Is compressed colchicine tablet superior to other colchicine preparations in patients with familial Mediterranean fever?

AIMS: The aim of our study is to evaluate the differences in effectiveness, dosage, and side effect profiles in the use of colchicine preparations and evaluate the superiority of compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine tablets. MATERIALS AND METHODS: Patients who were diagnosed with FMF according to the Tel Hashomer criteria, aged 18 years and older, and switched from compressed colchicine to coated colchicine tablets in the rheumatology clinic of Gazi University were identified. The daily colchicine dose and FMF attack frequency before and after switching from coated colchicine tablets to compressed colchicine tablets were compared. RESULTS: The study included 43 female (72.9%) and 16 male patients (27.1%), and the mean age was 34.54 ± 8.3 years. The number of attacks per year was significantly reduced after switching to compressed colchicine tablets, and daily colchicine doses were lower after switching to compressed colchicine tablets (1.97 ± 0.23 vs 1.78 ± 0.39 mg, p < 0.001). CONCLUSION: Compressed colchicine tablets were shown to be superior to other colchicine preparations and compressed colchicine tablets to be a useful treatment option before initiating biological agents in patients who were unresponsive to coated colchicine.

Calcineurin inhibitors in unplanned pregnancies with active lupus disease: A retrospective observational study.

OBJECTIVES: To evaluate the use of calcineurin inhibitors (CNIs), specifically tacrolimus, in unplanned pregnancies with active lupus disease among patients with systemic lupus erythematosus (SLE). MATERIALS AND METHODS: The study includes data from pregnancies in women diagnosed with SLE at Gazi University Hospital in Ankara, Türkiye, between January 2010 and July 2022. The study categorized pregnancies into planned and unplanned groups based on lupus nephritis presence, emphasizing the need for inactive lupus disease for at least 6 months before attempting conception in planned pregnancies. The outcomes of pregnancies involving CNIs, particularly tacrolimus, were assessed. RESULTS: In our cohort comprising 632 SLE patients, 39 individuals reported 42 pregnancies. Among the 42 pregnancies, 14 have a history of lupus nephritis. We observed that 8 of 14 patients with a history of lupus nephritis had unplanned pregnancies. Three patients used cyclosporine and 2 used tacrolimus during their pregnancy; their pregnancies were completely healthy, and no lupus flare was observed during their pregnancies. The pregnancy of 2 patients who used azathioprine and 1 last patient who used no immunosuppressive treatment ended in abortion. CONCLUSION: This study reveals that tacrolimus can be effectively used in unplanned pregnancies with active lupus disease, providing favorable maternal and fetal outcomes. The findings emphasize the importance of considering CNIs, particularly tacrolimus, in the management of SLE pregnancies, even in cases of unplanned pregnancies with a history of lupus nephritis.

Effects of interleukin-1 antagonists on de novo and pre-existing damage in patients with familial Mediterranean fever.

OBJECTIVES: Colchicine is the mainstay of familial Mediterranean fever treatment and interleukin (IL-1) antagonists are the treatment of choice in resistant patients. We aimed to investigate efficacy of IL-1 antagonists in the prevention of damage, as well as the causes of treatment failure. METHODS: A total of 111 patients fulfilling Euro fever and Tel-Hashomer criteria and treated with IL-1 antagonists were included in the study. Patients were grouped according to their recent damage status: no damage, pre-existing damage and de novo damage that developed under IL-1 antagonist treatment. The degree of damage was determined using the Auto Inflammatory Disease Damage Index (ADDI). Total damage score was calculated separately as its original definition and with excluding chronic musculoskeletal pain, creating the modified ADDI (mADDI). RESULTS: Forty-six patients (43,2 %) had damage according to the mADDI. Damage was commonly observed at musculoskeletal, renal and reproductive domains. Median duration of treatment was forty-five months. Two patients developed de novo damage: one musculoskeletal and one reproductive in this time-period. Five patients had a worsening of their damage while using IL-1 antagonists. De novo damage with IL-1 antagonist treatment was associated with acute phase protein levels. CONCLUSIONS: We evaluated change in damage accrual while using IL-1 antagonists in patients with FMF. Physicians should pay attention to controlling inflammation to prevent further damage, especially in those with pre-existing damage.

