Current Rheumatology Reviews - Volume 18, Issue 3, 2022

Background: The presentation of a hot swollen joint is common in the emergency department, general practice, rheumatology and orthopedic clinics. There is a wide set of differential diagnoses for a hot swollen joint, thus making it difficult to diagnose and manage, especially for junior doctors. Initially, it is pertinent to exclude/diagnose medical and surgical emergencies. Objective: This paper aims to summarize the key indications within the history, examination and investigations in order to quickly and effectively diagnose a hot swollen joint based on the original 2006 management guidelines and the papers discussing other possible indications and management strategies published since then. Results: Currently, the management of crystal and non-infectious arthropathies is well recognized with little disparity. However, the treatment of infectious arthritis is not concrete, and there are discrepancies in management between doctors. Conclusion: We have summarized the key indications and provided a diagnostic flow chart to aid with the management of a hot swollen joint.

Osteoarthritis (OA) is a whole joint disease that is significantly related to abnormal mechanical loads. Subchondral bone alterations, during the evolution course of OA, are considered a reflection of the adaptation of the bone tissue to mechanical loads. However, some of these alterations are taken as a detriment and paradoxical. What are these structure, composition, and mechanical property alterations or mechanical functions for are not quite clear. In this review, we discuss the possibility that these alterations are used for maintaining the joint function. With taking excessive load as a risk factor and under conditions of articular cartilage gradually lose its thickness and its function of evenly distributing the load on the subchondral bone plate, and applying Poroelasticity to bone mechanics; moreover, Boussinesq’s pressure bulb theory and bone optimal design principles are utilized. We found that each subchondral bone alteration has its unique mechanical function in resisting loads and maintaining the joint function, and these alterations comply with both bone optimal design principles and Wolff’s law within a proper range.

Cryofibrinogenemia refers to the presence of cryofibrinogen in plasma. This protein has the property of precipitating at lower temperatures. Cryofibrinogenemia is a rare disorder, clinically characterized by skin lesions, such as ulcers, necrosis, livedo reticularis, arthralgia, thrombosis, and limb ischemia. These features are most often observed in rheumatological practice and consist of differential diagnoses of antiphospholipid syndrome, primary vasculitis, thrombotic thrombocytopenic purpura, and cryoglobulinemia. Classical histopathological findings include the presence of thrombi within the lumen of blood vessels of the skin without vasculitis. To date, there are no validated classification criteria. Management includes corticosteroids, immunosuppressive therapy, anticoagulants, and fibrinolytic agents. This narrative review aims to make physicians, particularly rheumatologists, aware of the existence of this underdiagnosed condition. There are no epidemiological studies evaluating the prevalence of cryofibrinogenemia in different rheumatological disorders. Studies are also required to investigate if certain features of rheumatological diseases are related to the presence of cryofibrinogenemia.

Objective: Behcet’s disease (BD) is a chronic multisystem inflammatory disease classified as Variable Vessel Vasculitis with unclear etiology. We designed this systematic review and meta-analysis to evaluate vitamin D status in Behcet’s disease patients with this background. Methods: We performed this systematic review and meta-analysis according to PRISMA guidelines. We included all observational studies in humans published in English, evaluating the association of 25(OH)D concentrations in Behcet’s patients. Two reviewers (HRK and AE) independently searched the databases and screened articles based on their titles and abstracts. A third reviewer resolved all disagreements. We performed analysis using Cochrane Program Review Manager Version 5.3. The protocol for this review was registered on PROSPERO (CRD42020197426). Results: A total of 341 publications were initially identified according to the search strategy. Finally, 12 publications were included in the meta-analysis. We performed this meta-analysis on 1265 participants from different studies with a sample size ranging from 63 to 224 individuals. In studies comparing active and inactive subgroups of patients with Behcet’s disease, we found a significantly lower serum level of vitamin D in patients with Active BD (-0.4; 95% CI: -0.61, -0.25; p<0.001). We found that the serum level of vitamin D in Behcet’s disease is significantly higher than in health controls (0.5; 95% CI: 0.15, 0.50; p=0.001). Conclusion: We demonstrated that the existing evidence is consistent with the hypothesis that an increased serum level of vitamin D would be associated with a substantially lower risk of active Behcet’s disease.

