RESUMO
Takayasu arteritis (TA) is an extremely uncommon vasculitis that primarily affects the aorta and its branches. Due to the genetic and ethnicity effect, a diverse array of TA clinical manifestations has been reported worldwide. The purpose of the present study was to compare the clinicodemographic characteristics and pattern of vascular involvement of Iranian and Turkish TA patients. This study was a retrospective, cross-sectional investigation of 126 TA patients in Iran and Turkey. All of the variables analyzed were extracted from historical medical records. In 126 TA patients, the ratio of females to males was 8.6:1, and the average age at onset of disease was 30.5±11.1 years. Fatigue (49.2%) and a weak or absent pulse (79.4%) were the most prevalent symptoms and signs, respectively. The most prevalent angiographic classifications were types V and I in Iranian patients (41.09%) and type I in the Turkish population (47.7%) The left subclavian artery was the vessel most frequently affected by TA (66.6%). Our findings indicated that there were no significant differences between the two countries in terms of clinicodemographic characteristics or vascular involvement. Some clinical manifestations, such as claudication, were more prevalent in the Turkish population due to a higher incidence of occlusive lesions in the right subclavian artery.
Assuntos
Arterite de Takayasu , Masculino , Feminino , Humanos , Adulto Jovem , Adulto , Arterite de Takayasu/complicações , Arterite de Takayasu/diagnóstico por imagem , Arterite de Takayasu/epidemiologia , Estudos Retrospectivos , Irã (Geográfico)/epidemiologia , Turquia/epidemiologia , Estudos TransversaisRESUMO
BACKGROUND: Psoriatic arthritis (PsA) is a chronic and debilitating disease that can be managed by different clinical specialists. OBJECTIVES: The objective of the LOOP study was to evaluate the impact of clinical specialty setting on the time to diagnosis and treatment of patients with PsA. Clinical disease activity and disease burden were also compared between clinical settings. METHODS: LOOP was a cross-sectional, multicentre, observational study conducted in 17 countries in Western and Eastern Europe, the Middle East, Latin America and Asia. Adult patients (≥18 years) with a suspected or established diagnosis of PsA who were routinely visiting a rheumatologist, dermatologist or non-rheumatology/non-dermatology physician were enrolled. All patients were assessed by both a rheumatologist and a dermatologist. RESULTS: Of 1483 enrolled patients, a total of 1273 had a confirmed diagnosis of PsA. There was no significant difference in the median time from onset of inflammatory musculoskeletal symptoms to PsA diagnosis between patients enrolled by rheumatologists and dermatologists (6.0 vs. 3.9 months). However, the median time from diagnosis to first treatment with a conventional synthetic disease-modifying anti-rheumatic drug (csDMARD) was significantly shorter in the rheumatology setting compared with the dermatology setting (0 vs. 2.0 months; P < 0.001). In addition, disease activity was significantly higher in the dermatology setting compared with the rheumatology setting. CONCLUSIONS: Differences in the management and clinical status of patients with PsA were observed between the rheumatology and dermatology settings. Importantly, median time from diagnosis to first csDMARD was significantly shorter in the rheumatology setting, and patients in the dermatology setting had higher disease activity. These data show the importance of improved collaboration between rheumatologists and dermatologists.
