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https://www.readbyqxmd.com/read/29094181/cardiovascular-disease-in-patients-with-autoinflammatory-syndromes
#1
REVIEW
Rainer Hintenberger, Agnes Falkinger, Kathrin Danninger, Herwig Pieringer
Autoinflammatory syndromes (AIS) are characterized by recurring events of inflammation, leading to a variety of organ manifestations and fever attacks. A subgroup of AIS is commonly referred to as hereditary periodic fever syndromes (HPFS). There is substantial evidence that autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus are strongly associated with cardiovascular morbidity and mortality. The link between AIS and cardiovascular disease is not that clear, even if the concept of continuous inflammation as a risk factor for cardiovascular disease is widely accepted...
November 1, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29051974/cardiac-disease-in-familial-mediterranean-fever
#2
REVIEW
Eren Erken, Ertugrul Erken
Familial Mediterranean fever (FMF) is an autoinflammatory disease manifested by inflammatory attacks of peritonitis, pleuritis, pericarditis accompanied by fever and arthritis. Mutations of MEFV gene results in pyrin dysfunction, which causes uncontrolled interleukin-1 beta production and triggers the inflammatory attacks. Inflammation persists even during attack-free periods in one-third of the FMF patients. Findings of elevated proinflammatory cytokine patterns during remission as well as inflammatory attacks indicate the continuous subclinical disease activity and inflammation...
October 20, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28149656/immune-and-inflammatory-gene-expressions-are-different-in-beh%C3%A3-et-s-disease-compared-to-those-in-familial-mediterranean-fever
#3
Filiz Türe Özdemir, Emel Ekşioğlu Demiralp, Sibel Z Aydın, Pamir Atagündüz, Tülin Ergun, Haner Direskeneli
OBJECTIVE: The immune classification of Behçet's disease (BD) is still controversial. In this study, we aimed to compare the immune/inflammatory gene expressions in BD with those in familial Mediterranean fever (FMF), an autoinflammatory disorder with innate immune activation. MATERIAL AND METHODS: CD4+ T cells and CD14+ monocytes were isolated from the peripheral blood mononuclear cells of Behçet's disease patients (n=10), FMF (n=6) patients, and healthy controls (n=4) with microbeads, and then, the mRNA was isolated...
December 2016: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27776949/beh%C3%A3-et-s-disease%C3%A2-and-familial-mediterranean-fever-two-sides-of-the-same-coin-or-just-an-association-a-cross-sectional-study%C3%A2
#4
Abdulla Watad, Shmuel Tiosano, Dana Yahav, Doron Comaneshter, Yehuda Shoenfeld, Arnon D Cohen, Howard Amital
BACKGROUND: Familial Mediterranean fever (FMF) is a genetic disease, characterized by attacks of fever, arthritis, serositis and pain. Behçet's disease (BD) is an inflammatory disorder with a genetic basis, characterized by oral and genital ulcers, uveitis, pustular erythematous cutaneous lesions, arthritis, central nervous system involvement and possible vascular manifestations such as venous thrombosis, arteritis and aneurysms. OBJECTIVES: To investigate the association and actual differentiation between these two entities in a large-scale population-based study...
April 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/27733942/the-role-of-mefv-mutations-in-the-concurrent-disorders-observed-in-patients-with-familial-mediterranean-fever
#5
Sabri Güncan, N Şule Y Bilge, Döndü Üsküdar Cansu, Timuçin Kaşifoğlu, Cengiz Korkmaz
OBJECTIVE: This study aimed to investigate the frequency in which familial Mediterranean fever (FMF) coexists with other diseases and determine whether Mediterranean fever (MEFV) gene mutations are involved in such coexistence. MATERIAL AND METHODS: In total, 142 consecutive patients with FMF investigated for MEFV mutation were enrolled in this study [Female: 87; Male: 55, mean age 32±12 years (11-62)]. All the patients were questioned for the presence of concurrent disorders, and the medical records of these patients were revised retrospectively...
September 2016: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27402112/juvenile-spondyloarthropathies
#6
REVIEW
Amra Adrovic, Kenan Barut, Sezgin Sahin, Ozgur Kasapcopur
Juvenile spondyloarthropathies represent a clinical entity separate from the adult disease. Initial clinical signs of juvenile spondyloarthropathies often include lower extremity arthritis and enthesopathy, without axial involvement at the disease onset. Asymmetrical oligoarthritis of lower extremities is typically seen in this type of arthritis. Enthesopathy, which is the hallmark of the disease, is most commonly seen in the Achilles tendon, being manifested by heel pain. Anterior uveitis and HLA-B27 positivity are seen in a proportion of cases...
August 2016: Current Rheumatology Reports
https://www.readbyqxmd.com/read/26928096/assessment-of-17-pediatric-cases-with-colchicine-poisoning-in-a-2-year-period
#7
Demet Alaygut, Suar Caki Kilic, Ali Kaya, Mehmet Burhan Oflaz, Fatih Bolat, Ömer Cevit, Fusun Dilara Icagasioglu
AIM: The aim of the study is to discuss clinical effects, treatments, and outcomes of pediatric colchicine poisoning. METHOD: This study was designed as an observational case series study. The medical records of children aged between 0 and 18 years, who were hospitalized for colchicine poisoning at the Department of Pediatric Intensive Care Unit, Cumhuriyet University Faculty of Medicine, between January 2010 and January 2012, were retrospectively evaluated. RESULTS: We presented 17 children with colchicine poisoning...
