Open Access Rheumatology-Research and Reviews最新文献

Introduction: Budd-Chiari syndrome (BCS) is a rare disorder characterized by hepatic outflow obstruction. It can be classified as primary or secondary BCS. Common causes of BCS include myeloproliferative diseases, infections, malignancies, and systemic autoimmune illnesses. Systemic lupus erythematosus (SLE) can be complicated with BCS. However, only a few case reports have described the uncommon occurrence of BCS as a primary presentation of SLE.

Case presentation: We report the case of a 32-year-old female patient who presented with progressive abdominal distension of four months. On the abdominal CT scan, the left and middle hepatic veins were not visualized; the right hepatic vein and intrahepatic IVC had luminal narrowing; and there was caudate lobe enlargement suggestive of Budd-Chiari syndrome (BCS). Six months after the diagnosis of BCS, the patient developed other clinical features suggestive of systemic lupus erythematosus (SLE) and was finally diagnosed with SLE.

Conclusion: Acquired or inherited thrombotic conditions are the most common underlying causes of Budd-Chiari syndrome. Systemic lupus erythematosus (SLE) is the most common cause of secondary APS and most patients present with Budd-Chiari syndrome as a manifestation of APS after the diagnosis of SLE. In rare cases, such as the current case, Budd-Chiari syndrome can present even before the diagnosis of SLE. Hence, we would like to emphasize that Budd-Chiari syndrome can be an initial presentation of SLE.

Background: Abnormal liver function tests (LFTs) can indicate cirrhosis or liver cancer leading to mortality among systemic sclerosis (SSc) patients. No recent studies have investigated the clinical predictors of an abnormal LFT in SSc. We aimed to determine the incidence of abnormal LFT (including from hepatitis and cholestasis) and to identify its clinical predictors in SSc patients.

Methods: An historical cohort was conducted on 674 adult SSc patients who attended the Scleroderma Clinic, Khon Kaen University, between January 2012 and November 2019 and who underwent routine screening for LFT. A Cox regression was used to analyze the clinical predictors of abnormal LFT.

Results: Four hundred and thirty cases, representing 4190 person-years, had abnormal LFTs (viz, from hepatitis, cholestasis, and cholestatic hepatitis) for an incidence rate of 10.2 per 100 person-years. The respective incidence of hepatitis, cholestasis, and cholestatic hepatitis was 20.5, 12.9, and 20.4 per 100 person-years. The respective median first-time detection of hepatitis, cholestasis, and cholestatic hepatitis was 3.0, 5.9, and 2.8 years, and none had signs or symptoms suggestive of liver disease. According to the Cox regression analysis, the predictors of an abnormal LFT in SSc were elderly onset of SSc (hazard ratio (HR) 1.02), alcoholic drinking (HR 1.74), high modified Rodnan Skin Score (mRSS) (HR 1.03), edematous skin (HR 2.94), Raynaud's phenomenon (HR 1.39), hyperCKaemia (HR 1.88), and methotrexate use (HR 1.55). In contrast, current sildenafil treatment (HR 0.63) and high serum albumin (HR 0.70) were protective factors.

Conclusion: Occult hepatitis, cholestasis, and cholestatic hepatitis can be detected in SSc patients using LFT screening, especially in cases of early disease onset. The long-term outcome is uncertain, and more longitudinal research is required.

Infections remain one of the leading causes of death in systemic lupus erythematosus (SLE), despite awareness of factors contributing to increased susceptibility to infectious diseases in SLE. Clinicians report challenges and barriers when encountering infection in SLE as certain infections may mimic a lupus flare. There are no evidence-based practice guidelines in the management of fever in SLE, with suboptimal implementations of evidence-based benefits related to infectious disease control and/or prevention strategies in SLE. Vigilance in identifying an opportunistic infection must be stressed when confronted by a diagnostic challenge during a presentation with a febrile illness in SLE. A balanced approach must focus on management of infections in SLE, and reduction in the glucocorticoids dose, given the need to control lupus disease activity to avoid lupus related organ damage and mortality. Clinical judgement and application of biomarkers of lupus flares could reduce false positives and overdiagnosis and improve differentiation of infections from lupus flares. Further precision-based risk and screening measures must identify individuals who would benefit most from low dose immunosuppressive therapy, targeted immune therapy, and vaccination programs.

