Importance of hematological markers in familial Mediterranean fever in terms of disease severity and amyloidosis.

There are limited follow-up parameters for familial Mediterranean fever (FMF) related to disease severity and amyloidosis. Some hematological markers are emerging to assess inflammation. In this study, we hypothesized that some hematological parameters could be used to determine disease severity and amyloidosis in FMF. We included 274 adult FMF patients, and evaluated the relationship between neutrophil lymphocyte ratio (NLR), monocyte-lymphocyte ratio (MLR) and platelet-lymphocyte ratio (PLR), platelet counts and leukocyte counts, mean erythrocyte haemoglobin concentration (MCHC) and mean erythrocyte haemoglobin (MCH) with disease severity and amyloidosis. First, we classified patients according to disease severity and presence of amyloidosis. We then compared the parameters within the groups. In addition, we determined predictive cut-off values with ROC analysis. Finally, we correlated the change in ISSF scores with the change in hematological parameters of 52 patients with follow-up hematological indices after six months. The patients with severe-moderate group had higher CRP levels (p < 0.001), white blood cell (p = 0.002) and neutrophil counts (p = 0.004) and, conversely, lower MCHC levels (p = 0.001) than patients with mild disease severity. FMF patients with amyloidosis had higher neutrophil (p = 0.04) and monocyte count (p = 0.02), increased NLR (p = 0.01) and lower MLR (p = 0.02) levels than those without. In addition, MCHC levels were also lower in the severe-moderate group in the follow-up analyses after sixth months (p = 0.03). MCHC, neutrophil and monocyte counts, NLR, MLR may be associated with poor prognosis in FMF patients. These parameters can be used in conjunction with acute phase reactant and clinical features to assess disease status.

Apical fibrosis was the most common incidental pulmonary finding in a familial Mediterranean fever cohort.

INTRODUCTION: Familial Mediterranean fever (FMF) is one of the common autoinflammatory diseases with multisystemic manifestation. Pleuritis is the only known pulmonary involvement of FMF; however, as far as we know, thoracic involvements in pleural, parenchymal, bronchial, and vascular structures have not been evaluated yet. METHOD: We included 243 consecutive FMF patients who applied to our clinic within the last 5 years and were requested to have a thorax CT for any reason and 122 trauma patients without any comorbidity. An experienced radiologist evaluated the thorax CT images blindly according to the relevant guidelines. We then presented the common incidental pulmonary and mediastinal findings on the thorax CT. Additionally, we compared patients with and without lung involvement according to demographic and disease-related parameters. RESULTS: In our study, 167 of 243 patients (68.7%) had at least one of the pulmonary findings on their thorax CT. The most common pulmonary findings were apical fibrosis in 96 (39.5%) patients, parenchymal fibrotic changes in 48 (19.8%) patients, and a solitary parenchymal nodule smaller than 4 mm in 33 (13.6%) patients. All demographic, genetic, and disease-related characteristics, including the frequency of spondyloarthropathy, were similar in patients with and without pulmonary findings. CONCLUSIONS: We showed that the most common incidental pulmonary finding in our FMF cohort was apical fibrosis on thoracic CT. Our data did not show causality between FMF and apical fibrosis; therefore, more studies are needed to evaluate the frequency and clinical significance of apical fibrosis in FMF. Key Points • More than two-thirds of familial Mediterranean fever (FMF) patients in our study group who underwent a thoracic scan for any reason had pulmonary and mediastinal findings on thorax computed tomography (CT). • In our FMF cohort, the most common incidental pulmonary finding on their thorax CT was apical fibrosis. • All demographic and disease-related characteristics, including the frequency of spondyloarthritis, were similar between patients with and without pulmonary and mediastinal findings.

Familial mediterranean fever patients may have unmet needs for the treatments of exertional leg pain and enthesitis.