COVID-19 in familial Mediterranean fever: Clinical course and complications related to primary disease.

OBJECTIVES: To evaluate the impact of familial Mediterranean fever (FMF) features on the clinical course and outcomes of coronavirus disease 2019 (COVID-19) and clinical course of FMF after COVID-19. METHODS: Consecutive FMF patients with COVID-19 were enrolled from three referral hospitals. Clinical features of FMF and detailed COVID-19 information were obtained from patient interviews and medical records. RESULTS: Seventy-three FMF patients were included in the study. 94.5% of patients had clinical symptoms of COVID-19. We found 24.7% hospitalization, 12.3% respiratory support, 4.1% intensive care unit admission, 6.8% complication, and 1.4% mortality rate in patients. The risk factors of hospitalization for respiratory support were male gender [OR: 7.167 (95% CI: 1.368-37.535)], greater age [OR: 1.067 (95% CI: 1.016-1.121)], and non-adherence to colchicine treatment before the infection [OR: 7.5 (95% CI: 1.348-41.722)]. One-third of patients had reported attacks after COVID-19. The patterns of triggered attacks were fever, peritonitis, pleuritis, transient arthritis, chronic knee mono-arthritis, and protracted febrile myalgia. CONCLUSIONS: FMF characteristics were not associated with worse outcomes of COVID-19. Colchicine non-adherence was the risk factor of hospitalization for oxygen support. The rate of FMF attacks after COVID-19 is prominently increased, with some of them being protracted and destructive.

Factors affecting patient-acceptable symptom states and treatment decision in familial Mediterranean fever.

BACKGROUND: Familial Mediterranean fever [FMF] is the most common autoinflammatory disease characterized by inflammatory attacks of fever and polyserositis. Patients' quality of life is significantly affected due to recurrent excruciating pain attacks and complications. This study is performed to evaluate the parameters most affecting patients' satisfaction from treatment. METHODS: : Three hundred and forty-six consecutive patients diagnosed with FMF were enrolled in this study. Current treatment, acute phase proteins, number, type, and severity of predominant attacks, absenteeism from work/school in the last three months were recorded, and the participants were asked whether they needed additional treatment to evaluate Patient Acceptable Symptom State (PASS) status. RESULTS: Mean age of the overall group was 38.2 ± 11.7 years (62.4% female, 37.6% male). Two hundred and twenty-seven patients were treated with colchicine, 97 patients with colchicine plus Interleukin-1 (IL-1) antagonist, and 22 only with IL-1 antagonist (67.1%, 26.3%, 6.64% in order). Of the overall group, 33.8% (n = 117) believed to need additional treatment options. Additional treatment need of patients was significantly affected by work impairment due to attacks, absent days from work, disease activity, the discomfort of patients during attacks, the number of attacks, and treatment options; but not by the level of acute-phase proteins between attacks. DISCUSSION: PASS score is significantly related to clinical parameters and quality of life. Patients' PASS scores and treatment choices are notably affected by the severity and frequency of attacks and absenteeism from work/school. Clinical activity and quality of life should be evaluated at every visit to provide patients' satisfaction with treatment.

Comparison of Lateral Tooth Volume and Morphology Between Buccally and Palatally Localised Unilateral Impacted Maxillary Canine Cases on Cone-Beam Computed Tomography.

