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Journal: Rheumatology international


Fibromyalgia (FM) syndrome is a disabling clinical condition of unknown cause, and only symptomatic treatment with limited benefit is available. Gluten sensitivity that does not fulfill the diagnostic criteria for celiac disease (CD) is increasingly recognized as a frequent and treatable condition with a wide spectrum of manifestations that overlap with the manifestations of FM, including chronic musculoskeletal pain, asthenia, and irritable bowel syndrome. The aim of this report was to describe 20 selected patients with FM without CD who improved when placed on a gluten-free diet. An anti-transglutaminase assay, duodenal biopsy, and HLA typing were performed in all cases. CD was ruled out by negative anti-transglutaminase assay results and absence of villous atrophy in the duodenal biopsy. All patients had intraepithelial lymphocytosis without villous atrophy. Clinical response was defined as achieving at least one of the following scenarios: remission of FM pain criteria, return to work, return to normal life, or the discontinuation of opioids. The mean follow-up period was 16 months (range 5-31). This observation supports the hypothesis that non-celiac gluten sensitivity may be an underlying cause of FM syndrome.

Concepts: Medical terms, Wheat, Constipation, Irritable bowel syndrome, Coeliac disease, Gluten, Gluten-free diet, Dermatitis herpetiformis


Shoulder-hand syndrome is a reflex sympathetic dystrophy which is usually associated with minor trauma, fracture or surgical procedures on bones, or follows peripheral nerve injury. In the present report, we describe a patient who developed sympathetic dystrophy which revealed a lung cancer. Reflex sympathetic dystrophy, therefore, should be considered an occasional manifestation of a paraneoplastic syndrome warranting a thorough search for underlying malignancy.

Concepts: Cancer, Metastasis, Oncology, Lung cancer, Cancer staging, Surgery, Small cell carcinoma, Paraneoplastic syndrome


Our aim was to assess the effect of dietary elimination of monosodium glutamate (MSG) and aspartame on perceived pain in fibromyalgia. A total of 72 female patients with fibromyalgia were randomized to discontinuation of dietary MSG and aspartame (n = 36) or waiting list (n = 36). Patients were requested to rate their pain using a seven-point scale. Comparisons between both groups showed no significant differences on pain referred during the baseline or after the elimination of dietary MSG and aspartame. The discontinuation of dietary MSG and aspartame did not improve the symptoms of fibromyalgia.

Concepts: Amino acid, Glutamic acid, Excitotoxicity, E number, Monosodium glutamate, Umami, Flavour enhancer, Ajinomoto


More than 14 years of clinical practice in rheumatology led the author to discover the prognostic role of anti-citrullinated protein antibody (ACPA) as well as the erosions found by MRI, in detecting the RA patients resulting in establishing a new set of criteria by revising the 1987 ACR classification-Iran Criteria for Rheumatoid Arthritis. Medical records of 243 patients at the outpatient Rheumatology Clinic of the author (private sector) were reviewed for the data on the criteria of the 1987 ACR, 2010 ACR/European League against Rheumatism (EULAR), and Iran Criteria for RA. In addition to modifying the 1987 ACR classification, Iran Criteria for RA adds some additional information to the ACR criteria (including ACPA and bony erosions detected by MRI), and any patient who satisfies 6 out of 12 points is considered as a definite RA patient. Sensitivity of the three classifications was calculated considering the clinical diagnosis by a single rheumatologist as the gold standard. A total of 63 male and 180 female patients with a mean follow-up duration of 28.24 ± 50.19 months were considered. Mean age at diagnosis and mean disease duration were 49.16 ± 15.38 years and 7.04 ± 6.87 months, respectively. The sensitivity for Iran Criteria for RA, 1987 ACR classification, and 2010 ACR/EULAR criteria were calculated as 98.4, 59.7, and 66.3%, respectively. Comparing Iran Criteria for RA with ACR and ACR/EULAR criteria, it was concluded that our newly introduced criteria is a more sensitive instrument in determining RA patients in the early stages of the disease.

Concepts: Medical terms, Rheumatoid arthritis, Rheumatology, Vasculitis, Gout, Arthritis, Rheumatism, European League Against Rheumatism