Assuntos
Artrite Psoriásica , Dermatologia , Psoríase , Reumatologia , Adulto , Artrite Psoriásica/diagnóstico , Artrite Psoriásica/tratamento farmacológico , Ásia , Estudos Transversais , Gerenciamento Clínico , Europa Oriental , Humanos , Oriente MédioRESUMO
PURPOSE: The aim of this study was to determine the fracture strength of endocrowns made of lithium disilicate ceramic and two different indirect resin composites. MATERIALS AND METHODS: Forty human mandibular molars were randomly separated into four groups (n = 10 in each group) - Group IN: control group, Group IPS: endocrowns made of lithium disilicate ceramic (IPS e.max CAD, IvoclarVivadent, Schaan, Liechtenstein); Group SL: Endocrowns made of Solidex microhybrid composite (Shofu, Ratingen, Germany); and Group GR: Endocrowns made of Grandia microhybrid composite (GC Europa, Leuven, Belgium). In all of the groups, dual-cure resin cement (Relyx Ultimate Clicker, 3M ESPE, St. Paul, MN, USA) was used to cement the endocrowns. All of the teeth were subjected to fracture by means of a universal testing machine (Instron), and compressive force was applied. The failure type and location after fracture were classified. The data were analyzed using one-way ANOVA, Tukey's post hoc test, and Chi-square test (P < 0.05). RESULTS: Group IPS showed significantly higher fracture strength than Groups SL and GR (P < 0.05). There was no significant difference between the SL and GR groups (P > 0.05). In Group SL, 80% of the specimens exhibited favorable fractures; also, 60% of the specimens exhibited favorable fracture in group GR, and only 10% of the specimens exhibited favorable fracture in group IPS. CONCLUSIONS: The lithium disilicate ceramic endocrowns exhibited higher fracture resistance than indirect composite groups. Both of the composite endocrowns showed more favorable failure than the lithium disilicate ceramic endocrowns.
Assuntos
Resinas Compostas/uso terapêutico , Coroas , Cimentos Dentários/química , Porcelana Dentária/química , Dente Molar , Cimentos de Resina/química , Cerâmica , Resinas Compostas/química , Análise do Estresse Dentário , Cimentos de Ionômeros de Vidro/química , Humanos , Cimentos de Resina/uso terapêutico , Resistência à TraçãoRESUMO
PURPOSE: This study aimed to evaluate the enamel remineralization efficacy of enamel matrix derivative (EMD), experimental bioactive glass (BAG), and fluoride varnish in vitro. METHODS AND MATERIALS: Artificial initial caries lesions were developed on fifty human enamel specimens using demineralization solution (pH 4.5, 37°C, 96 hours). Specimens were randomly assigned to five groups (n=10): I-5% NaF varnish (Enamelast), II-experimental 58S5 BAG+37% phosphoric acid (PA), III-EMD (Emdogain) + Ethylenediaminetetraacetic Acid (EDTA), IV-EMD+37% PA, V-Control (untreated). All remineralization agents were applied with pH cycling for seven days. The specimens were scanned by spectral domain optical coherence tomography (SD-OCT) at baseline, at demineralization, and after pH cycling. Lesion depths were measured using image analysis software (ImageJ). Lesions were evaluated using surface microhardness (SMH) and two fluorescence methods (FluoreCam and DIAGNOdent Pen [DDPen]). The data were statistically analyzed by Kruskal Wallis, Friedman, and Wilcoxon tests (α=0.05). RESULTS: According to SD-OCT results, fluoride varnish was found to be the most effective agent in reducing lesion depth (p=0.005). All agents increased the SMH values after pH cycling. No significant difference was found among fluoride varnish, BAG, and EMD+PA groups. These SMH values were significantly higher than EMD+EDTA and control groups (p<0.001). All groups showed lower DDPen scores compared with the control group (p<0.001), however, no significant difference was found among the remineralization agents. In FluoreCam assessment, size and intensity values of all treated groups showed improvement. However, there was no significant difference between the treatment groups in terms of FluoreCam size measurements (p=0.186). CONCLUSION: 58S5 BAG and EMD+PA have remineralization capacity as effective as fluoride varnish. EMD+PA showed better SMH and lesion intensity results than EMD+EDTA.