ABSTRACT: The aim of this study was to compare the lateral tooth volume and morphology differences between buccally and palatally localized unilateral impacted maxillary canine (IMC) patients with volumetric and linear measurements on cone-beam computed tomography (CBCT) records. CBCT records of 48 individuals with 24-buccally and 24-palatally localized unilateral IMCs were included to the study. The patients comprised 34 females, 14 males, with a mean age of 17.71 (±SD 6.63 years). This split-mouth, retrospective case-control, study consisted of buccally and palatally IMC group, and each group was categorized into 2 sub-groups as impacted and erupted side. Lateral tooth volume, tooth length, mesiodistal-buccolingual crown widths, mesiodistal- buccolingual root widths at cemento-enamel junction (CEJ), at 4 mm apical to CEJ, at 8 mm apical to CEJ, mesiodistal crown-root angle, arch perimeter measurements were performed on CBCT images. The data were analyzed by factorial design repeated measures ANOVA. Bonferroni test was used for post-hoc analysis. The total volume, mesiodistal - buccolingual crown widths, buccolingual root width of lateral incisor at CEJ were statistically smaller in palatally IMC group. The lateral incisor's length, and arch perimeter were statistically shorter, mesiodistal, buccolingual root widths at 8 mm apical to CEJ, and crown-root angle of the lateral were narrower on impacted side than erupted side. The volume and crown widths of lateral incisor are effective in sagittal localization of IMC. The shorter length, narrower apical root morphology and mesially inclined crown-root angle of lateral incisor are noteworthy on impacted sides, regardless of sagittal position of IMC.

Validation of cervical vertebral maturation stages: Artificial intelligence vs human observer visual analysis.

INTRODUCTION: This study aimed to develop an artificial neural network (ANN) model for cervical vertebral maturation (CVM) analysis and validate the model's output with the results of human observers. METHODS: A total of 647 lateral cephalograms were selected from patients with 10-30 years of chronological age (mean ± standard deviation, 15.36 ± 4.13 years). New software with a decision support system was developed for manual labeling of the dataset. A total of 26 points were marked on each radiograph. The CVM stages were saved on the basis of the final decision of the observer. Fifty-four image features were saved in text format. A new subset of 72 radiographs was created according to the classification result, and these 72 radiographs were visually evaluated by 4 observers. Weighted kappa (wκ) and Cohen's kappa (cκ) coefficients and percentage agreement were calculated to evaluate the compatibility of the results. RESULTS: Intraobserver agreement ranges were as follows: wκ = 0.92-0.98, cκ = 0.65-0.85, and 70.8%-87.5%. Interobserver agreement ranges were as follows: wκ = 0.76-0.92, cκ = 0.4-0.65, and 50%-72.2%. Agreement between the ANN model and observers 1, 2, 3, and 4 were as follows: wκ = 0.85 (cκ = 0.52, 59.7%), wκ = 0.8 (cκ = 0.4, 50%), wκ = 0.87 (cκ = 0.55, 62.5%), and wκ = 0.91 (cκ = 0.53, 61.1%), respectively (P <0.001). An average of 58.3% agreement was observed between the ANN model and the human observers. CONCLUSIONS: This study demonstrated that the developed ANN model performed close to, if not better than, human observers in CVM analysis. By generating new algorithms, automatic classification of CVM with artificial intelligence may replace conventional evaluation methods used in the future.

Bioaccumulation of thallium by the wild plants grown in soils of mining area.

Gümüsköy Ag (As, Pb, and Tl) deposits are one of the largest silver deposits in the country and located about 25 km west of Kütahya, Turkey. This study investigated the accumulation and transport of thallium into 11 wild plants in soil of the mining area. Plant samples and their associated soils were collected from the field and Tl contents were measured with inductively coupled plasma mass spectroscopy (ICP-MS). The mean concentrations in the soil, roots, and shoots of the studied plants were, respectively, 170, 318, and 315 mg kg(-1) for Tl. The plants analyzed and collected from the studied area were separated into different groups based on enrichment coefficients of roots and shoots (ECR and ECS). The results showed that because of their higher ECR and ECS, the following could be good bioaccumulators: CY, IS, SL, and VR for Tl. Therefore, these plants can be useful for remediation or phytoremediation of soils polluted by Tl.

