Mediterranean Journal of Rheumatology最新文献

Objective: To differentiate the nailfold capillaroscopy (NFC) findings in patients with MCTD-ILD and SSc-ILD and correlate the NFC changes and lung functions among them.

Methods: In this observational study from Oct 2020 to Oct 2022, 27 patients with MCTD-ILD and 27 patients with SSc-ILD were included. NFC was performed using Jiangsu Jiahua, JH 1004, China. Statistical analysis was conducted using IBM SPSS software, version 26, and tests including Mann-Whitney U-test, student t-test, chi-square test, or Fisher's exact test were used to compare between groups.

Results: In this study, major capillaroscopic changes were more frequent in SSc-ILD group (92%) than in MCTD-ILD group (72.3%), with normal capillaries seen in 7.4% of MCTD-ILD cases. The mean FVC was higher in SSc-ILD group compared to MCTD-ILD group, and patients with capillary loss had a lower mean FVC. Loss of capillaries was more frequent in SSc-ILD group, while dilated capillaries were predominantly observed in MCTD-ILD group. A significant association was found between the severity of restriction in spirometry and NFC.

Conclusion: There is an important role for NFC in detecting the severity of lung involvement, as the grading of restrictive severity in spirometry is strongly associated with capillaroscopic abnormalities.

Objective: Patients with rheumatoid arthritis (RA) are associated with low bone mineral density (BMD). Chronic comorbidities such as type II diabetes mellitus have shown to affect BMD parameters in patients with RA. Hypertension (HT) is a chronic disease and its coexistence with RA can alter bone health. The aim of this study was to investigate if HT affected BMD parameters in RA patients diagnosed for the first time.

Methods: Patients with the diagnosis of RA who underwent BMD studies formed the study population. Patients with HT were sorted from this population and formed a separate group. Healthy controls were drawn from subjects who came for a check-up. BMD was done with the GE Lunar DPX machine. Mean T Scores at spine, femur neck and total femur were recorded. Data from the three groups were analysed and compared. Linear regression analyses were performed.

Results: Analysis suggested that the age had inverse and BMI had direct correlation with BMD T scores in all groups. The additional diagnosis of HT in RA patients was associated with higher BMD as compared to patients with RA, but lower than controls. R² values were 0.341, 0.402 and 0.436 for mean T scores at spine, femur neck and femur total respectively. Figures from multiple regression analysis suggest that BMI alone did not explain the higher T score values in HT patients.

Conclusion: Additional morbidity of HT in RA patients negates the porotic effect of RA as judged by bone densitometry. Hence, BMD reports should be read with caution in these patients.

Background: Familial Mediterranean fever is a hereditary autoinflammatory disease affecting mainly Arabs, Turks, Armenians, and Jews with genotype-phenotype heterogeneity, presenting as recurrent episodes of fever along with polyserositis and rash. To date, more than 370 mutations in the MEFV gene have been recognized to cause the disease.

Methods: We conducted a retrospective cohort study involving 124 patients in Hebron, Palestine, diagnosed with FMF at the Al-Ahli, and Palestinian Red Crescent Society (PRCS) Hospitals.

Results: The median age of diagnosis was five years, presenting as abdominal pain (76.6%), fever (67.7%), joint pain and arthritis. Regarding MEFV gene mutations, we had 62 patients (50%) with heterozygous genotypes, 40 patients (32.3%) with homozygous phenotypes, 21 patients (16.9%) with compound heterozygous genotypes, and one was a missing state. Regarding variant frequencies, M694V was the most common one (43.4%), followed by E148Q (15.6%), V726A (5.7%), A744S (4.1%), and R202Q (4.1%). Positive family history was detected in 59 patients (54.6%), and there was no significant difference in zygosity regarding characteristics, consanguinity, and family history.

Conclusions: We affirm in this study of 124 children with FMF, abdominal pain, followed by fever, joint pain and arthritis were the main manifestations. Further, M694V, E148Q, V726A, A744S, and R202Q were the most frequent mutations, and carrying the M649V mutations is associated with a predisposition to other comorbidities. We believe that this study gives a pervasive overview of FMF in Palestinian patients. Looking forward, future studies on a larger number of patients could precisely highlight the genotype-phenotype association among FMF patients.

Objective: MicroRNAs (miRNAs) are involved in a range of pathological and biological processes. Vascular involvement is an important complication associated with morbidity and mortality in Behçet's disease (BD). In this study, we aimed to evaluate the expression levels of miR-195, miR-424, miR-10b, miR-103a-3p, and miR-542-3p in Turkish patients with BD, and their possible association with vascular involvement and clinical activity.

Methods: This cross-sectional study included 61 BD patients and 25 age- and sex-matched healthy individuals. The patients were categorised into two groups based on the presence or absence of vascular involvement. Demographic data, disease duration, disease activity, and medical treatments were recorded. Disease activity was evaluated using the Behçet's Disease Current Activity Form (BDCAF) and the Behçet's Syndrome Activity Scale (BSAS). The expression levels of miRNAs were measured using real-time quantitative polymerase chain reaction (RT-qPCR).