INTRODUCTION: Exertional leg pain (ELP) and enthesitis are musculoskeletal findings in familial Mediterranean fever (FMF). They are not accepted as principal treatment targets. In this study, we assessed the effectiveness of treatments on ELP and enthesitis. MATERIAL AND METHODS: We have included 218 FMF patients to the study. We retrospectively compared the FMF attacks' frequency, duration and intensity (FMF attack VAS score) and levels of ELP VAS and enthesitis VAS scores between pre-treatment stage and while patients were on treatment at the last visit. RESULTS: Forty-nine (22.5%) and 52 (23.9%) of the patients had enthesitis and ELP respectively. All patients were on colchicine treatment. Serositis attacks respond the treatments significantly. Moreover, both ELP VAS scores (p=0.002) and enthesis VAS scores (p=0.17) were improved with treatment. But only improvement in ELP VAS scores was significant. CONCLUSION: FMF treatments had favourable effect on ELP and enthesitis in FMF patients. However, the response rates would be inadequate. Therefore, there would be unmet need for treatment of both conditions.

Familial mediterranean fever patients may have unmet needs for the treatments of exertional leg pain and enthesitis / Los pacientes con fiebre mediterránea familiar podrían tener necesidades no satisfechas de tratamiento del dolor en piernas con el esfuerzo y entesitis

Introduction: Exertional leg pain (ELP) and enthesitis are musculoskeletal findings in familial Mediterranean fever (FMF). They are not accepted as principal treatment targets. In this study, we assessed the effectiveness of treatments on ELP and enthesitis. Material and methods: We have included 218 FMF patients to the study. We retrospectively compared the FMF attacks’ frequency, duration and intensity (FMF attack VAS score) and levels of ELP VAS and enthesitis VAS scores between pre-treatment stage and while patients were on treatment at the last visit. Results: Forty-nine (22.5%) and 52 (23.9%) of the patients had enthesitis and ELP respectively. All patients were on colchicine treatment. Serositis attacks respond the treatments significantly. Moreover, both ELP VAS scores (p=0.002) and enthesis VAS scores (p=0.17) were improved with treatment. But only improvement in ELP VAS scores was significant. Conclusion: FMF treatments had favourable effect on ELP and enthesitis in FMF patients. However, the response rates would be inadequate. Therefore, there would be unmet need for treatment of both conditions.(AU)

Introducción: El dolor en piernas con el esfuerzo (ELP) y la entesitis son hallazgos musculoesqueléticos en la fiebre mediterránea familiar (FMF), no aceptados como dianas de tratamiento principales. En este estudio evaluamos la efectividad de los tratamientos para ELP y entesitis. Material y métodos: Incluimos en el estudio a 218 pacientes con FMF. Comparamos retrospectivamente la frecuencia de los ataques de FMF, su duración e intensidad (escala analógica visual [VAS] del ataque de FMF) y los niveles VAS para ELP y las puntuaciones VAS para entesitis entre la etapa previa al tratamiento y la etapa en que los pacientes estaban siendo tratados en la última visita. Resultados: Cuarenta y nueve (22,5%) y 52 (23,9%) pacientes tuvieron entesitis y ELP, respectivamente. Todos los pacientes recibieron colchicina. Los ataques de serositis respondieron significativamente a los tratamientos. Además, tanto las puntuaciones VAS para ELP (p=0,002) como para entesis (p=0,17) mejoraron con el tratamiento, pero únicamente fueron significativas las puntuaciones VAS para ELP. Conclusión: Los tratamientos para FMF tuvieron un efecto favorable para ELP y entesitis en los pacientes con FMF. Sin embargo, las tasas de respuesta serían inadecuadas. Por tanto, existiría una necesidad no satisfecha de tratamiento de ambas situaciones.(AU)

Familial Mediterranean Fever Patients may have Unmet Needs for the Treatments of Exertional Leg Pain and Enthesitis.

INTRODUCTION: Exertional leg pain (ELP) and enthesitis are musculoskeletal findings in familial Mediterranean fever (FMF). They are not accepted as principal treatment targets. In this study, we assessed the effectiveness of treatments on ELP and enthesitis. MATERIAL AND METHODS: We have included 218 FMF patients to the study. We retrospectively compared the FMF attacks' frequency, duration and intensity (FMF attack VAS score) and levels of ELP VAS and enthesitis VAS scores between pre-treatment stage and while patients were on treatment at the last visit. RESULTS: Forty-nine (22.5%) and 52 (23.9%) of the patients had enthesitis and ELP respectively. All patients were on colchicine treatment. Serositis attacks respond the treatments significantly. Moreover, both ELP VAS scores (p=0.002) and enthesis VAS scores (p=0.17) were improved with treatment. But only improvement in ELP VAS scores was significant. CONCLUSION: FMF treatments had favourable effect on ELP and enthesitis in FMF patients. However, the response rates would be inadequate. Therefore, there would be unmet need for treatment of both conditions.