Background: The hand is an excellent work tool that provides the functional ability to mechanical work. The hand is affected in rheumatoid arthritis (RA) patients, it is a significant problem in the functional sphere as a result of deformities, the grasping function limitation and muscle strength. Objectives: The aim of the study was the assessment of grip strength, endurance and manipulation abilities of rheumatoid hands with or without deformities treated with methotrexate (MTX) or MTX plus biologics (MTX+BIO). Methods: The study involved 80 RA women, (40 received MTX+BIO, 40 MTX), treated at the Rheumatology Department of the Central Clinical Hospital of Interior Affairs in Warsaw. VASpain, DAS28, SDAI, HAQ, HAQ hands, estimation of hand grip strength, endurance, and manipulation ability were analyzed. Results: In group MTX+BIO, values of DAS28 (3.7±1.3 vs. 4.3±1.2, p=0.019), HAQ (0.72 ± 0.57 vs. 1.08± 0.87, p=0.011) and HAQ-hand (0.85±0.65 vs. 1.19±0.68, p=0.024) were statistically lower than in MTX group. Hand deformations were recorded in 35 (43.7%) cases, 16 (40%) in MTX group, 19 (47.5%) in MTX+BIO. Comparison of grip strength, endurance, and manipulation ability showed better results in MTX+BIO group with deformities (significance level from 0.013 to 0.046) than in MTX group. Relative differences in hand function in MTX + BIO group ranged from 10.8% (maximal power grip strength) to 127.6% (minimal hand endurance), after disease duration adjustment - from 28.2% (maximal power grip strength) to 148.4% (minimal hand endurance). Conclusion: Measuring grip strength, hand endurance, manipulation abilities are useful in RA patients with hand deformities.

Objective: This study aimed at comparing the Edmonton Ankylosing Spondylitis Metrology Index (EDASMI) and Bath Ankylosing Spondylitis Metrology Index (BASMI) to determine which of the two is best correlated with disease-related parameters in axial spondyloarthritis (axSpA) patients. Methods: A cross-sectional study was carried out involving 86 patients with radiographic axSpA. Sociodemographic data, the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), the Ankylosing Spondylitis Disease Activity Score (ASDAS), the Bath Ankylosing Spondylitis Functional Index (BASFI), and the Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire were applied. Spinal mobility was assessed by two indices: the BASMI and the EDASMI. Structural damage of the spine was also evaluated by two indices: the Bath Ankylosing Spondylitis Radiology Index (BASRI) and the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS). Results: Eighty-six patients with an average age of 43.21 ± 11.43 years (20-79) were included. Impaired spinal mobility, which corresponds to higher BASMI scores, was correlated with prolonged disease duration (p < 0.01, r = 0.310), higher ASDAS-CRP (p < 0.001, r = 0.386), severe functional disability on the BASFI (p < 0.01, r = 0.505) and poorer quality of life according to the ASQoL (p < 0.01, r = 0.369). However, the EDASMI score did not correlate with any disease parameter. The BASMI was correlated with the total BASRI (p < 0.01, r = 0.634) and mSASSS (p < 0.01, r = 0.388). Unlike the BASMI, the EDASMI was neither correlated with the BASRI (p = 0.520, r = 0.245) nor the mSASSS (p = 0.252, r = -0.120). Conclusion: Our results indicate that among the studied metrological indices, the BASMI is more contributory since it is correlated with clinical disease parameters and structural damage, unlike the EDASMI.

Aim: The present study aims to compare interleukin 6 concentration in synovial fluid in patients with known types of arthritis. Background: Persistent synovitis without known markers, such as Rheumatoid Factor (RF), Anti- Citrullinated Protein Antibodies (ACPA), and genetic markers as HLA-B27, is not uncommon. It is valuable to determine the presence of chronic inflammation and put it in correlation with agerelated changes, which are especially relevant for middle-aged patients with mono- or oligoarthritis, when the dilemma to start disease-modifying drugs for inflammatory disease often is present. Interleukin 6 (IL-6) plays a significant role in chronic inflammation. Objectives: IL-6 concentration in synovial fluid reflects the presence and activity of joint inflammation. Methods: Synovial fluid was obtained from 101 patients with chronic synovitis. IL-6 concentration was determined by the immunochemical luminescence method. Results: The median IL-6 concentration in synovial fluid in patients with osteoarthritis (OA) was 138.0 pg/ml (interquartile range (IQR) 43.4 to 296.0); in patients with rheumatoid arthritis was 2516.5 pg/ml, (IQR 1136.0 to 25058.0); in reactive arthritis 2281.0 pg/ml (IQR 1392.0 to 8652.0); psoriatic arthritis 1964.0 pg/ml (IQR 754.0 to 7300.0); ankylosing spondylitis 2776.0 pg/ml (IQR 514.7, 3944.0); in a group with negative RF, ACPA and HLA-B27 inflammatory arthritis 2163.0 pg/ml (IQR 822.0 to 7875.0). There is statistically significant difference of IL-6 concentration comparing OA and each inflammatory arthritis group, p<0.0001. Conclusion: IL-6 detection in the synovial fluid is helpful in arthritis evaluation. The results show that an IL-6 level over 1000 pg/ml suggests the diagnosis of inflammatory arthritis.