Fatigue is a frequent symptom during ankylosing spondylitis (AS) often under estimated which needs to be measured properly with respect to its intensity by appropriate measures, such as the multidimensional assessment of fatigue (MAF). The aims of this study were to translate into the classic Arabic version of the MAF questionnaire and to validate its use for assessing fatigue in Moroccan patients with AS. The MAF contains 16 items with a global fatigue index (IGF). The MAF was translated and back-translated to arabic, pretested and reviewed by a committee following the Guillemin criteria (J Clin Epidemiol 46:1417-1432, 1993). It was then validate on 110 Moroccan patients with AS. Reliability for the 3-day test-retest was assessed using internal consistency by Cronbach’s alpha coefficient and the intra-class correlation coefficient (ICC). External construct validity was assessed by correlation with pain, activity of disease and other keys variable. The reproducibility of the 15 items was satisfactory with a kappa statistics of agreement superior to 0.6. The ICC for IGF score reproducibility was good and reached 0.98 (IC 95%, 0.96-0.99). The internal consistency was at 0.991 with Cronbach’s alpha coefficient. The construct validity showed a positive correlation between MAF and the axial (r = 0.34) and peripheral (r = 0.32) visual analogical scale, the Bath ankylosing spondylitis disease activity index (BASDAI) (r = 0.77), the first item of BASDAI (r = 0.85), the functional disability by the Bath ankylosing spondylitis functional index (r = 0.64), the erythrocyte sedimentation rate (r = 0.43) and the C reactive protein (r = 0.30) (for all P < 0.001). There was no statistical correlation between MAF and the other variables. The Arabic version of the MAF has good comprehensibility, internal consistency, reliability and validity for the evaluation of Arabic speaking patients with AS.

Concepts: Psychometrics, Correlation and dependence, Reliability, Pearson product-moment correlation coefficient, Covariance and correlation, C-reactive protein, Cronbach's alpha, Ankylosing spondylitis


To compare the characteristics of patients with systemic sclerosis who died within 2 years of diagnosis to those who died after 2 years of diagnosis. A retrospective chart review of all medical records of deceased systemic sclerosis (SSc) patients who had been followed at our institution from 1985 to 2007 was performed. We identified 87 deceased SSc patients within this period. From the 87 deceased individuals, 20 had died within 2 years after they were diagnosed, and 67 had died after 2 years of their diagnosis. Patients who died within 2 years of diagnosis were more likely to be anticentromere antibody negative when compared to the patients who died after 2 years (17/20 vs. 48/67, P = 0.03). The time from the first appearance of non-Raynaud’s symptoms to diagnosis was significantly shorter in the group who died within 2 years than in the group who died after 2 years of diagnosis (11.8 ± 10.3 vs. 60.7 ± 64.9 months, P = 0.002). According to the Medsger severity score, there was more severe muscle (0.82 ± 1.13 vs. 1.8 ± 1.28, P = 0.0014) and heart (0.86 ± 1.37 vs. 2.1 ± 1.71, P = 0.0013) involvement at the initial evaluation in patients who died before 2 years of diagnosis when compared to the group of patients who died after 2 years of diagnosis. The time from the first symptoms to treatment initiation was significantly shorter in patients who died early (9.43 ± 6.3 vs. 38.3 ± 54.4 months, P = 0.05). The interval between treatment initiation and death was also significantly shorter (15.1 ± 9.48 vs. 60.7 ± 49.7 months, P = 0.001), reflecting greater severity of disease. Patients who died within the first 2 years of SSc diagnosis were typically anticentromere negative and had significant muscle and cardiac involvement. The time from the first appearance of non-Raynaud phenomenon symptoms to death was much shorter in the patients who died within 2 years of diagnosis, suggesting a very fulminant form of systemic sclerosis.

Concepts: Disease, Death, Medical terms, Demography, Heart, Rheumatology, Scleroderma, Systemic scleroderma


The objective of this study is to estimate the value of diffusion-weighted MRI (DWI) in the detection of abnormalities in sacroiliac joints in the patients with early ankylosing spondylitis (AS) and investigate the feasibility of whole-body DWI (WB-DWI) in systemic evaluation of AS. Sixteen patients with early AS, 18 patients with simple low back pain (LBP), and 18 healthy volunteers were involved in this study. All subjects underwent conventional MRI and DWI. Apparent diffusion coefficient (ADC) in subchondral bone marrows of sacroiliac joints was measured. Independent-sample t test was used to statistically analyze the difference of ADC values between groups. WB-DWI was performed in additional 12 patients with clinically confirmed AS. The image results were analyzed by multiple post-processing techniques, as compared to conventional MRI. In AS patients, mean ADC values were (0.494 ± 0.170) × 10(-3) mm(2)/s in sacrum and (0.513 ± 0.129) × 10(-3) mm(2)/s in ilium, which were significantly higher than those of LBP ((0.306 ± 0.057) × 10(-3) mm(2)/s in sacrum and (0.323 ± 0.083) × 10(-3) mm(2)/s in ilium) and healthy volunteers ((0.315 ± 0.009) × 10(-3) mm(2)/s in sacrum and (0.319 ± 0.012) × 10(-3) mm(2)/s in ilium). No statistical differences were found between simple LBP and healthy volunteers. Mean ADC value of multiple lesions in AS was (0.932 ± 0.299) × 10(-3) mm(2)/s. By WB-DWI, abnormal signals of sacroiliac joints and extra-sacroiliac joint lesions were demonstrated in the locations corresponding with clinical findings. Mean ADC values of multiple lesions were (1.298 ± 0.323) × 10(-3) mm(2)/s in sacrum and (1.216 ± 0.311) × 10(-3) mm(2)/s in ilium. DWI and WB-DWI were shown to be effective in differentiation and systemic evaluation of early AS. Both techniques are likely to play an importance role in the early diagnosis of AS and assessment of treatment response.