Enthesitis may be one of the signs of severe disease in familial Mediterranean fever.

OBJECTIVE: Familial Mediterranean fever (FMF) is an auto-inflammatory disease that is also characterized with some of the common musculoskeletal features of spondyloarthritis (SpA). Enthesitis is the hallmark of SpA. Recently, it was postulated that exertional leg pain is a possible sign of lower extremity enthesitis associated with FMF severity. In this study, we have evaluated the association between the enthesitis, enthesitis score and disease severity in FMF patients. METHODS: We enrolled 238 FMF patients that fulfilled the modified Tel-Hashomer criteria. We assessed the presence of enthesitis at the Maastricht Ankylosing Spondylitis Enthesitis Score (MASES) defined sites with standard palpation method. Then, FMF patients dichotomised two groups as enthesitis group and controls. Herein, we evaluated the enthesis extensity with MASES. FMF disease severity was determined via the international severity scoring system for FMF (ISSF). Firstly, we have compared demographic properties, disease-related features and ISSF scores of the groups. Then, we have correlated ISSF with MASES in enthesitis group. RESULTS: We showed that 54 (22.6%) of 238 patients had enthesitis. The demographic features were similar between the groups. The enthesitis group had higher ISSF scores (p < 0.001); higher frequency of fever (p = 0.004), exertional leg pain (p < 0.001), myalgia (p < 0.001) and arthritis (p = 0.01); and more intense, widespread, frequent and longer attacks compared with controls. Moreover, there was a weak correlation between ISSF and MASES in the patients with enthesitis. CONCLUSION: Enthesitis may be a sign of more severe FMF phenotype and frequently associated with other musculoskeletal manifestations resemble SpA. Key points •More than one-fifth of the patients with FMF would suffer from enthesitis. •The FMF patients with enthesitis had higher ISSF scores; higher frequency of fever, exertional leg pain, myalgia and arthritis; and more intense, widespread, frequent and longer attacks as compared with controls. •Enthesitis may be a sign of more severe FMF phenotype and frequently associated with other SpA-like musculoskeletal feature.

Predictors and the optimal duration of sustained remission in rheumatoid arthritis.

OBJECTIVE: To determine predictors and optimal duration of sustained remission (SR) in patients with rheumatoid arthritis (RA). METHODS: A total of 428 consecutive patients with RA visiting our clinic routinely between 2012 and 2013 were evaluated. Seventy seven of these patients in DAS28 remission were enrolled and followed up for 62.2 ± 9.9 months. Patients in remission ≥ 6 months (SR) and shorter (non: N-SR) were compared in terms of demographic-clinical data and the psychosocial factors. At enrollment, 1st and 5th years, patients in DAS28, SDAI, and Boolean remission were determined. RESULTS: Sixty three patients were in SR and 14 in N-SR. Lower baseline DAS28 and HAQ scores, anti-CCP were positive predictors of SR. Although the presence of anxiety, depression, fibromyalgia, and fatigue were lower in the SR group, there was no significance. Patients in DAS28 remission (100%) at baseline reduced to 64% at 1st and 42.6% at 5th years. Patients satisfying SDAI and Boolean remission at these three visits were 49%, 44%, and 32.4% vs 41%, 28%, and 20.6%, respectively. If the duration of remission is defined as 6 months, the remission rates of SDAI at inclusion and fifth years' visits were similar but Boolean remission rates differed significantly and if it is accepted as ≥ 12 months, both the SDAI and Boolean remission rates were not different. CONCLUSION: Low DAS28 and HAQ scores at baseline, anti-CCP were positive predictors of SR. Instead of 6 months, remission duration for ≥ 12 months would probably help us to predict SR independently from the chosen criteria; Boolean or SDAI.