Mercury uptake and phytotoxicity in terrestrial plants grown naturally in the Gumuskoy (Kutahya) mining area, Turkey.

This study investigated mercury (Hg) uptake and transport from the soil to different plant parts by documenting the distribution and accumulation of Hg in the roots and shoots of 12 terrestrial plant species, all of which grow naturally in surface soils of the Gumuskoy Pb-Ag mining area. Plant samples and their associated soils were collected and analyzed for Hg content by ICP-MS. Mean Hg values in the soils, roots, and shoots of all plants were 6.914, 460, and 206 µg kg(-1), respectively and lower than 1. The mean enrichment factors for the roots (ECR) and shoots (ECS) of these plants were 0.06 and 0.09, respectively and lower than 1. These results show that the roots of the studied plants prevented Hg from reaching the aerial parts of the plants. The mean translocation factor (TLF) was 1.29 and higher than 1. The mean TLF values indicated that all 12 plant species had the ability to transfer Hg from the roots to the shoots but that transfer was more efficient in plants with higher ECR and ECS. Therefore, these plants could be useful for the biomonitoring of environmental pollution and for rehabilitating areas contaminated by Hg.

Location of facial foramina and mandibular angle from cone beam computed tomographic scans.

PURPOSE: The current study's purpose was to determine morphometric analysis of all facial foramina and mandibular angle relative to surgical landmarks from cone beam computed tomographic scans. MATERIALS AND METHODS: Three-dimensional computed tomographic scans were reconstructed from data of 100 patients (200 sides) aged between 19 and 76 years. Morphometric measurements of all facial foramina relative to surgical landmarks were taken. Mandibular angle was measured. RESULTS: There was no statistically significant difference between the left and right sides for all parameters (P > 0.05). Therefore, we found bilateral symmetry in the position of all facial foramina and mandibular angle. However, statistically significant differences were determined in sexes in some of these parameters and mandibular angle. CONCLUSIONS: The knowledge about locations of facial foramina and mandibular angle is important for performing local nerve block and surgery in the face to avoid the neurovascular structures. This study provides a guideline for locations of facial foramina and mandibular angle, which may help surgeons to understand the nerve location precisely during surgery.

Three-dimensional anatomic analysis of the lingula and mandibular foramen: a cone beam computed tomography study.

PURPOSE: The authors analyzed the anatomic location differences of the mandibular foramen (MF) and lingula in a cone beam computed tomography study, aiming to obtain information that could be used when performing mandibular osteotomies and the inferior alveolar nerve block (IANB). METHODS: Three-dimensional mandibular computed tomography images were reconstructed from data for 139 patients (278 sides) aged between 9 and 18 years (growth group, 27 patients) and aged 19 to 71 years (adult group, 112 patients). RESULTS: In the adult group, positive correlations were seen between right and left measurements. In the growth group, there are significant differences in lingula-anterior and MF-posterior ramus measurements. In the adult group, there are significant differences between man and woman MF-gonion distance measurements. Differences were seen in edentulous and asymmetry patients. CONCLUSIONS: The MF is an important anatomic landmark for ramus surgery and IANB. When applied to ramus operations and IANB, the anatomic data provided by this study may help surgeons gain more understanding of nerve position during surgery.

Investigation of sensory and motor functions of wrist in patients with rheumatoid arthritis.

BACKGROUND: Rheumatoid arthritis (RA) is a systemic and autoimmune disease that affects many tissues and organs in the body, especially the synovial joint and periarticular structures. One of the most affected joints is the hand joints, and there is also involvement in the wrist joint. OBJECTIVES: The purpose of this study was to assess the sensory and motor functions of the wrist with a gold standard method and to determine the relationship between the motor and sensory functions of the wrist and wrist-related pain/disability level and hand grip strength in patients with RA. METHOD: Flexor and extensor muscle strength and joint position sense of the wrist were evaluated using isokinetic dynamometer, hand grip strength was measured using dynamometer, and wrist related pain and disability was assessed by Patient Rated Wrist Evaluation (PRWE) questionnaire. RESULTS: Thirty-one patients with RA and 31 healthy controls were included in the study. Control group had better results regarding wrist flexor and extensor muscle strength and wrist joint position sense than RA patients for both hands/wrists. A positive and strong correlation and a negative and weak correlation were found between muscle strength and joint position sense of the wrist and hand grip strength, respectively. CONCLUSIONS: Compared to healthy subjects, RA patients showed wrist-related sensorimotor deficits. Therefore, evaluation of the wrist should not be ignored in the examination of patients with RA. In addition, when planning the treatment program of the patients, not only hand exercises but also exercises involving the wrist should be taken into consideration.