There is a significant variation in symptoms and clinical presentation of connective tissue disorders (CTD) associated with interstitial lung disease (ILD) (CTD-ILD). This presents difficulties in the diagnosis and treatment of CTD-ILD. Early detection and treatment of CTD-ILD using a multidisciplinary approach have been shown to enhance patient outcomes. This exercise aims to explore clinical components to develop a screening tool for pulmonologists for early detection of CTD in ILD and to provide a framework for a multidisciplinary approach in managing CTD-ILD. This in turn will lead to early treatment of CTD-ILD in collaboration with rheumatologists. A panel of 12 leading rheumatologists from the Middle East and North Africa (MENA) region met virtually to select the most relevant clinical findings to aid in identifying CTD-ILD. Twelve panellists opted to investigate seven of the most common inflammatory autoimmune disorders. The panel discussed how to improve the early detection of CTD-ILD. Clinical characteristics were categorized, and a nine-item questionnaire was created. A biphasic algorithm was developed to guide early referral to a rheumatologist based on the presence of one of nine clinical features of CTD (Phase 1) or the presence of CTD-specific antibodies (Phase 2). A brief questionnaire has been developed to serve as a simple and practical screening tool for CTD-ILD detection. Additional research is needed to validate and evaluate the tool in longitudinal cohorts.

The early identification of patients with inflammatory arthritis and their referral to rheumatologists in order to establish a diagnosis and to start treatment plays a crucial role in patient outcomes. However, it is recognized that a large proportion of patients with inflammatory arthritis are diagnosed very late, losing the opportunity to start treatment in the very early stages of disease, resulting in a worse prognosis. This delay depends on several factors related to the patient, the disease, socio-demographic and health system aspects. Over time, several strategies have been developed and implemented at different levels aiming to overcome such barriers and to reduce the time from the onset of the symptoms until the diagnosis and start of adequate treatment. In this non-systematic comprehensive review, we will describe the main barriers in the identification of patients with inflammatory arthritis at different levels. We will also discuss the different strategies that have been implemented with the objective to overcome the recognized barriers and their impact in the reduction of delays.

Introduction/objectives: This rapid evidence assessment (REA) was conducted to assess the burden of weight-bearing joint osteoarthritis in the developing countries of Africa and the Middle East.

Methods: Our REA methodology used a standardized search strategy to identify observational studies, published between January 1, 2010, and April 23, 2020, reporting on outcomes pertaining to the epidemiology and humanistic or economic burden of weight-bearing osteoarthritis. Relevant data from the included studies were used for qualitative analysis.

Results: Among the 20 publications reporting on knee osteoarthritis in 10 countries in Africa and the Middle East, 2 also reported on hip, and 1 on foot osteoarthritis. Prevalence of symptomatic/radiographic knee OA was 9-14% among rheumatology outpatients and 31-34% among those with mixed etiology osteoarthritis. Prevalence of knee OA diagnosed by magnetic resonance imaging was 70% among patients ≥40 years of age attending a hospital in Saudi Arabia. Quality-of-life outcomes were reported in 16 publications and suggested a substantial humanistic burden of osteoarthritis, including worse pain, function, and quality of life, and more depression; comparisons between studies were hampered by the variety of tools and scoring scales used, however. No studies reported on economic outcomes.

Conclusion: This REA indicates a substantial burden of osteoarthritis in weight-bearing joints in Africa and the Middle East, consistent with publications from other regions of the world.

Purpose: Depression is the most common psychiatric disorder associated with rheumatoid arthritis (RA). However, little is known about its prevalence and risk factors among Saudi patients, specifically. Therefore, this study sought to determine the prevalence and predictors of depression in patients with RA in Saudi Arabia.

Patients and methods: A cross-sectional study was conducted with patients registered at the Saudi Charitable Association for Rheumatic Diseases. Inclusion criteria were that the patients either met the American College of Rheumatology 1987 revised criteria for the classification of RA or the 2010 RA classification criteria. Demographic data and clinical variables were collected, and Beck's 21-item Depression Inventory was used to assess for depression.

Results: Of the 210 participants with RA, 171 were women (81.4%), and 39 were men (18.6%). The prevalence of depression was 68%. There were significant relationships between age, gender, marital status, and having depression. Rheumatoid factor (RF) was positive in 144 participants (68.6%), which positively correlated with the risk of having depression (P value < 0.001). Moreover, depression severity correlated with age, gender, marital status, RF positivity, and prolonged disease duration.

Conclusion: Based on the results, depression is highly prevalent in Saudi patients with RA, especially those with positive RF and those who are female, middle-aged, and divorced. Early detection and treatment of depression in patients with RA is highly recommended to improve their quality of life and avoid unfavorable effects on RA clinical progression.