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. Materials and methods: Data of the 2246 FMF patients, from 15 adult rheumatology clinics located in different parts of the country, were evaluated retrospectively. The following mutations have been tested in all patients: M694V, M680I, M694I, V726A, and E148Q. Results: There were 1719 FMF patients with available genetic testing. According to the genotyping, homozygous M694V, present in 413 patients (24%), was the most common mutation . One hundred and fifty-four (9%) of patients had no detectable mutations. Allele frequencies of common mutations were: M694V (n = 1529, 44.5%), M680I (n = 423, 12.3%), V726A (n = 315, 9.2%), E148Q (n = 214, 1%), and M694I (n = 12, <1%). Conclusion: In this large-scale multicenter study, we provided information about the frequencies of common MEFV gene mutations obtained from adult Turkish FMF patients. Nearly half of the patients were carrying at least one M694V mutations in their alleles.

Prevalence of inflammatory back pain and radiologic sacroiliitis is increased in patients with primary Sjögren's syndrome.

INTRODUCTION: The prevalence of Sjögren's syndrome (SS) in patients with the diagnosis of SpA has been reported to be higher than normal population. Yet, the vice-versa is unclear. In this study, we aimed to investigate the prevalence of IBP, radiologic sacroiliitis and SpA in patients with primary SS. METHODS: 85 patients followed at the rheumatology clinics of the Marmara and Kocaeli Universities with the diagnosis of primary SS between November 2011 and August 2012 were included in this study. The control group consisted of 100 age-and gender-matched patients. Inflammatory back pain and axial SpA were diagnosed according to the assessment of spondylo arthritis International Society (ASAS) criteria. RESULTS: 83 patients were (97%) female and 2 (3%) were male. Mean age of the patients was 49.1 (±11) years. Mean disease duration was 7.3 (±4) years. The patient and control groups were comparable in terms of age and gender (p > 0.05). Inflammatory back pain was observed in 21 (24.7%) of 85 primary SS patients and in 4 (4%) of 100 control subjects (p < 0.001), radiographic sacroiliitis was demonstrated in 9 (10.5%) of primary SS patients and 2 (2%) of the control subjects (p = 0.025). Remaining SpA findings were not encountered in either group. CONCLUSION: inflammatory back pain and radiologic sacroiliitis is increased in patients with primary SS. Whether IBP, SI joint inflammation and radiologic sacroiliitis is due to the co-existence of SpA and primary SS or IBP is an underdiagnosed clinical feature of SS deserves further studies of large patient numbers.

Comparison of early versus late onset familial Mediterranean fever.

AIM: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. One of the common characteristics of this disease is its young age predominance. Nearly 90% of patients experience disease flares during early adult age periods. Currently there are limited data for the comparison of early versus late onset FMF and therefore the primary aim of this study was to investigate these two subsets with regard to their certain demographic, clinical and genetic differences. METHODS: Early (≤ 20 years, Group 1) and late (> 20 years, Group 2) onset FMF patients were identified from the national FMF registry that involves 2246 patients from 15 adult rheumatology clinics located in different geographical areas of Turkey. RESULTS: Of the 2246 patients, 1633 (72.7%) were aged ≤ 20 years old (Group 1) and the remaining 613 were older than 20 years (Group 2). Delay in diagnosis was longer in Group 1 and fever, peritonitis, pleuritis, erysipelas-like erythema (ELE), arthritis, family history of FMF and amyloidosis were more common in Group 1. On the other hand, sex distribution, rates of amyloidosis, vasculitis and kidney failure were not different between the groups. Among patients with available genotypes, homozygous and heterozygous M694V mutations were significantly higher and heterozygous E148Q mutation was significantly lower in Group 1 compared to Group 2. CONCLUSION: Patients with FMF whose symptoms start before 20 years of age seem to have severe symptoms and M694V mutation may be responsible for the early expression of the disease.

Fatigue in patients with Behçet's syndrome: relationship with quality of life, depression, anxiety, disability and disease activity.