Vascular calcification in Takayasu arteritis: relationship with metabolic syndrome, left ventricular mass index, and intima-media thickness.

OBJECTIVE: The present study aims to determine the frequency of vascular calcification in Takayasu arteritis (TA) and the risk factors for it and to evaluate its relation with atherosclerotic predictors such as metabolic syndrome (MS), left ventricular mass index (LVMI) and carotid intima-media thickness (CIMT). METHODS: A cross-sectional study was conducted in patients with TA; MS was defined according to the US National Cholesterol Education Program/Adult Treatment Panel III (NCEP/ATP III) criteria. The study included 49 TA patients (22 with MS, 27 without MS) and 31 healthy controls (HCs). Non-contrast computed tomography measured calcification in coronary arteries, aorta, and branches. RESULTS: Forty-seven patients (95.9%) were female and mean age was 33.45 ± 8.53 years. Total calcification score (mean ± SD; 5223.9 ± 18041.1 AU vs. 35.87 ± 72.70 AU (p = 0.05)), CIMT, and LVMI were found to be significantly higher in TA patients than HCs (p < 0.05). While there was no significant difference between the total calcification score of MS (+) TA patients and MS (-) TA patients, in both patient groups, the total calcification score was found to be significantly higher than HCs. MS (+) and MS (-) groups were found to have significantly higher CIMT and LVMI values than the control group, in addition, MS (+) patients were found to have significantly higher LVMI and CIMT values than MS (-) group (p < 0.05). CONCLUSION: Vascular calcification, CIMT, and LVMI are elevated in all TA patients, with greater impact in the presence of MS.

Assessment and comparison of the 2023 ACR/EULAR APS criteria with the revised Sapporo criteria.

OBJECTIVE: To analyze antiphospholipid antibody (aPL)-positive patients using the 2023 American College of Rheumatology/The European Alliance of Associations for Rheumatology (ACR/EULAR) antiphospholipid syndrome (APS) classification criteria and compare the revised Sapporo criteria and the 2023 ACR/EULAR criteria and evaluate whether the 2023 ACR/EULAR criteria provide added value over the revised Sapporo criteria. METHODS: In this descriptive study, 94 aPL-positive patients (with or without APS diagnosis) were identified from two hospital-based registries (Gazi and Hacettepe University). Patients were classified into four groups to compare both criteria sets. These four groups are as follows: (1) patients classified with only the revised Sapporo criteria; (2) patients classified with only the 2023 ACR/EULAR APS criteria; (3) patients classified with both two criteria sets; and (4) patients classified with neither two criteria set. RESULTS: Of the 94 patients, 11 were classified with only the revised Sapporo criteria; one with only the 2023 ACR/EULAR APS criteria; 52 with both criteria sets; and 30 with neither set of criteria. For these 94 patients, the operating characteristics of the 2023 ACR/EULAR APS criteria, using the revised Sapporo criteria as the gold standard, the 2023 ACR/EULAR APS entry criteria demonstrated 100% sensitivity, and the 2023 ACR/EULAR APS classification criteria demonstrated 98% specificity and 82.5% sensitivity. CONCLUSION: The study emphasizes the importance of recognizing differences in clinical manifestations, such as early pregnancy loss without severe preeclampsia (PEC) and/or severe placental insufficiency (PI) and calls for a nuanced discussion on anticardiolipin (aCL) and anti-beta 2-glycoprotein-I (anti-ß2GPI) immunoglobulin G (IgG) cutoff values.