Assuntos
Esmalte Dentário , Fluoretos Tópicos , Remineralização Dentária , Humanos , Remineralização Dentária/métodos , Fluoretos Tópicos/uso terapêutico , Esmalte Dentário/efeitos dos fármacos , Cariostáticos/uso terapêutico , Cárie Dentária/prevenção & controle , Técnicas In Vitro , Fluoreto de Sódio/uso terapêutico , Vidro , Tomografia de Coerência Óptica/métodos , Desmineralização do Dente/prevenção & controle , Desmineralização do Dente/tratamento farmacológico , Ácido Edético/uso terapêuticoRESUMO
OBJECTIVE: In our study, we analyzed the efficacy and safety data of patients with systemic lupus erythematosus (SLE) after switching to biosimilar rituximab (RTX). PATIENTS AND METHODS: Twenty-two patients who switched to RTX were included in the study. Efficacy data were analyzed using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score, and safety data were analyzed using the frequency of side effects. RESULTS: The mean treatment duration of originator RTX was 35.6 ± 23.0 months, and the median treatment duration of biosimilar RTX was 17 months. The SLEDAI-2K score, approximately three months after the first dose of biosimilar RTX, was significantly lower (p = 0.027). A statistically significant difference was found between the SLEDAI-2K score assessed at the follow-up visit three months after the last dose of originator RTX and the SLEDAI-2K score obtained approximately three months after the first dose of biosimilar RTX (p = 0.011) and the calculated median SLEDAI-2K score was significantly lower than the SLEDAI-2K score assessed after administration of originator RTX. The side effect frequency that developed during the treatment of originator RTX was 15.3 per 100 patient-years. The most common side effect was infection, which was 15.3 per 100 patient-years. The most frequent infection was urinary tract infection. The side effect frequency during treatment of biosimilar RTX was 39 per 100 patient-years, and the most frequent infection was pneumonia. CONCLUSIONS: In our study, SLEDAI-2K scores demonstrated that no efficacy loss was experienced after switching to CT-P10 molecule, which is a biosimilar RTX. It was observed that switching to biosimilar RTX did not decrease treatment efficacy in the patient group diagnosed with SLE and biosimilar RTX was found to be safe.
Assuntos
Medicamentos Biossimilares , Lúpus Eritematoso Sistêmico , Rituximab , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/diagnóstico , Rituximab/efeitos adversos , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Medicamentos Biossimilares/administração & dosagem , Medicamentos Biossimilares/efeitos adversos , Medicamentos Biossimilares/uso terapêutico , Estudos Retrospectivos , Feminino , Masculino , Adulto , Pessoa de Meia-Idade , Resultado do Tratamento , Substituição de Medicamentos , Anticorpos Monoclonais MurinosRESUMO
OBJECTIVE: Behçet's disease etiology is uncertain, and no specific diagnostic markers exist in the laboratory. This retrospective study aimed to evaluate the role of inflammatory and hematological parameters, mainly Pan-Immune-Inflammation-Value (PIV), in predicting vascular Behçet's disease (VBD). PATIENTS AND METHODS: A total of 85 patients with VBD and 92 patients without vascular involvement (non-VBD) were included in this study. Neutrophil, monocyte, platelet, and lymphocyte subsets are all included in the PIV, a new blood-based biomarker. RESULTS: The optimal cut-off values for the PIV were determined to be ≥261.6. White blood cell, neutrophil, monocyte, hemoglobin, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration (MCHC), red cell distribution, platelet, plateletcrit, PIV, neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, sedimentation, c-reactive protein (CRP) values were significantly associated with VBD in univariate analysis. After multivariate analysis, PIV [odds ratio (OR): 2.758; 95% confidence interval (CI): 1.327-5.736; p=0.007] and CRP (OR: 4.029; 95% CI: 1.924-8.438; p<0.001) were found to be a positive predictor for VBD, while MCHC (OR: 0.722; 95% CI: 0.530-0.983; p=0.039) was seen as a negative predictor. CONCLUSIONS: Based on our results, PIV, an easily accessible, cost-effective, and new composite biomarker, has a significant predictive value in VBD.