Background: Coronavirus disease 2019 (COVID 19) is a worldwide pandemic that has devastated the world in a way that has not been witnessed since the Spanish Flu in 1918. In this study, we aim to investigate the outcomes of patients with rheumatic diseases infected with COVID-19 in Oman. Methods: A multi-center retrospective cohort study included patients with underlying rheumatological conditions and COVID-19 infection. Data were collected through the electronic record system and by interviewing the patients through a standard questionnaire. Results: 113 patients with different rheumatic diseases were included with the following rheumatological diagnoses: rheumatoid arthritis (40.7%), systemic lupus erythematosus (23.1%), psoriatic arthritis (8%), Behcet's disease (7%), ankylosing spondylitis (6.2%), other vasculitides, including Kawasaki disease (4.4%), and other diagnoses (10.6%). The mean (SD) age of patients was 43 (14) years, and 82.3% were female. The diagnosis of COVID-19 was confirmed by PCR test in 84.1% of the patients. The most common symptoms at the time of presentation were fever (86%), cough (81%), headache (65%), and myalgia (60%). Hospitalization due to COVID-19 infection was reported in 24.1% of the patients, and 52.2% of these patients had received some form of treatment. In this cohort, the intake of immunosuppressive and immunomodulating medications was reported in 91.1% of the patients. During the COVID-19 infection, 68% of the patients continued taking their medications. Comorbidities were present in 39.8% of the patients. Pregnancy was reported in 2% of the patients. The 30 days mortality rate was found to be 3.5%. Diabetes, obesity, and interstitial lung diseases (ILD) were the strongest risk factor for mortality (p-value 0.000, 0.000, and 0.001, respectively). Rituximab was given in 3.8% of the patients, and it was significantly associated with increased mortality among the patients (p-value <0.001). Conclusion: COVID-19 infection in patients with rheumatic diseases have an increased mortality rate in comparison to the general population, with diabetes, morbid obesity, chronic kidney diseases, interstitial lung disease, cardiovascular disease, obstructive lung disease, and liver diseases as comorbidities being the most severe risk factors associated with death. Greater care should be provided to this population, including the prompt need for vaccination.

Background and Aims: Changes in body mass composition, such as reduced lean and fat mass, can occur in a patient with rheumatic diseases. However, data about body composition in spondyloarthritis (SpA) showed conflicting results. The aim of the present study was to assess by DXA the distribution of lean and fat mass in SpA patients compared to healthy controls and to investigate the association between body mass composition and disease characteristics. Methods: We conducted a cross-sectional case-control study including 50 SpA patients and 50 controls. Sociodemographic data, as well as disease characteristics, were assessed. Body composition measurements and biochemical and inflammatory serum markers were evaluated. Radiographic data (Bath Ankylosing Radiologic Index and the modified Stroke Ankylosing Spondylitis Spine Score) was also recorded. Results: No statistically significant correlation was found between the two groups regarding bone mineral density and body mass distribution. However, a lower weight was observed in the study group compared to the control group (p = 0.043). Male gender and younger age were associated with a higher lean mass (p = 0.05). C-reactive protein (CRP) level was positively correlated with lean mass (r = 0.38, p = 0.023). Similarly, BASFI was higher in patients with lower fat mass (r = -0.42, p = 0.024). A longer duration of NSAIDs intake was associated with a lower lean mass and a higher fat mass. However, no correlations were found between body mass composition and BASRI mSASSS scores as well as bone mineral density and calcium intake. Conclusion: Our findings suggest that younger age, male gender, and axial phenotype were associated with higher lean mass.

Background: Autoimmune inflammatory rheumatic diseases have long been treated by conventional disease-modifying anti-rheumatic drugs. Biological therapy is a new era in the treatment of rheumatic diseases, but satisfaction and adherence to it is still not well tested. Aims: To assess the satisfaction and adherence to biological treatment among patients with autoimmune inflammatory rheumatic diseases. Methods: A cross sectional study was conducted among 56 patients suffering from inflammatory rheumatic diseases using Morisky 8 questionnaire and Treatment Satisfaction Questionnaire for Medication (TSQM) over a period of one month. Results: About 76.8 % of the patients had medium adherence and the underlying cause of missing doses was the unavailability of the drugs. The mean satisfaction with biological treatment was 62.7±6.9. Patients who did not receive formal education had significantly higher satisfaction with the biological treatment than others 64.94±5.01 at a P value 0.04 (<0.05). Conclusion: Patients with inflammatory rheumatic diseases in our study showed medium adherence and satisfaction. Authorities in the medical field are providing great help to these patients in need of biological therapy, but ensuring the availability of all doses of the biological treatment regimen is still necessary. Patient, family and nurse education programs are also necessary to maximize adherence and satisfaction.