OBJECTIVES: Fatigue is a common symptom of chronic inflammatory diseases. The objective of this study was to investigate fatigue in patients with Behçet's syndrome (BS) and to examine the relationship between fatigue and disease activity, quality of life, anxiety and depression. METHODS: This is a cross-sectional study of 123 BS patients and 71 healthy controls in Turkey. All subjects completed the Multidimensional Assessment of Fatigue (MAF) questionnaire, Short form-36 (SF-36), Hospital Anxiety and Depression (HADS) scale and Health Assessment Questionnaire (HAQ). Disease activity among BS patients was assessed using the Behçet Syndrome Activity Scale (BSAS) and the physician's global assessment (PGA). RESULTS: BS patients had significantly higher MAF, HADS-depression (HADS-D) and HADS-anxiety (HADS-A) scores than the healthy controls (P < 0.001). Both the physical and mental components of the SF-36 scale were impaired in BS patients (P = 0.0001). BS patients with active disease, depression and anxiety had significantly higher MAF scores compared to BS patients without active disease, depression and anxiety (P = 0.0001). MAF scores showed positive correlations with HADS-A, HADS-D, HAQ scores and negative correlations with SF-36 mental and physical components. In regression analyses, depression, anxiety and physical dysfunction were significantly associated with fatigue, after adjusting for age, sex, SF-36 physical and mental scores, HAQ, HADS-A, HADS-D and BSAS scores (P < 0.05). Decreases in SF-36 physical and mental scores were significant predictive factors for high MAF score in healthy controls (P < 0.05). CONCLUSIONS: Fatigue is common in clinically active BS patients compared with healthy controls and inactive BS patients. Depression, anxiety and physical dysfunction were significantly associated with fatigue.

Single Gallstone Associated with High Fasting Insulin Level.

Gallstone (GS) formation is a multifactorial process and one of the associated factors is hyperinsulinemia. The aim of this cross-sectional study was to determine the association between size and count of gallstones, and insulin levels and resistance. The study group composed of 84 patients who have ultrasonographically confirmed gallstone(s). Insulin level of all participants were measured and insulin resistance was calculated with the homeostasis model assessment of insulin resistance (HOMA-IR) index. All p-values &lt; 0.05 were considered statistically significant. There were 28 patients with single stone and 56 with multiple stones. Mean insulin level was 12.54 &plusmn;11.66 ml/U (median 9.91 ml/U, IQR 6.33) and 56.3% of patients had insulin resistance. Mean stone size was 7.82 &plusmn;7.52 mm (median 6 mm and IQR 11.75). There was a non-significant association and correlation between insulin level and size of GS (p=0.129; r =0.16). There was significant difference between single stone group and multiple stones group, according to the insulin level.

Health-related quality of life assessed by LupusQoL questionnaire and SF-36 in Turkish patients with systemic lupus erythematosus.

The LupusQoL is a disease-specific health-related quality of life (HRQoL) measure for patients with lupus. We conducted this study to compare the efficiency of LupusQoL-TR (validated Turkish version of the LupusQoL questionnaire) with the 36-item Short-Form Health Survey (SF-36), a generic quality of life (QoL) scale, in Turkish patients with lupus. Both questionnaires were conducted at a single visit to the clinic. Disease activity was measured with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Associations between the LupusQoL-TR and SF-36 domains were examined while also examining age, disease duration, and disease activity for each questionnaire. Descriptive statistics, Spearman's correlation coefficients, and Students t test were performed to analyze the data. A total of 113 consecutive patients with lupus (F/M 108:5, mean age 40.6 ± 11.9 years, mean disease duration 8.5 ± 7.0 years) were included, and 69 % of these were active. The median SLEDAI score was 2 (0-24), the mean global LupusQoL-TR score was 60.9 ± 23.3, and the mean SF-36 score was 41.2 ± 9.0. There was a significant correlation between LupusQoL-TR and SF-36 mean scores (r = 0.83; p < 0.001). QoL assessed by LupusQoL-TR and SF-36 did not correlate with disease activity (r = -0.11; p = 0.244 and r = -0.03; p = 0.721, respectively). LupusQoL-TR and SF-36 questionnaires were beneficial instruments in evaluating HRQoL in Turkish lupus patients. However, LupusQoL-TR and SF-36 were not associated with SLEDAI scores, which suggested that QoL might be affected by other factors besides disease activity, especially in clinically inactive or mildly active patients.

Biomarkers in Remission According to Different Criteria in Patients with Rheumatoid Arthritis.