Assuntos
Síndrome de Behçet , Humanos , Síndrome de Behçet/diagnóstico , Estudos Retrospectivos , Inflamação/diagnóstico , Proteína C-Reativa , BiomarcadoresRESUMO
This study investigated the effect of different intermediary layer materials under class II mesio-occluso-distal (MOD) resin composite restorations on volumetric cuspal deflection, gap formation, and fracture strength. In total, 32 sound human maxillary premolars were used. After large, standardized Class II MOD cavities were prepared, a universal adhesive (Clearfil Universal Bond Quick, Kuraray) was applied. The premolars were randomly allocated into four groups according to different intermediary layer materials (n=8): Group Z250 (control)/micro-hybrid composite (Filtek Z250, 3M ESPE); Group EST/low-viscosity bulk-fill resin composite (Estelite Bulk Fill Flow, Tokuyama Dental Corp) + micro-hybrid composite; Group NOV/nanofiber-reinforced low-viscosity composite (NovaPro Flow, Nanova) + micro-hybrid composite; and Group RIB/polyethylene fiber [Ribbond, Ribbond Inc] + micro-hybrid composite. Distilled water was used for storage for 24 hours. Using microcomputed tomography (micro-CT), the teeth were scanned immediately after cavity preparation (T0), then 24 hours after restorative procedures (T1). Volumetric cuspal deflection in cubic millimeters (mm3) was analyzed on the palatal and buccal regions of each restoration individually at T0 and T1 scans. Gap formation (mm3) was evaluated to quantify the volume of black spaces at the tooth-resin interface on the T1 scan. After these scans, using a universal testing machine, the teeth were subjected to a fracture strength test (0.5 millimeters/minute [mm/min]). The fracture surfaces were analyzed with a stereomicroscope. The data were analyzed using the Kruskal-Wallis, one-way analysis of variance (ANOVA), and Dunn's tests (p< 0.05). No significant differences in volumetric cuspal deflection and fracture strength were detected for all tested groups (p>0.05). Group RIB exhibited significantly higher gap formation values in comparison with all other groups (p<0.05). Predominant failure mode was favorable.
Assuntos
Restauração Dentária Permanente , Resistência à Flexão , Humanos , Restauração Dentária Permanente/métodos , Microtomografia por Raio-X , Resinas Compostas/uso terapêutico , Resinas Compostas/química , Materiais Dentários/química , Preparo da Cavidade Dentária/métodos , Teste de MateriaisRESUMO
PURPOSE: The objective of this study was to assess the effect of bioactive pulp-capping materials on human dental pulp stem cell (hDPSC) behavior in terms of cell viability and bioactivity via mineralization potential. METHODS AND MATERIALS: Nanoparticles of 58S5 bioactive glass (nBG) powder were elaborated by a sol-gel process. Primer hDPSCs were cultured with experimental nBG, Biodentine, TheraCal LC, and ProRoot mineral trioxide aggregate (MTA) extracts. Cell viability was measured for 1, 3, and 7 days by water-soluble tetrazolium salts (WST-1) assay. Expression of mineralization-related marker genes (dentin sialophosphoprotein [DSPP] and osteocalcin [OCN]) was quantified by a real-time polymerase chain reaction. Detection of DSPP protein expression in hDPSCs was also assessed by western blotting. Alizarin red staining was used to detect the formation of mineralized nodules, and alkaline phosphatase (ALP) activity was quantified by a photometric method (days 7 and 14). All data were statistically analyzed with a one-way analysis of variance (ANOVA) and Tukey's post-hoc test (p<0.05). RESULTS: The cell viability of hDPSCs in all groups decreased except for nBG, and the lowest cell viability was determined in TheraCal LC at all incubation times. nBG and MTA showed significantly higher ALP activity than the control group. The tested materials elevated the calcium nodule form of hDPSCs except for TheraCal LC. The highest DSPP expression was seen in nBG for both incubation times. CONCLUSION: nBG promotes differentiation and mineralization of hDPSCs at a higher rate than other bioactive pulp-capping materials tested.
Assuntos
Compostos de Cálcio , Polpa Dentária , Humanos , Sobrevivência Celular , Compostos de Cálcio/farmacologia , Silicatos/farmacologia , Diferenciação Celular , Células-Tronco , Combinação de Medicamentos , Óxidos/farmacologiaRESUMO
The aim of this study was to evaluate the effects of different polishing systems on the surface roughness of different nanocomposite resins using various analysis methods. Three types of nanocomposite resins were investigated in this study: supra-nanohybrid (Estelite Asteria), nanohybrid (GrandioSo), and nanoceramic composite resins (Ceram-X Spheretec One). Forty-eight disc-shaped specimens (4 mm in diameter, 2 mm in thickness) were fabricated using a Teflon mold and divided into four groups according to the different polishing systems (n=12). All specimens were processed with one of the following methods: Mylar strip (control), one-step polishers (Opti1step), two-step polishers (Clearfil TwistDia), or multistep polishers (Sof-Lex XT Pop-on). The surface roughness (Ra, µm) was measured by contact profilometry (Mahr, Marsurf PS1) (n=10) and scanning electron microscopy (SEM) (Thermo Fisher Scientific, Phenom XL) at 400× magnification (n=2). The data were statistically analyzed using Kruskal-Wallis and Bonferroni correction tests (p<0.05). In addition, the surface morphology and elemental content were examined by SEM and energy dispersive x-ray spectroscopy (EDS) analyses. Under SEM evaluation, in terms of the polishing systems, there were no significant differences in the surface roughness for supra-nanohybrid composite resin (p>0.05). The multistep polishers created lower surface roughness than the one-step polishers for nanohybrid and nanoceramic composites. In terms of the composite resins, supra-nanohybrid composite exhibited lower surface roughness than nanohybrid composite for all polishing systems (p<0.05). The SEM observations confirmed the surface roughness measurements related to the surface morphology. One-step and two-step polishers created porosity on the surface of nanohybrid and nanoceramic composites. EDS analysis indicated the elemental composition of the particles in the porous zones was quite close to diamond abrasives and glass fillers.