Results: The comparison of the clinical features of BD patients with and without vascular involvement revealed no significant difference. However, the expression levels of miR-195, miR-424, miR-10b, miR-103a-3p, and miR-542-3p were significantly higher in BD patients than in healthy controls (p<0.001, p<0.001, p=0.010, p<0.01, p=0.039, respectively). Moreover, the expression level of miR-195 was significantly higher in vasculo-Behçet patients than in the other groups (p=0.0318). However, no significant association was found between the expression levels of miR-195 and clinical activity.

Conclusion: Our study results indicated elevated serum levels of miR-195 in BD patients, which may be associated with vascular involvement. Therefore, miR-195 could potentially serve as a biomarker for the diagnosis and monitoring of vasculo-Behçet's disease.

Polymyalgia Rheumatica (PMR) and Giant Cell Arteritis (GCA) are chronic inflammatory disorders that usually affect older people. Although the aetiology of these diseases remains unknown, genetic, environmental, and immune factors have been implicated. Specific cytokines such as the IL-6, IL-1β, IL-12, IL-17, and interferon -γ seem to play an essential role. The diagnosis of the disease is usually based on clinical manifestations and the use of histology or imaging, while disease monitoring is based on physical examination, laboratory, and imaging findings. However, there is the unmet need in identifying possible biomarkers that could help the diagnosis and the monitoring as well. The present study aims to investigate the epidemiological, clinical, and immunological characteristics of PMR and/or GCA patients in the region of northwest Greece and to evaluate the role of specific molecules associated with the pathogenesis of the diseases, giving evidence to possible future biomarkers.

Introduction: Methotrexate (MTX) is the bed rock of inflammatory arthritis management. However, intolerance is a limiting factor for drug optimisation and retention. There is data to suggest subcutaneous (SC) MTX is better tolerated. It is less clear whether this strategy is effective in those where the oral preparation is inefficacious and its potential to avoid escalation to biologic therapy.

Objectives: To analyse the reasons for switching to SC MTX in a real-world setting, clinical outcomes achieved and proportion requiring biologic prescription.

Materials and methods: A retrospective survey of patients prescribed SC MTX in a university teaching hospital identified 352 patients. 298 switched from oral to SC MTX- 164 stopped oral MTX due to side effects, 134 stopped due to inefficacy, and 54 started SC MTX as first line therapy. 103 patients progressed to biologic therapy. Rheumatoid arthritis (RA): DAS-28 improved from a mean of 4.06 (0.63-8.06) to 2.83 (0.14-7.32) following the switch (p<0.0001). Psoriatic arthritis (PsA): total joint count improved from a mean of 7 (0-42) to 2 (0-25) (p<0.0001). Swollen joint count improved from a mean of 2 (0-26) to 1 (0-6) (p=0.09).

Discussion: SC MTX is an effective solution for RA and PsA, irrespective of whether oral MTX is inefficacious or intolerable. Where oral MTX was ineffective, a switch to SC achieved low disease activity despite multi-morbidity, long disease course and protracted oral MTX exposure. This intervention prevented over two-thirds of patients requiring biologics. SC MTX is a durable strategy with excellent disease outcomes and substantial economic benefits.

Sjögren's syndrome (SS) is a systemic chronic autoimmune disorder that classically affects the exocrine glands. Only 15% of the patients with primary SS (pSS) develop extraglandular symptoms involving the lungs, kidneys, joints, nervous system, and skin. Hypokalaemic paralysis is a rare presentation. The most common cause of hypokalaemia is distal renal tubular acidosis. The prevalence of clinically significant lung involvement in pSS is 9-20 %. Primary SS is an indolent disease leading to increased morbidity and poor quality of life. We present a case of a 40-year-old female with severe hypokalaemic paralysis, tubulointerstitial nephritis, and lung involvement as the initial presentation of catastrophic pSS without sicca symptoms. The course of hospitalisation was complicated by ventilator-associated pneumonia. She was managed with broad spectrum antibiotics, five sessions of plasma exchange and alternate-day haemodialysis followed by oral glucocorticoids and intravenous cyclophosphamide. To the best of our knowledge, this is the first case of catastrophic presentation of pSS with a favourable outcome.

Arthritis is an unusual manifestation of paraneoplastic syndrome, appearing in a variety of cancers, including pulmonary and colorectal. It can often pose a diagnostic challenge to physicians, since it may be difficult to distinguish from more commonly encountered rheumatic illnesses. Moreover, synchronous cancers are rare and unexpected in patients with symmetrical polyarthritis. Hypertrophic pulmonary osteoarthropathy is to be considered in patients with polyarthritis and lung neoplasia. The aim of this report is to highlight the case of a patient presenting with paraneoplastic polyarthritis, which led to identifying the presence of underlying synchronous lung and colorectal malignancies. Lymph node biopsy was performed raising suspicion of Caplan's syndrome but lung lobectomy confirmed adenocarcinoma. Rheumatologists should be reacquainted with rheumatic manifestations in malignant diseases.