Mediterranean Journal of Rheumatology最新文献

Objectives: High biologic requirement in inflammatory rheumatic diseases (IRD) may indicate a difficult to treat (D2T) condition. In axial spondyloarthritis (axSpA), a consensual definition for this concept is still lacking. Our objectives are to identify the prevalence and characteristics of multiswitcher patients with axSpA, and to analyse the number and reasons for switches.

Methods: This is a longitudinal observational study including patients treated with biologic agents for axSpA. We propose to define D2T patients as those who required more than 2 b/tsDMARD. Patients who did not fulfil this definition were used as controls. The prevalence of multiswitchers was calculated, and characteristics were compared between the two groups. The number and reasons for switches were analysed in the D2T group.

Results: 124 patients were included. The prevalence of multiswitchers was 24.19%. There were no significant differences between the two groups in the age, sex, and comorbidities. D2T patients have more arthritis (p=0.01), and fibromyalgia (p=0.04), and higher disease activity before initiating biotherapy, (BASDAI:p=0.04), (ASDAS:p=0.04). Additionally, the time from diagnosis to the first use of biologic was longer (p=0.04). In the multivariate analysis, the D2T condition was found to be associated with fibromyalgia (p=0.01). Among this group, the prevalence of those treated with 3, 4, and 5 b/tsDMARD was 86%, 9%, and 5%, respectively, the primary and secondary failures were the most common reasons for switching.

Conclusion: We suggest that D2T-axSpA present several characteristics. Identification of this category in large studies is necessary to establish a consensus definition.

Objective: To assess patients' understanding and attitudes towards biosimilars in rheumatoid diseases in Greece.

Methods: A convenience sample of patients with rheumatoid diseases who were members of the largest rheumatoid patient association (RHEUMAZIN) in Greece was selected for this survey. Data on patients' knowledge and attitudes towards biosimilars were collected with a web-based questionnaire.

Results: Among the 309 patients, 60.2% were being treated with bio-originator products, 11% with biosimilars and another 28.8% did not know the type of their biologic therapy. Only 43.7% of the respondents reported they had adequate information about biologic treatments. About 47.9% knew what biosimilars are exactly and 81.2% stated that they need more information about them. The most influential patient information sources about biologics were rheumatologists (88.3%), the Internet (45%), and patient associations (40.5%). Only about 55-60% of the participants thought that biosimilars are comparable to their reference products in terms of safety, effectiveness, quality and regulatory requirements. Patients with adequate knowledge about biosimilars were significantly less concerned about switching from their reference products. A higher education level, previous biosimilar treatment experience, having rheumatologists, patient associations, regulatory bodies and the internet as main information sources, being better informed about the disease, biologic therapies and biosimilars, working and having adequate information about biosimilars were univariately associated with a significantly higher likelihood of having a positive attitude towards biosimilars.

Conclusions: There is an urgent need for patient education about biosimilars in rheumatic diseases in Greece to enhance patient knowledge and ensure informed decisions on biosimilar use.

Late-onset rheumatoid arthritis (LORA) presents a unique diagnostic challenge among older patients, particularly in poorly resourced healthcare settings. As global life expectancy increases, so does the prevalence of LORA, a condition that differs significantly from young-onset rheumatoid arthritis (YORA). This review explores the distinct clinical presentation, differential diagnosis, laboratory findings, and treatment challenges of LORA, emphasising its impact on low- and middle-income countries. The atypical and often acute onset of LORA, coupled with limited access to healthcare and diagnostic tools, contributes to significant diagnostic delays. These delays are compounded by a scarcity of healthcare providers, particularly rheumatologists, and the lack of essential laboratory tests in remote areas. Moreover, older adults often face additional barriers, including poor social support, reluctance to use allopathic medicines, and non-compliance with follow-ups. Effective management of LORA requires not only an understanding of its unique characteristics but also a tailored approach that considers the constraints of resource-limited settings. This review highlights the urgent need for specific guidelines and strategies to improve the diagnosis and management of LORA, thereby addressing the growing healthcare needs of older population in LMICs.

Objective: To describe the clinical profile and treatment outcomes of a longitudinal series of patients with rapidly progressive interstitial lung disease (RP-ILD) associated with anti MDA 5 antibody.