Coronavirus Disease 2019 Outcomes in Amyloid A Protein Amyloidosis Secondary to Rheumatic Conditions and Signs of Post- Coronavirus Disease 2019 Proteinuria Progression.

BACKGROUND: We aimed to investigate coronavirus diease 2019 (COVID-19) outcomes in patients with amyloid A protein (AA) amyloidosis secondary to rheumatic diseases and discuss factors associated with disease course. METHODS: A retrospective cohort was formed from adult patients with a diagnosis of AA amyloidosis. In patients with a positive severe acute respiratory syndrome coronavirus 2 polymerase chain reaction (PCR) test, rates of hospitalization, intensive care unit admission and mortality due to COVID-19 were collected from medical records. Data regarding to demographics, comorbidities, laboratory tests, medical treatments, adherence to previous treatments during COVID-19 and treatment administered for COVID-19 were collected from hospital databases and patient reviews. RESULTS: In 96 patients with AA amyloidosis, 16 had COVID-19 with a positive PCR. Ten (62.5%) patients were hospitalized, 2 (12.5%) were admitted to ICU, 1 (6.25%) was died. Hospitalized patients tended to be older. Comorbidities seemed to be more frequent in hospitalized patients. None of the patients had rapid progression to end-stage renal disease post-COVID-19. Seven patients had pre-COVID-19 and post-COVID-19 proteinuria levels. Three had notable increase in proteinuria after COVID-19 in 2 of which amyloidosis treatment was revised accordingly. CONCLUSION: Despite high rates of hospitalization in AA amyloidosis patients, mortality was observed only in 1 patient. Progression of proteinuria requiring treatment adjustment may be an issue in these patients. Cite this article as: Güven SC, Erden A, Küçük H, et al. Coronavirus disease 2019 outcomes in amyloid A protein amyloidosis secondary to rheumatic conditions and signs of post-coronavirus disease 2019 proteinuria progression. Eur J Rheumatol. Published online April 4, 2024. DOI:10.5152/eurjrheum.2024.23050.

Colchicine-intolerant familial mediterranean fever patients: A comparative study between different colchicine doses and IL-1 inhibitor monotherapy.

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Colchicine is the primary treatment for FMF, although some patients do not respond well or are unable to tolerate it. For these patients, the addition of interleukin-1 (IL-1) antagonists is the preferred option. However, the impact of colchicine treatment alongside the use of IL-1 antagonists remains unclear. METHODS: We recruited adult FMF patients who satisfied the Eurofever and Pediatric Rheumatology International Trials Organization classification criteria and were receiving IL-1 antagonist treatment from our FMF cohort. All the patients exhibited colchicine intolerance or resistance. As per the FMF cohort protocol, the patients were longitudinally followed up, including assessments of their C-reactive protein, erythrocyte sedimentation rate, autoinflammatory disease activity index (AIDAI), and autoinflammatory damage index (ADDI). RESULTS: Among the 125 patients (68 female and 57 male), 96 received a combination of IL-1 antagonists and the maximum tolerated dose of colchicine, whereas 29 were treated exclusively with IL-1 antagonists due to colchicine intolerance. The patients' inflammatory markers, AIDAI activity, and ADDI damage scores did not differ significantly between the two groups during the follow-up period. Notably, the drug retention rates were significantly higher in the patients treated solely with IL-1 antagonists. CONCLUSION: While the typical approach is to maintain colchicine treatment alongside the use of IL-1 antagonists, for patients who cannot tolerate effective colchicine doses, IL-1 antagonists alone may effectively control FMF disease activity.

Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry.

VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03-5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up.

Risk for cancer development in familial Mediterranean fever and associated predisposing factors: an ambidirectional cohort study from the international AIDA Network registries.