Complement system (CS) dysregulation is a key factor in the pathogenesis of different autoimmune diseases playing a central role in many immune innate and adaptive processes. Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by ta breach of self-tolerance leading to a synovitis and extra-articular manifestations. The CS is activated in RA and seems not only to mediate direct tissue damage but also play a role in the initiation of RA pathogenetic mechanisms through interactions with citrullinated proteins. Interstitial lung disease (ILD) represents the most common extra-articular manifestation that can lead to progressive fibrosis. In this review, we focused on the evidence of CS dysregulation in RA and in ILD, and highlighted the role of the CS in both the innate and adaptive immune responses in the development of diseases, by using idiopathic pulmonary fibrosis as a model of lung disease. As a proof of concept, we dissected the evidence that several treatments used to treat RA and ILD such as glucocorticoids, pirfenidone, disease modifying antirheumatic drugs, targeted biologics such as tumor necrosis factor (TNF)-inhibitors, rituximab, tocilizumab, and nintedanib may act indirectly on the CS, suggesting that the CS might represent a potential therapeutic target in these complex diseases.

Purpose: Systemic Sclerosis related Interstitial Lung Disease (SSc-ILD) is the most common clinical manifestation of SSc with a high morbidity and mortality rate. However, the Thorax High-Resolution Computed Tomography (HCRT) as the gold standard diagnostic tool for SSc-ILD is not widely equipped in health-care facilities. Recently, specific autoantibody examination (anti-topoisomerase-1 (ATA), anti-Th/To antibody, and anti-fibrillarin) has been studied and used for SSc-ILD diagnosis. This study aims to evaluate the diagnostic performance of specific autoantibody examination among SSc-ILD.

Patients and methods: This retrospective study reviews data from local dedicated SSc database (Sclerosis Systemic Register System Development Electronic Medical Record) which were collected between March 2019 and August 2021. Population of this study include adult inpatients and outpatients at Dr. Hasan Sadikin General Hospital, who have been diagnosed with SSc based on ACR/EULAR 2013 criteria, which met inclusion and exclusion criteria. The SSc patients were grouped into SSc-ILD and SSc non-ILD based on HRCT and tested for SSC-ILD specific autoantibody test (ATA, anti-Th/To antibody, and anti-fibrillarin) to obtain the diagnostic performance (sensitivity, specificity, and positive- and negative-predictive value).

Results: A total of 74 subject grouped into 47 SSc-ILD and 27 SSc-non ILD patients. ATA validity test results showed 85.1% sensitivity, 19.2% specificity, 65.6% PPV, and 41.7% NPV. Anti-Th/To antibody obtained 27.7% sensitivity, 88.9% specificity, 81.3% PPV, and 41.4% NPV. The anti-fibrillarin validity test result showed a 12.8% sensitivity, 96.3% specificity, 85.7% PPV, and 38.8% NPV. The combination of the three parameters had 95.7% sensitivity, 18.5% specificity, 67.1% PPV, and 71.4% NPV.

Conclusion: The combination of the SSc-ILD specific autoantibody test and HCRT is expected to detect all affected patients. Based on these results, SSc-ILD autoantibody-specific test can be used as an alternative examination for screening and diagnosis in health-care facilities that are not equipped with HRCT.

Background: Ocular morbidities associated with rheumatoid arthritis (RA) have not received much attention in Africa, particularly in sub-Saharan Africa. They are among the commonest (40%) extra-articular organ involvement in RA. If undiagnosed, there is a potential risk of them causing visual impairment or blindness. There is no documented study in Uganda on the magnitude of eye disorders among RA patients.

Aim: To determine the spectrum of eye disorders and associated factors among patients with RA attending Mulago National Referral Hospital.

Methods: A hospital based cross-sectional study was conducted among adults with RA attending the rheumatology clinic between July 2021 and September 2021. Clinical and sociodemographic data were collected, and ophthalmologic examinations were performed on all consenting participants. Modified Poisson regression with robust standard error was used to determine factors associated with eye disorders.

Results: Overall, 105 patients with RA were enrolled, of which, 53 (50.5%) had eye disorders. The commonest disorder (54.7%, n=29) was dry-eye syndrome. Factors that were significantly associated with eye disorders were age 36-55 years (aPR 1.56, p=0.015), duration of RA >5 years (aPR 1.81, p=0.001), use of hydroxychloroquine >5 years (aPR 1.77, p=0.041), dose of oral steroids >10 mg/day (aPR 1.49, p=0.034), and history of both diabetes and hypertension (aPR 1.87, p=0.014).

Conclusion: The prevalence of eye disorders among patients with RA was high, with the commonest being dry-eye syndrome. We recommend that ocular examinations be performed on every patient at the time of RA diagnosis for early detection of eye disorders.