March 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/26239119/colchicine-as-an-anti-inflammatory-and-cardioprotective-agent
#8
REVIEW
Armen Yuri Gasparyan, Lilit Ayvazyan, Marlen Yessirkepov, George D Kitas
INTRODUCTION: Colchicine has been successfully used for the treatment of neutrophilic disorders such as familial Mediterranean fever (FMF), Behçet disease (BD) and gout. There is a growing interest in its cardiovascular effects. AREAS COVERED: A MEDLINE/PubMed search for English articles published from January 1972 to June 2015 was completed using the following terms: therapy, pharmacokinetics, efficiency, side effects, toxicity, heart, colchicine, inflammation, FMF, amyloidosis, BD, gout, cardiovascular disorders, pericarditis, arrhythmias, inflammation, neutrophils, platelets...
2015: Expert Opinion on Drug Metabolism & Toxicology
https://www.readbyqxmd.com/read/25604718/19-year-old-male-with-a-history-of-recurrent-episodes-of-calf-pain-headache-and-fever
#9
Eli Ben-Chetrit, Chamutal Gur, Eldad Ben-Chetrit
No abstract text is available yet for this article.
December 2015: Arthritis Care & Research
https://www.readbyqxmd.com/read/25507981/cryopyrin-associated-periodic-syndrome
#10
Eitan Giat, Merav Lidar
CAPS is a rare autoinflammatory disease associated with mutations in the NLRP3 gene that result in overactivation of the inflammasome, increased secretion of IL-1beta and IL-18, and systemic inflammation. Genetic testing has allowed for grouping of the three, previously distinct clinical syndromes of FCAS, MWS and NOMID, into a single syndrome termed CAPS. The clinical features include urticarial rash and fever, CNS and musculoskeletal involvement, ocular disorders and progressive deafness. Onset, severity and complications (mainly retardation, seizures, destructive arthropathy and amyloidosis) depend on the specific mutation...
October 2014: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/25438464/cryopyrin-associated-periodic-syndrome
#11
Eitan Giat, Merav Lidar
CAPS is a rare autoinflammatory disease associated with mutations in the NLRP3 gene that result in overactivation of the inflammasome, increased secretion of IL-1beta and IL-18, and systemic inflammation. Genetic testing has allowed for grouping of the three, previously distinct clinical syndromes of FCAS, MWS and NOMID, into a single syndrome termed CAPS. The clinical features include urticarial rash and fever, CNS and musculoskeletal involvement, ocular disorders and progressive deafness. Onset, severity and complications (mainly retardation, seizures, destructive arthropathy and amyloidosis) depend on the specific mutation...
October 2014: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/24765296/coexistence-of-beh%C3%A3-et-s-disease-with-ankylosing-spondylitis-and-familial-mediterranean-fever-a-rare-occurrence
#12
Makram Frigui, Manel Kechaou, Moez Jallouli, Neila Kaddour, Habiba Bouhamed Chaabouni, Zouhir Bahloul
Behçet's disease (BD) and familial Mediterranean fever (FMF), which are two separate diseases sharing some clinical features, may also coexist in the same patient. Further investigations are needed to understand whether this coexistence is due to either chance or geographical distribution patterns of these diseases or to common etiopathogenetic characteristics. Spondylarthritis as part of the clinical picture in these two diseases has been questioned and probably it is not a prominent characteristic of any of them...
May 16, 2011: Clinics and Practice
https://www.readbyqxmd.com/read/24592325/idiopathic-uveitis-and-familial-mediterranean-fever-is-there-any-relationship
#13
Farhad Salehzadeh, Ozra Yasrebi, Mahsa Hosseini Khotbesara, Maryam Hosseini Khotbesara
Introduction. Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by attacks of fever and polyserositis. FMF is often associated with other autoimmune diseases such as rheumatoid arthritis, polyarteritis nodosa (PAN), and Behcet. Uveitis is an inflammatory process caused by underlying infectious and inflammatory disorders. This study investigates the probable relationship between idiopathic uveitis and FMF. Methods. Patients with idiopathic uveitis were analyzed for the 12 most common MEFV mutations (P369S, F479L, M680I(G/C), M680I(G/A), I692del, M694V, M694I, K695R, V726A, A744S, R761H, E148Q) by a reverse hybridization assay (FMF StripAssay,Vienna lab,Vienna, Austria)...