OBJECTIVE: Remission is the primary aim in the treatment of patients with rheumatoid arthritis (RA). In this study, we aimed to evaluate biomarker profiles of patients in remission by different criteria and compare these profiles with controls. METHODS: Serum levels of calprotectin, interleukin 6 (IL-6), type II collagen helical peptide, C-terminal crosslinking telopeptide of type I collagen generated by matrix metalloproteinases (ICTP), matrix metalloproteinase 3 (MMP-3), resistin, and leptin were measured by ELISA in 80 patients. The patients were in Disease Activity Score at 28 joints with erythrocyte sedimentation rate (DAS28-ESR) remission, and had these characteristics: female/male 54/26, mean age 51.4 ± 12.1 years, mean disease duration 11.4 ± 8.1 years, rheumatoid factor positivity 68.7% (n = 55), anticyclic citrullinated peptide positivity 60.7% (n = 48). These patients were also evaluated for the American College of Rheumatology/European League Against Rheumatism (Boolean) and Simple Disease Activity Index (SDAI) remissions. Additionally, 80 age-, sex-, and comorbidity-matched individuals without rheumatic diseases were included in the study as controls. RESULTS: At recruitment of 80 patients in DAS28 remission, 33 patients (41.2%) were found in Boolean remission and 39 patients (48.7%) were in SDAI remission. Serum MMP-3, ICTP, resistin, and IL-6 levels of the 80 patients in DAS28 remission were statistically significantly higher than the controls. Patients in Boolean and SDAI remissions had significantly higher serum ICTP, resistin, and IL-6 levels in comparison with the controls. CONCLUSION: The 3 commonly used remission criteria of RA are almost similar with regard to patients' biomarker levels. Biomarker profiles of patients may provide complementary information to clinical evaluation of remission and may help to determine the patients under the risk of progression.

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.

Anxiety and depression predict quality of life in Turkish patients with systemic lupus erythematosus.

OBJECTIVES: The aim of our study was to evaluate quality of life (QoL) in patients with systemic lupus erythematosus (SLE) and assess the impact of disease activity and psychological distress on health-related quality of life (HRQoL) in Turkey. METHODS: The Medical Outcomes Study Short Form (SF) -36 was used in a cohort of 113 consecutive patients with SLE and 123 age- and gender-matched healthy subjects to measure HRQoL. Patients' disease activity was assessed with SLE disease activity index (SLEDAI) and psychological distress was evaluated by the Hospital Anxiety and Depression Scale (HADS) for all participants. Patients' demographic and clinical data were recorded at the time of HRQoL and HADS testing. Multiple logistic regression analysis was performed to explore the relationships between demographics, disease duration, disease activity as well as psychological (anxiety and depression) variables and the HRQoL. RESULTS: SLE patients have lower quality of life than healthy controls. No relationship between HRQoL and SLE activity or disease duration were observed. Patients with anxiety and/or depression reported worse SF-36 scores than those without psychological distress. The results of multivariate analysis suggested that HADS-A, HADS-D scores and working status were associated with the impairment of HRQoL. CONCLUSIONS: HRQoL is impaired in patients with SLE and is associated with mood disorders. Physicians should pay close attention to detect anxiety and depression and manage them in order to improve the quality of life in patients with SLE.

A severe case of systemic lupus erythematosus with increased pressure communicating hydrocephalus.

Normal/increased pressure hydrocephaly is an unusual manifestation of systemic lupus erythematosus (SLE), and the pathogenesis is still unclear. We report the case of an 18-year-old white female with severe refractory renal and pulmonary involvement who developed stupor during intensive immunosuppressive treatment. Enlarged ventricles on imaging and increased intracranial pressure with the exclusion of infectious and hemorrhagic/thrombotic processes suggested increased pressure communicating hydrocephalus associated with SLE. Few case reports are reviewed, and potential pathophysiologic mechanisms are discussed.

The role of depression, anxiety, fatigue, and fibromyalgia on the evaluation of the remission status in patients with rheumatoid arthritis.