Assuntos
Polimento Dentário , Nanocompostos , Resinas Compostas , Diamante , Teste de Materiais , Microscopia Eletrônica de Varredura , Propriedades de SuperfícieRESUMO
OBJECTIVE: The purpose of this study was to determine the fracture strength of endodontically treated mandibular premolar teeth restored with composites and different reinforcement techniques. METHODS AND MATERIALS: Forty-eight freshly extracted human mandibular premolar teeth were randomly divided into four groups: group IN, group CR, group FRC, and group PRF. Group IN consisted of teeth with intact crowns and served as the control group. In the other three groups, endodontic treatment was performed and standard mesio-occluso-distal (MOD) cavities were prepared. Then cavities were restored with hybrid resin composite only, flowable composite and hybrid resin composite, and Ribbond, flowable composite and hybrid resin composite in groups CR, FRC and PRF, respectively. All of the teeth were subjected to fracture by means of a universal testing machine, and compressive force was applied with a modified stainless-steel ball at a crosshead speed at 0.5 mm/min. RESULTS: The highest values were observed in group IN, while the lowest values were determined in group CR. There was not any statistically significant difference between group CR and group FCR ( p>0.05). When groups CR, FCR, and PRF were compared, group PRF showed significantly better fracture strength than did groups CR and FCR ( p<0.05). It was determined that there was not any significant difference between group IN and group PRF ( p>0.05). CONCLUSIONS: Polyethylene ribbon fiber considerably increases the fracture strength of mandibular premolar teeth with MOD cavities restored with composite.
Assuntos
Resinas Compostas/química , Restauração Dentária Permanente/métodos , Fraturas dos Dentes/prevenção & controle , Dente não Vital , Dente Pré-Molar , Força Compressiva , Preparo da Cavidade Dentária , Materiais Dentários/química , Análise do Estresse Dentário , Humanos , Técnicas In Vitro , Mandíbula , Teste de Materiais , Polietilenos , Distribuição AleatóriaRESUMO
OBJECTIVES: To investigate the role of shared epitope (SE) alleles in the short-term clinical response to leflunomide for the treatment of active RA. METHODS: In an open-label, multi-centre study of 16-weeks duration, 93 patients (82% female) fulfilling ARA 1987 RA criteria were treated with leflunomide (100 mg loading dose for 3 days, then 20 mg/day as the maintenance dose). The primary efficacy criterion was the response status according to the European League Against Rheumatism (EULAR) response criteria using Disease Activity Score-28 (DAS28) activity measure. SE determinations have been undertaken by polymerase chain reaction and sequence-specific oligonucleotide genotyping methods. RESULTS: The mean (s.d.) Disease Activity Score-28 (DAS28) was 5.1 (1.3) before the treatment, which was significantly decreased after 16 weeks [3.0 (1.1), P < 0.001]. According to the EULAR response criteria, 55 patients (59.1%) were classified as good responders. SE was positive in 51 (54.8%) of the patients, with 13 (13.9%) having SE homozygosity or carrying any two SE alleles. Among SE-positive patients, 68.6% (35/51) were good responders, compared with 47.6% (20/42) in SE negatives (P = 0.04). No difference was present according to SE hetero- or homozygosity (68.4 vs 69.2%). RF was also present significantly more frequently in the SE-positive group compared with negatives (78.4 vs 57.1%, P = 0.03). However, no significant difference was observed in the prevalence of RF positivity in patients with a good clinical response (72.7 vs 63.2%, P = 0.32). CONCLUSIONS: The results suggest that HLA-DRB1 SE presence may favourably affect the outcome of leflunomide monotherapy in an unselected group of RA patients with an active disease and naive to leflunomide.