2014: Autoimmune Diseases
https://www.readbyqxmd.com/read/24589726/fibromyalgia-in-patients-with-other-rheumatic-diseases-prevalence-and-relationship-with-disease-activity
#14
Sema Haliloglu, Ayse Carlioglu, Derya Akdeniz, Yasar Karaaslan, Ali Kosar
Fibromyalgia (FM) is a syndrome characterized by chronic widespread pain and the presence of specific tender points. The prevalence of FM has been estimated at 2-7 % of the general global population. The presence of FM in several rheumatic diseases with a structural pathology has been reported as 11-30 %. The objectives of this study were to determine the prevalence of FM and to evaluate the possible relationship between FM existence and disease activity among rheumatic diseases. The study group included 835 patients--197 rheumatoid arthritis (RA), 67 systemic lupus erythematosus (SLE), 119 ankylosing spondylitis (AS), 238 osteoarthritis (OA), 14 familial Mediterranean fever (FMF), 53 Behçet's disease (BD), 71 gout, 25 Sjögren's syndrome (SS), 20 vasculitis, 29 polymyalgia rheumatica (PMR), and two polymyositis (PM)--with or without FM...
September 2014: Rheumatology International
https://www.readbyqxmd.com/read/24583586/-a-case-of-colchicine-responsive-mollaret-s-meningitis-with-mefv-gene-mutation
#15
Tomomi Kinohshita, Akira Matsushima, Shunichi Satoh, Kenichi Hoshi, Dai Kishida, Hiroyuki Yahikozawa
A 66-year-old woman was admitted to our hospital with recurrent meningitis. She presented with 10 episodes of meningitis in 10 months. Examination of cerebrospinal fluid demonstrated pleocytosis, with neutrophils dominant at the early stage, and lymphocytes dominant at the late stage. Mollaret cells were found and the level of IL-6 was increased in cerebrospinal fluid. Several antibiotics and antiviral agents failed to prevent relapse. However, colchicine therapy successfully prevented the recurrence of meningitis...
2014: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/24551444/familial-mediterranean-fever-in-an-iranian-patient-with-behcet-disease
#16
Maryam Mobini
BACKGROUND: Familial Mediterranean fever (FMF) is the most prevalent disorder among the hereditary autoinflammatory syndromes. This disorder is characterized by fever and some painful attacks such as abdominal, chest or joint pain and potentially development of AA amyloidosis. Several vasculitis are more common in FMF than general population. There are some reports about association of FMF with Behcet Disease (BD). CASE PRESENTATION: In this study, we describe a 27 year old patient with BD who suffered from attacks of fever, arthralgia, abdominal pain and genetic study confirmed the diagnosis of FMF...
2011: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/24369417/no-appreciable-decrease-in-fertility-in-beh%C3%A3-et-s-syndrome
#17
Didem Uzunaslan, Caner Saygin, Gulen Hatemi, Koray Tascilar, Hasan Yazici
OBJECTIVES: Behçet's syndrome (BS) follows an active course during the childbearing years in both men and women. We formally surveyed the infertility rate and the effect of drugs and types of organ involvement on fertility in BS. METHODS: We compared fertility among BS patients with and without major organ involvement with those with FMF, AS and healthy controls. A structured interview was performed and the medical records of the patients were reviewed to confirm the sites of involvement and drugs they used during their entire follow-up...
May 2014: Rheumatology
https://www.readbyqxmd.com/read/24338581/ophthalmic-manifestations-in-familial-mediterranean-fever-a-case-series-of-6-patients
#18
Alper Yazici, Pinar Ozdal, Pinar Yuksekkaya, Ufuk Elgin, Mehmet Yasin Teke, Esin Sari
PURPOSE: To describe the ocular involvement of patients with familial Mediterranean fever (FMF) followed in a tertiary referral center. METHODS: The data of 6 patients with FMF were retrospectively reviewed. Detailed ophthalmologic examinations, type of inflammation, course of the disease, number of recurrences, treatment regimens, complications, and comorbid ocular or systemic diseases were noted. RESULTS: The mean age ± SD at diagnosis was 29...
July 2014: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/24064018/the-prevalence-of-atopy-in-patients-with-familial-mediterranean-fever-and-beh%C3%A3-et-s-disease
#19
Ayten Yazici, Emel Orge Gonullu, Bulent Kardes, Ayse Cefle
OBJECTIVES: The purpose of this study is to investigate the prevalence of atopy in patients with Behçet's disease (BD) and familial Mediterranean fever (FMF). METHODS: In this study, 42 BD patients, 40 FMF patients and 49 healthy subjects were included. The skin test was applied to the whole group. If one or more allergen response was equal or greater than histamine response, it was accepted as atopy. At the same time, total serum IgE and peripheral blood eosinophil levels were also determined...
May 2013: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/24064017/fever-in-beh%C3%A3-et-s-syndrome
#20
Emire Seyahi, Huseyin Karaaslan, Serdal Ugurlu, Hasan Yazici
OBJECTIVES: Fever is taken to be rare in Behçet's syndrome (BS) and when present it is usually considered to be associated with vascular disease. The aim of this study was to formally investigate the presence of fever as a clinical feature in BS patients and suitable controls. METHODS: The study consisted of 2 parts. In the first part, 500 patients with BS, 94 with familial Mediterranean fever (FMF), 100 with ankylosing spondylitis (AS), and 72 with systemic lupus erythematosus (SLE) along with 100 healthy controls (HC) were surveyed with the help of a questionnaire for the history of periodic fever episodes...
May 2013: Clinical and Experimental Rheumatology
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