OBJECTIVE: To investigate the effect of depression, anxiety, fatigue, and fibromyalgia (FM) on the remission status in patients with rheumatoid arthritis (RA), defined according to the 28-joint count Disease Activity Score (DAS28)-erythrocyte sedimentation rate (ESR) and the Boolean-based new American College of Rheumatology/European League Against Rheumatism remission criteria. METHODS: The subjects were patients with RA who participated in a hospital-based observational cohort. Patients who met the DAS28-ESR remission criteria at their latest visit were invited to participate in our study. The patient groups fulfilling or not fulfilling the Boolean remission criteria were identified and compared with each other with regard to the presence of depression, anxiety, fatigue (0-50), and FM. The relationship between psychosocial factors and Simplified Disease Activity Index (SDAI) remission, which is the index-based definition of remission in RA, was also investigated. RESULTS: A total of 87 out of 428 patients (20%) with RA met the DAS28-ESR remission criteria and 32 (37%) of these also met the Boolean remission criteria, while 55 (63%) did not. Forty patients were also in SDAI remission. In the Boolean remission group, 2 patients had depression and 2 had anxiety (p = 0.004). In the Boolean nonremission group, 19 patients had depression and 13 had anxiety (p = 0.04). Continuous scales of anxiety (3.34 ± 3.76 vs 5.83 ± 4.70, p = 0.012) and depression (2.18 ± 2.75 vs 4.63 ± 4.10, p = 0.001) were also lower in the Boolean remission group in comparison with the nonremission group. Though FM syndrome was detected in only 1 patient of the Boolean remission group and in 7 patients of the Boolean nonremission group (p = 0.249), patients' polysymptomatic distress scores of FM in the Boolean remission group were significantly lower than those of the nonremission group (3.12 ± 3.25 vs 6.27 ± 5.19, p = 0.001). The mean fatigue scores were 9.5 ± 10.6 in the Boolean remission group and 16.8 ± 12.8 in the Boolean nonremission group (p = 0.006). In multivariate analysis, patient's global assessment (PtGA) and depression were found as the independent discriminators of Boolean-based definition. Similar relationships were also observed between psychosocial factors and SDAI remission. CONCLUSION: In patients with RA who do not fulfill the Boolean remission criteria, to avoid overtreatment, assessment of anxiety, fatigue, FM, and especially depression must be considered if PtGA scores and disease activity variables are significantly different.

An unusual case with vasculo-Behçet's disease: peripheral arterial aneurysm in an elderly woman.

Behçet's disease (BD) is a chronic, multi-systemic disorder that can affect all sizes of arteries and veins. Vascular involvement of BD is generally observed in young men and is an important cause of morbidity and mortality. Arterial lesions can appear as aneurysms, stenosis and occlusions in BD. We, here, present the case of a woman who developed a peripheral arterial aneurysm in her sixth decade, 20 years after disease onset. BD patients with aneurysms should be consulted at any age for pre- and post-operative assessment for immunosuppressive treatment to reduce recurrences, complications and disease activation.

Accelerated infusion rates of rituximab are well tolerated and safe in rheumatology practice: a single-centre experience.

Due to the possible risk of infusion reactions of rituximab (RTX), a slow infusion rate (total infusion time, 255 min) is suggested for rheumatological use. However, especially in oncology field, accelerated infusion of RTX is reported to be well tolerated and safe. The aim of our study was to evaluate whether accelerated infusion rates of RTX would similarly be safe and tolerable in rheumatoid arthritis (RA) patients and other off-label rheumatological indications. All patients treated with RTX for RA and other autoimmune diseases between May 2011 and January 2012 were recruited to the study. Each treatment course consisted of two RTX 1,000 mg infusions, 2 weeks apart. Total time of the infusion for the first cycle was 255 min. Second and subsequent infusions were administered over 120 min as follows: 0-30 min, 100 mg; 30-60 min, 200 mg; 60-90 min, 300 mg; and 90-120 min, 400 mg. The Clinical Trials Classification of Adverse Events (CTCAE) version 4.3 was used to categorise side effects. The study population comprised 68 patients [F/M, 59:9; mean age, 52.4 (10.6) years]: 60 with RA, 4 with systemic lupus erythematosus (SLE), 1 with non-Hodgkin's lymphoma with SLE and 3 with vasculitis. A total of 77 fast infusions were administered. Eleven patients (16.2 %) had taken a fast infusion at the first course. A total of nine patients experienced at least one AE. Seven patients had a reaction on the first infusion (infusion-related reaction (IRR)), two patients on the second infusion and one patient on both infusions. When graded from 1 to 5 according to CTCAE v. 4.3, grade 1 IRRs were observed in a total of seven patients and grade 2 IRR in three patients. In this study of fast infusions, adverse events after RTX were mostly mild and seem to be well tolerated. Faster rituximab infusion times seem to be safe and might be incorporated into routine practice.