Methods: RP-ILD patients were identified from a prospective cohort of adult patients with idiopathic inflammatory myopathy (IIM). Clinical, demographic, and serological parameters of all patients were recorded using a structured proforma. Rapidly progressive ILD was defined as the development of radiological deterioration and hypoxemia within 3 months of the onset of respiratory symptoms. The diagnosis of RP-ILD was made after high-resolution CT chest and multidisciplinary discussion. RPILD patients were followed up with serial pulmonary function tests (PFT) every 3 months and echocardiography every 6 months.

Results: Among 58 patients with IIM, five patients (3 female, 2 male) had RP-ILD. All the five patients had amyopathic presentation with polyarthritis, negative anti-nuclear antibody (ANA) and strong positivity (3+) for anti MDA 5 antibody by line immunoblot assay. The patients were treated with various combinations of immunosuppressants/immunomodulators. Two patients expired, one had stabilisation of lung function and the other two patients showed improvement of lung function over a median follow up of 24 months. High levels of serum ferritin and LDH were seen in non-survivors.

Conclusion: A clinically amyopathic presentation with polyarthritis, negative ANA and a favourable long-term response to combination immunosuppressive therapy defined the clinico-serological profile and treatment response of our anti MDA5 positive RP-ILD patients.

Introduction: Spondyloarthropathies (SpA) are a family of inflammatory disorders that affect the spine and peripheral joints. The most common representatives are axial Spondylarthritis (axSpA) and Psoriatic Arthritis (PsA). Despite the fact that SpA are characterised by new bone formation, paradoxically, total Bone Mineral Density (BMD) may be decreased.

Methods: An electronic search was conducted on Medline in order to explore the prevalence, risk factors and pathophysiology of Osteoporosis (OP) in SpA patients.

Results: The prevalence of OP globally is reported to be 18.3%. The prevalence of OP in Axial Spondylarthritis (axSpA) patients ranges from 11.7% to 34.4%, while in Psoriatic Arthritis (PsA) patients seems to be similar to the general population. Several factors have been proposed for the development of OP in SpA, such as corticosteroid use and physical inactivity. Moreover, systemic inflammation appears to participate in the pathophysiology of OP with inflammatory cytokines such as Tumour Necrosis Factor (TNF) and Interleukin (IL)-23/IL-17 potentially having a key role in the pathogenesis of bone loss.

Discussion: The current literature points to the direction that OP is an established comorbidity in axSpA. Local or/and systemic inflammation is possibly the main pathway contributing to bone loss in axSpA patients. However, it remains unclear whether OP is an established comorbidity in PsA patients, as it seems that OP is a treatment-associated adverse event.

The spondyloarthritides (SpA) are a group of chronic inflammatory diseases that affect the axial skeleton (ax-SpA), peripheral joints and entheses (p-SpA) and are expressed with several clinical phenotypes such as psoriasis, psoriatic arthritis (PsA), inflammatory bowel disease (IBD), and uveitis. The pathogenesis of SpA involves the pivotal role of tumour necrosis factor alpha (TNFα) and the interleukins (IL) IL-17/IL-23. Their distribution and hierarchy in the affected organs and tissues is differently expressed in SpA. TNFα is expressed in all tissues and organs, while IL-17 and IL-12/IL-23 is lacking from the gut and the axial skeleton respectively. This knowledge is a dilemma for physicians when they must choose a biological therapy. Nowadays, the armamentarium of SpA treatment has been expanded comprising biological therapies such as TNFα inhibitors (TNFαi), IL-17 inhibitors (IL-17i), IL-12/IL-23 inhibitors (IL-12/IL-23i), as well as the Janus Kinase inhibitors (JAKi). Several studies have shown that IL-12/IL-23i are very effective to treat psoriasis, PsA and IBD, but are ineffective in treating ax-SpA. IL-17i are very effective in patients with ax-SpA, psoriasis and PsA, but seem ineffective in IBD. Finally, TNFαi have shown to be effective in all SpA phenotypes with an acceptable toxicity profile. On the other hand, JAKi are also effective in almost all SpA phenotypes, but caution is required for elderly patients who may develop Herpes-Zoster infection, thromboembolic events and malignancies. However, the treatment of SpA is individualised according to the clinical phenotype and after shared decision between patients and physicians.