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Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/genética , Antígenos HLA-DR/genética , Isoxazóis/administração & dosagem , Adulto , Alelos , Artrite Reumatoide/imunologia , Biomarcadores/análise , Relação Dose-Resposta a Droga , Esquema de Medicação , Epitopos , Feminino , Seguimentos , Antígenos HLA-DR/análise , Cadeias HLA-DRB1 , Humanos , Leflunomida , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Medição de Risco , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
Our aim is to assess the prevalence and associated clinical features of anti-CCP (cyclic citrullinated peptide) antibodies for RF (rheumatoid factor)-positive and RF-negative rheumatoid arthritis (RA) and psoriatic arthritis (PsA). In a prospective, cross-sectional, multi-centre study, we determined the titres of anti-CCP antibodies in 208 RA patients (129 RF-positive, 79 RF-negative), 56 PsA patients and 39 healthy controls (HC). Clinical parameters including disease activity (disease activity score 28-DAS28), physical disability (health assessment questionnaire-HAQ), functional capacity (functional class) and radiological erosions were investigated in patients with RA. In PsA patients, clinical and radiological features were determined. Anti-CCP2 antibodies were measured using a second-generation anti-CCP enzyme-linked immunosorbent assay (Euro-Diagnostica, Netherlands). One-hundred four of 129 RF-positive RA (81%), 16 of 79 RF-negative RA (20%), seven of 56 PsA patients (12.5%) and none of the HC had anti-CCP antibodies. RA patients with anti-CCP antibodies had significantly higher disease activity, greater loss of function and more frequent erosive disease than anti-CCP antibody-negative group. In subgroup analysis, anti-CCP antibodies in RF-negative patients were also associated with erosive disease. All PsA patients with anti-CCP antibodies had symmetric arthritis with higher number of swollen joints. The prevalence of anti-CCP antibodies in RF-positive RA patients was significantly higher than in RF-negative RA and PsA patients. Anti-CCP antibodies were also associated with erosive disease in RF-negative RA patients. Both anti-CCP and RF tests were negative in 30% of the patients. Anti-CCP positivity was a frequent finding in PsA and associated with symmetrical polyarthritis.
Assuntos
Artrite Psoriásica/imunologia , Artrite Reumatoide/imunologia , Autoanticorpos/sangue , Peptídeos Cíclicos/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Estudos Transversais , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Países Baixos , Estudos Prospectivos , Sensibilidade e EspecificidadeRESUMO
It has been generally accepted that the clinical onset of familial Mediterranean fever (FMF) begins before 20 years of age in most patients. In this study, we aimed to investigate the demographic and clinical characteristics of our FMF patients with an age of onset > or =20. Records of 401 patients (female/male: 204/197) that followed up between 1990 and 1999 were reviewed according to a pre-defined protocol. All patients fulfilled the diagnostic criteria of Livneh et al. The demographic and clinical features of adult-onset FMF patients were compared to those of patients with a disease onset before 20 years of age. There were 57 patients (14%) who experienced symptoms of FMF at > or =20 years of age; 34 of them (8.5%) reported their first attack between 20 and 29 years of age; 18 of them (4.5%) between 30 and 39 years of age and five patients (1.25%) had their first attack after 40 years of age. Arthritis (42 vs. 65%, p = 0.001) and erysipelas-like erythema (7 vs. 17%, p = 0.047) were significantly less frequent in patients with adult-onset FMF compared to patients with disease onset before 20 years of age. Arthritis and erysipelas-like erythema were less frequent in adult-onset patients compared to those with an earlier disease onset. Adult-onset FMF may be a form of disease with distinct clinical, demographic and molecular characteristics. Prospective clinical studies and investigation of genotypic features are needed to identify the characteristics of this phenotypic variant.