Concepts: Low back pain, Statistics, Medical imaging, Magnetic resonance imaging, Joint, Pelvis, Diffusion MRI, Sacroiliac joint


Although the inflammatory cascade of familial Mediterranean fever (FMF) is partially understood, triggering factors of those attacks has not been studied well. It is supposed that physical stresses such as cold exposure, tiredness and emotional stresses could provoke attacks. This study is aimed to survey the factors regarded as triggering the attacks in patients with FMF and their relationship with MEFV gene mutations. Clinical findings and genetic mutations (consist of M694V, M694I, M680I, V726A, E148Q) of patients were recorded. Patients were questioned about cold exposure, emotional stress, tiredness, long-lasting standing, long-duration travel, starvation, high intake of food, trauma, and infection as triggering factors for the attacks with both serositis and musculoskeletal pain. The study is comprised of 275 FMF patients (male/female: 177/98). The most common triggering factors for the attacks with serositis were cold exposure (59.3 %), emotional stress (49.8 %), tiredness (40.0 %) and menstruation (33.7 % in females). Long-lasting standing (78.8 %), long-duration travel (64.1 %) and tiredness (47.8 %) were the triggering factors for the attacks with musculoskeletal symptoms. The relationships between MEFV mutations and triggering factors were found as M694V allele with starvation, E148Q allele with high intake of food and V726A allele with long-duration travel. The attacks with serositis seem to be triggered by those factors to which whole body exposed, whereas the attacks with musculoskeletal complaints seem to be triggered by those factors to which regional or local part of body exposed. Since the number of alleles was small, a clear conclusion for a relationship between a particular gene variant and a specific trigger was not made.

Concepts: Gene, Genetics, Mutation, Evolution, Familial Mediterranean fever, Arthritis, Myalgia, MEFV


Erythema nodosum (EN) is the most common cause of inflammatory nodules and usually affects the lower extremities and especially pretibial regions. EN may be idiopathic or associated with a wide spectrum of conditions including systemic diseases, infection, treatment with various drugs, pregnancy, and exceptionally with malignancies. The purpose of this study is to investigate the EN patients with different etiologies and laboratory features admitted to the rheumatology department and to compare them with other EN patients admitted to different departments including dermatology and infectious diseases. Totally, 107 patients diagnosed with EN (male/female: 37/70) were enrolled in the study. Of the 107 EN patients, 37 participants who were categorized as primary (idiopathic) EN (34.6 %) had not any underlying diseases or precipitating events. Majority of the participants were women (male/female: 12/25; mean age: 42.9 ± 9.2 years). Precisely, 70 EN (secondary EN) patients (65.4 %) had an underlying disease (male/female: 25/45; mean age: 36.1 ± 10.1). Behçet’s disease (BD) was the foremost (n = 40, 37.4 %), followed by sarcoidosis (n = 17, 15.9 %), post-streptococcal (n = 9, 8.4 %), and other rheumatologic disease (one patient temporal arthritis, one patient Sjögren’s syndrome, 1.9 %). Consequently, it is observed that BD, sarcoidosis, and post-streptococcal infection were found as the main etiologies of EN patients treated in our rheumatology department. These diseases should be kept in mind as an etiological factor in the management of EN.

Concepts: Medicine, Cancer, Disease, Infectious disease, Hospital, Infection, The Canon of Medicine, Erythema nodosum


An increase in interstitial fluid is an expression of bone marrow edema (BME) and osteonecrosis (ON). The exact pathogenetic processes still remain unknown. Treatment options are mainly symptomatic with core decompression as surgical golden standard with immediate pain relief. Recently, it has been shown that intravenous iloprost can be used to achieve a reduction in BME and ON with a considerable improvement in the accompanying symptoms. The effect of intraveneously applied iloprost alone (12 patients) was studied against core decompression alone (12 patients) as well as iloprost following core decompression (12 patients). We could find a significant improvement in HHS, WOMAC score, SF-36 score and VAS 3 months and 1 year after therapeutical intervention in all treatment groups; however, statistically best results were obtained by combination. Concerning the MRI scans, we found a distinct reduction in BME in all groups again favoring the combination. Concerning ON, the results were not as promising as for BME. Intravenous prostacyclin and core decompression as monotherapy are of efficient therapeutical benefit in the treatment of BME, and the combination of both methods, however, seems to be most promising, also in the treatment of ON. Long-term results and higher number of patients are needed for final statements.

Concepts: Bone marrow, Lymphatic system, Avascular necrosis, Diving medicine, Osteochondritis dissecans, Femur head, Epiphysis, Dysbaric osteonecrosis