Background: Disability in patients with scleroderma (SSc) has been associated with poor health-related quality of life (HRQoL) in all dimensions, including physical, psychological, and social dimensions. Objective: This study was conducted to examine different factors that may be associated with functional disability and poor HRQoL, with the aim of targeting these factors in the future to improve physical activity, functional outcomes, and HRQoL. Methods: A single-center cross-sectional study was conducted on 38 patients with SSc to compare characteristics between patients with and without disability using the Health Assessment Questionnaire Disability Index (HAQ-DI). Quality of life was assessed using the Short Form-36 (SF-36). Linear regressions were performed to examine variables contributing to functional disability. Results: Almost 65.78% (n = 25) of patients in the study group reported functional disability. The presence of functional disability was associated with reduced HRQoL, as reflected by physical function (P = 0.0001), physical role (P = 0.016), bodily pain (P = 0.001), general health (P = 0.002), social functional (P = 0.002), emotional role (P = 0.042), and mental health (P = 0.025) domains of the SF-36 score. Multiple linear regression indicated that the main predictive factors associated with HAQ-DI were the modified Hand Mobility in Scleroderma, modified Rodnan skin score, DIstance walked in 6 minutes, BOrg dyspnea index, and SAturation of oxygen at 6 minutes (DIBOSA), and Fatigue Severity Scale among patients with SSc. Conclusion: In patients with SSc, recognizing the relationships between clinical findings and functional disability will allow the development of further management strategies to minimize disease severity and enhance HRQoL.

Background: Psychiatric disorders, including schizophrenia, could herald other manifestation( s) of systemic lupus erythematosus (SLE) potentially hindering timely and optimal management. Moreover, schizophrenia is among the described ‘extra-criteria’ manifestations of anti-phospholipid syndrome (APS). Hence, screening schizophrenia patients for SLE and APS may pose diagnostic and therapeutic implications. Objectives: Examine schizophrenia patients with no overt connective tissue disease(s) manifestation( s) for clinical and/or serologic evidence of SLE and/or APS. Methods: The study included 92 schizophrenia patients (61 (66.3%) males) and 100 age- and gender- matched healthy controls. Both groups were tested for anti-nuclear antibodies (ANAs), antidouble stranded deoxyribonucleic acid (anti-dsDNA) antibodies, complement 3 (C3) and C4, and criteria anti-phospholipid antibodies (aPL) (anticardiolipin Immunoglobulin (Ig) G and IgM, antibeta- 2-glycoprotein I IgG and IgM, and lupus anticoagulant (LAC)). Results: The patients’ mean age and disease duration were 28.8 ± 8.1 and 5.7 ± 2.2 years, respectively. The prevalence of ANA positivity, height of titre, and pattern was comparable between patients and controls (p = 0.9, p = 0.8 and p = 0.1, respectively). Anti-dsDNA antibodies and hypocomplementemia were absent in both groups. A significantly higher frequency of positive LAC was observed among patients compared with controls (7.6% vs. 1%, p = 0.02), whereas other aPL were comparable between both groups. None of the patients or controls demonstrated clinically meaningful (medium or high) aPL titres. Conclusion: In our study, schizophrenia was solely associated with LAC. Thus, in the absence of findings suggestive of SLE or APS, routine screening for both diseases is questionable.

Background: Notwithstanding the frequent coexistence of autoimmune thyroid disease (ATD) and primary Sjögren’s Syndrome (SS), it is still unknown how often this association is studied along with its clinical impact. Objective: This study aimed to describe real-world screening practices for ATD in patients with SS and evaluate clinical outcomes of patients with both diagnoses using validated activity and chronicity indexes. Methods: It is a retrospective study of 223 patients with SS attending a tertiary referral center. Patients were under rheumatology surveillance and might have attended other clinics, including internal medicine and/or endocrinology. We registered glandular and extraglandular features, serology and scored the activity (ESSDAI) and the accrual damage (SSDDI) indexes. We also identified any thyroid function tests (TFT) performed, anti-thyroid antibodies, images, and histological thyroid examinations. A single endocrinologist reviewed all data. Results: One hundred forty-nine patients had at least one set of TFT. Younger age was associated with a lack of screening (OR 0.98, 95 % CI 0.95-0.99, p=0.01). Sixty-nine patients had thyroid disease, with the most common diagnosis being ATD (n=24). Patients with ATD had a lower prevalence of Ro/SSA and anti-La/SSB antibodies but similar cumulative SS activity and damage scores. Conclusion: At least one-third of our patients were not screened for thyroid disease, with these patients being the youngest. Thyroid disorders were found in about 40 % of patients with SS, with ATD being the most common. Having SS/ATD did not confer more disease activity or damage accrual. These results highlight the importance of making treating physicians aware of screening for thyroid disease in this population.