Introduction: The interferon regulatory factor 7 (IRF7), a member of the IRF family of transcription factors, plays a major role in the regulation of numerous aspects of an immune response and has increasingly been surveyed to determine the aetiology and pathogenesis of systemic sclerosis (SSc). Objective: This study aimed to investigate the transcriptional levels of IRF7 mRNA in peripheral blood mononuclear cells (PBMCs) and the impact of promoter methylation on IRF7 mRNA expression in SSc patients compared to healthy controls.

Methods: PBMCs were obtained from confirmed 40 naïve SSc cases and 20 healthy controls for IRF-7 expression and methylation analysis. mRNA expression was performed using the quantitative real-time polymerase chain reaction (SYBR green method) concerning the housekeeping gene. A promoter methylation profile study was carried out by bisulfite treatment of DNA, followed by methylation-specific polymerase chain reaction (MS-PCR) in SSc cases against controls.

Results: The relative expression analysis revealed that the selected IRF7 gene was upregulated in the patient group compared to healthy controls (p=0.003). In addition, mRNA expression of IRF7 was significantly increased in the limited cutaneous group compared to the diffuse cutaneous group. Moreover, SSc cases had hypomethylated IRF7 promoters compared to controls, and the significant impact of IRF7 promoter methylation on mRNA expression was observed (p=0.001).

Conclusion: IRF7 overexpression in PBMCs from SSc patients may be caused by IRF7 promoter demethylation, and this aberrant expression of IRF7 in SSc might provide a link between the prominent IFN signature and the development of SSc.

Background: Behçet's Disease (BD) is characterised by recurrent aphthous oral and genital ulcers. Vascular involvement is one of the poor prognostic factors. Previously, von Willebrand Factor (VWF) has been detected higher in BD compared with healthy controls. We hypothesised that decreased activity or increased inhibitor levels of ADAMTS-13 may cause increased levels of VWF. Therefore, we investigated ADAMTS-13 in patients with BD.

Methods: We included in total of 42 patients with BD and 41 healthy controls (HCs) in this cross-sectional study. Parametric data with normal distribution were compared with Student's t-test and ANOVA, and nonparametric data with non-normal distribution were compared with Mann Whitney U and Kruskal Wallis tests.

Results: The patients showed lower ADAMTS-13 antigen, lower ADAMTS-13 inhibitor, lower ADAMTS-13 activity, and higher VWF levels compared with HCs. ADAMTS-13 activity was higher in vascular involvement compared with non-vascular involvement (18.26 ± 7.3 vs 12.05 ± 6.49, p=0.012). VWF levels were also similar between vascular and non-vascular subgroups.

Conclusion: Reduced ADAMTS-13 activity and increased VWF levels were detected in BD. This change has not been seen in vascular BD. The underlying pathogenetic mechanisms seem more complex in the formation of thrombosis.

Objective: While ultraviolet light is a well-known environmental trigger of Systemic lupus erythematosus (SLE), it is unknown whether other spectra of light including infrared could affect SLE activity. This study aimed to evaluate the effect of laser hair removal which emits red and infrared light on the activity of SLE.

Method: 20 patients with SLE were enrolled. Six monthly sessions of laser hair removal with Alexandrite laser were done. Demographic and clinical data were recorded. SLE disease activity index (SLEDAI-2K), serum levels of Anti-ds-DNA, C3, C4, and CH50 complement levels, and white blood cell and platelet counts were measured before and after the laser course to investigate the activity of SLE.

Results: Most of the participants were female (90%) with a mean age of 32.65. Prednisolone was the most commonly used medication (95%) followed by hydroxychloroquine (90%). The most common skin types according to Fitzpatrick's classification were types II and III. We found no significant differences between the SLEDAI-2K score, and serum level of Anti-ds-DNA, C4, and C3 before and after the laser hair removal.

Conclusion: Laser hair removal is safe and does not affect the activity of SLE and might not induce disease exacerbation.