, double vision, ptosis in his right eyelid and slurred speech. Cranial magnetic resonance imaging scan revealed a right-sided capsulothalamic lesion, which was extending to the right cerebral pedicle, pons and superior cerebellar pedicle.

Symmetric polyarthritis as an initial symptom in granulomatosis with polyangiitis: A report of six cases and review of the literature.

Granulomatosis with polyangiitis (GPA) is a primary systemic vasculitis characterized by granulomatous inflammation. Arthritis in GPA is most commonly associated with large joints, particularly the knees and ankles; however, symmetrical polyarthritis of small joints has been rarely reported in literature. Here, we describe retrospective analysis of six patients with GPA showing initial symptom of symmetrical polyarthritis who were followed-up by three different rheumatology departments.

Nationwide Experience With Off-Label Use of Interleukin-1 Targeting Treatment in Familial Mediterranean Fever Patients.

Objective: Approximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients.

Changes in Cerebral Blood Flow in Patients with Familial Mediterranean Fever.

Introduction: It is known that there is a relationship between systemic inflammation and atherosclerosis. Atherosclerosis is one of the best-known causes of cerebrovascular diseases. The aim of this study was to assess cerebral blood flow velocity using transcranial Doppler (TCD) ultrasonography in patients with familial Mediterranean fever (FMF).

Evaluation of olfactory function in Behçet's disease.

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.

The management of gout in different clinical specialties in Turkey: a patient-based survey.

Although gout is potentially curable, the management of this disease is often suboptimal. In this study, we investigated the treatment of gout in Turkey and also compared the management approaches to gout in different clinical specialties. Three hundred and nineteen consecutive patients (mean age 58.

Ethylenediaminetetraacetic acid-dependent pseudothrombocytopenia in a patient with systemic lupus erythematosus and lupus nephritis.

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.

Sjögren's syndrome after silicone breast implantation.

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.

A multicenter report of biologic agents for the treatment of secondary amyloidosis in Turkish rheumatoid arthritis and ankylosing spondylitis patients.

In this multicenter, retrospective study, we evaluated the efficacy and safety of biologic therapies, including anti-TNFs, in secondary (AA) amyloidosis patients with ankylosing spondylitis (AS) and rheumatoid arthritis (RA). In addition, the frequency of secondary amyloidosis in RA and AS patients in a single center was estimated. Fifty-one AS (39M, 12F, mean age: 46.

Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort.

Background: Adult-onset Still's disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD.

Methods: A multicenter study was used to reach sufficient patient numbers.

Assessment of the New 2012 EULAR/ACR Clinical Classification Criteria for Polymyalgia Rheumatica: A Prospective Multicenter Study.

Objective: To assess the performance of the new 2012 provisional European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) polymyalgia rheumatica (PMR) clinical classification criteria in discriminating PMR from other mimicking conditions compared with the previous 5 diagnostic criteria in a multicenter prospective study.

Methods: Patients older than 50 years, presenting with new-onset bilateral shoulder pain with elevated acute-phase reactants (APR), were assessed for the fulfillment of the new and old classification/diagnostic criteria sets for PMR. At the end of the 1-year followup, 133 patients were diagnosed with PMR (expert opinion) and 142 with non-PMR conditions [69 rheumatoid arthritis (RA)].

Characteristics Predicting Tuberculosis Risk under Tumor Necrosis Factor-α Inhibitors: Report from a Large Multicenter Cohort with High Background Prevalence.

Objective: Screening strategies for latent tuberculosis (TB) before starting tumor necrosis factor (TNF)-α inhibitors have decreased the prevalence of TB among patients who are treated with these agents. However, despite vigilant screening, TB continues to be an important problem, especially in parts of the world with a high background TB prevalence. The aim of this study was to determine the factors related to TB among a large multicenter cohort of patients who were treated with anti-TNF.

The IL-33 gene is related to increased susceptibility to systemic sclerosis.

Systemic sclerosis (SSc) is a chronic inflammatory disease characterized by widespread fibrosis of the skin and several visceral organs. The pro-fibrotic potential of interleukin (IL)-33 has been demonstrated by in both in vitro and in vivo settings; moreover, increased level of IL-33 has also been reported in patients with SSc. Therefore, the aim of the present study was to detect the potential association of IL-33 gene polymorphisms on the susceptibility of SSc.

Investigation of the association between Rho/Rho-kinase gene polymorphisms and systemic sclerosis.

Systemic sclerosis (SSc) is a disease characterized by inflammation, vascular abnormalities and fibrosis. The role of Rho/Rho-kinase pathway was demonstrated in the pathogenesis of fibrosis, inflammation and vascular abnormalities. This study was aimed to investigate the relation between SSc and Rho/Rho-kinase gene polymorphisms.

A rare comorbidity: neurosarcoidosis and cutaneous sarcoidosis.

We present a case of a neurosarcoidosis patient with skin lesions. A 50-year-old woman was admitted with a 1-year history of violaceous, smooth and shiny plaques on her face and right arm. These lesions were biopsied and the histological examination indicated sarcoidosis.

Association of TRPM Channel Gene Polymorphisms with Systemic Sclerosis.

Background/aim: Systemic sclerosis (SSc) is an inflammatory disease characterized by vascular abnormalities and fibrosis. The aim of the present study was to investigate the possible role of transient receptor potential melastatin (TRPM) channel genes in the susceptibility and phenotype expression of SSc.

Materials And Methods: A total of 339 patients with SSc and 302 healthy controls were studied.

Bilateral sacroiliitis and uveitis comorbidity: brucellosis? Ankylosing spondylitis?

We present a rare case of a comorbidity of sacroiliitis and brucellosis infection. A 28-year-old woman received irregular medication due to ongoing backache and hip pain for 5 years. The patient presented to our hospital for evaluation of visual loss and was diagnosed with uveitis.

The rate and significance of type 1/type 2 serum amyloid A protein gene polymorphisms in patients with ankylosing spondylitis and amyloidosis.

A relationship between the presence of amyloidosis and SAA1 genotype has been shown in recent studies of (principally) familial Mediterranean fever patients. We found that the SAA1 rs12218 polymorphism was significantly more prevalent in ankylosing spondylitis patients with amyloidosis.