Objective: Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF. Methods: The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still's disease patients and Behçet's disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression. Results: 580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet's disease patients and 497 Still's disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p=0.10) in FMF compared to Still's disease and 0.60 (95% CI. 0.29-1.28, p=0.18) in FMF compared to Behçet's disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (ß1 = 0.039, 95% CI. 0.001-0.071, p=0.02), the age at the diagnosis (ß1 = 0.048, 95% CI. 0.039-0.085, p=0.006), the age at the enrolment (ß1 = 0.05, 95% CI. 0.007-0.068, p=0.01), the number of attacks per year (ß1 = 0.011, 95% CI. 0.001- 0.019, p=0.008), the use of biotechnological agents (ß1 = 1.77, 95% CI. 0.43-3.19, p=0.009), the use of anti-IL-1 agents (ß1 = 2.089, 95% CI. 0.7-3.5, p=0.002). Conclusions: The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.

Impact of HLA-B51 on Uveitis and Retinal Vasculitis: Data from the AIDA International Network Registries on Ocular Inflammatory Disorders.

PURPOSE: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown. METHODS: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time. RESULTS: Records of 213 patients (341 eyes) were analyzed. No differences in complications were observed (p = 0.465). With regard to VA, a significant difference was detected in median BCVA (p = 0.046), which was not maintained after Bonferroni correction (p = 0.060). RV was significantly more prevalent in NIU-affected patients who tested positive for HLA-B51, irrespective of the systemic diagnosis of BD (p = 0.025). No differences emerged in the occurrence of macular edema (p = 0.99). CONCLUSIONS: Patients with NIU testing positive for HLA-B51 exhibit an increased likelihood of RV throughout disease course, irrespective of a systemic diagnosis of BD. The rate of complications as well as VA are comparable between NIU cases unrelated to BD testing positive for HLA-B51 and uveitis associated with BD. Therefore, it is advisable to perform the HLA-B typing in patients with NIU or retinal vasculitis, even in the absence of typical BD features.

Dentofacial characteristics of patients with rheumatoid arthritis.

OBJECTIVES: The aims of this study are to evaluate the dentofacial morphology of patients with rheumatoid arthritis (RA) and to compare the morphological data with those of healthy age- and sex-matched control subjects. METHODS: Twenty-seven RA patients (mean age, 45.77 ± 8.64 years) and 25 healthy subjects (mean age, 44.80 ± 8.24 years) participated in this prospective study. Clinical and functional evaluations of the RA patients were assessed. The erythrocyte sedimentation rate, C-reactive protein level, rheumatoid factor level, and anti-citrullinated peptide antibodies (ACPA) titers of RA patients were determined, and DAS28 scores were calculated. Linear and angular measurements were performed on cephalometric tracings and condylar erosion was evaluated on lateral panoramic radiographs. Statistical comparison of the two groups was performed with an independent samples t test. Pearson correlation analysis was used to assess the relationship between the clinical and laboratory parameters. RESULTS: Based on DAS28 scores, no patient with RA was in the remission period, 3 patients had low, 23 had medium, and 1 had high disease activity. Sixteen (59.26 %) patients with RA had positive ACPA titers. Lateral cephalometric radiographs revealed statistically significant difference between the two groups for the measurement of U1-NA (millimeter; p = 0.047), U1-NA (degrees; p = 0.031), L1-NB (degrees; p = 0.030), IMPA (L1-MP; p = 0.001), interincisal angle (U1-L1; degrees; p = 0.022) and midface length (Co-A; millimeter; p = 0.033). A significant positive linear correlation was found between disease duration time and DAS28 scores (r = 0.066, p = 0.040). CONCLUSIONS: Dentoalveolar effects of RA on dentofacial morphology are more significant than the skeletal effects. Future studies with larger sample sizes are required to evaluate the exact effects of RA on dentofacial morphology. CLINICAL RELEVANCE: Clinicians should consider the fact that RA-associated dentoalveolar changes can be observed and may affect the orthodontic treatment process.