Mediterranean Journal of Rheumatology最新文献

Objective: To explore efficacy of medications, namely steroids, ursodeoxycholic acid (UDC), methotrexate (MTX), azathioprine, mycophenolate mofetil (MMF), and infliximab in the treatment of hepatic sarcoidosis (HS).

Methods: We searched for the patients using ICD codes for sarcoidosis (ICD-10: D86) and granulomatous hepatitis (ICD-10: K75.3) at Louisiana State University, Shreveport, and generated 150 unique medical record numbers. We retrospectively reviewed notes, labs, imaging, and medications, and used descriptive statistics to calculate percentages.

Results: 47 patients had a diagnosis of HS. 72% of patients had ALP elevation of >200. 36 (76%) patients received steroids, 20 (42%) had MTX, 5 (10%) had azathioprine, 5 (10%) had rituximab, 12 (25%) had infliximab, 3 (6%) had UDC, 21 (44%) had MMF. 12 patients received a combination of prednisone with either MTX, azathioprine, MMF, infliximab, or rituximab. Treatment response was measured based on ALP improvement. 55% of patients responded to prednisone, 45% to MTX, 40% to azathioprine, 60% to rituximab, 66% to infliximab, 47% to MMF, and 30% to UDC.

Conclusion: Majority of the patients presented with ALP elevation of >200. Liver biopsy was performed in only 27% of the patients. Despite being one of the recommended initial therapies, UDC was used in only 3% of patients. Steroids were most commonly used. Among all the steroid-sparing agents, infliximab showed the best efficacy. Similarly, MTX showed improvement, but it was generally avoided due to the risk of hepatotoxicity. Azathioprine, MMF, and rituximab were used either in combination or as sole therapies and have shown improvement in ALP.

Objectives: This study aimed to evaluate and compare atherosclerotic risk factors and carotid intima-media thickness (cIMT) between psoriasis patients with and without arthritis.

Methods: Data on demographics, clinical characteristics, comorbidities, and treatments were collected in the medical charts. Laboratory assessments, including lipid profile and fasting glucose, were performed, along with cIMT measurements via ultrasound.

Results: A total of 127 participants were analysed, including 49 controls and 78 psoriasis patients (47 with psoriatic arthritis and 31 without). Psoriasis patients exhibited a higher frequency of diabetes (OR=2.3; 95% CI=1.009-5.08) and hypertension (OR=6.8; 95% CI=1.7-30.8) compared to controls. Additionally, cIMT values were significantly elevated in psoriasis patients compared to controls (median 0.68 mm vs. 0.57 mm, p=0.001). However, no significant differences in traditional atherosclerotic risk factors or cIMT measurements were observed between psoriasis patients with and without arthritis (all p>0.05).

Conclusion: Psoriasis patients, regardless of arthritis status, exhibit increased atherosclerotic risk compared to controls. However, in this sample, it was not possible to prove that the presence of arthritis further exacerbates this risk.

Yao syndrome, an autoinflammatory disease associated with specific NOD2 gene variants, presents with a wide range of symptoms, including fever, dermatitic rashes, polyarthritis, abdominal pain/bloating, and sicca symptoms. The initial clinical manifestation remains widely variable. The first-line treatment options remain unknown due to limited knowledge of the pathophysiology and the absence of robust literature. We report a case of a 36-year-old woman diagnosed with Yao syndrome, presenting with postprandial bloating, followed by polyarthralgia and low-grade fever. After experiencing reactions to treatments, including anaphylaxis secondary to anakinra and canakinumab, the patient showed improvement with upadacitinib and leflunomide, resulting in better control of her symptoms.

Silicone is a chemical compound that is composed of one silicone atom and two atoms of oxygen. It has a variety of clinical applications such as breast and joint implants, intraocular lenses and others. Silicone is associated with a variety of autoimmune/inflammatory syndromes induced adjuvant, called ASIA syndrome, among them is lupus development. A 48year-old woman who had silicone breast implantation bilaterally 4 months earlier, presented to us with abrupt, extensive erythematosus skin manifestations affecting her face, nose and lips. She presented annular lesions affecting the upper back and erythematosus lesions affecting the palms of both hands. She manifested also epidermal necrolysis involving the breasts, areolas and nipples bilaterally. Laboratory evaluation revealed low white blood cells, positive antinuclear antibodies at a titre of 1/320, fine speckled pattern and Ro(SSA) and Smith(Sm) antibodies. The patient satisfied the classification criteria for lupus and the proposed criteria for silicone induced ASIA syndrome. She was treated with prednisone 40mg/day plus hydroxychloroquine 400mg/day with excellent results. Thus, through the above case we review and discuss the relevant literature of silicone induced lupus. This is a unique described case with such extensive and severe skin manifestations induced from silicone. To this end, physicians must be aware and recognise these symptoms and signs of patients exposed to silicone and treat them promptly and appropriately.

Adult-onset Still's disease (AOSD) is a rare autoinflammatory disorder characterized by spiking fevers, arthralgia, and a transient salmon-pink rash, with an incidence of 0.16-0.4 per 100,000. AOSD shares overlapping clinical and immunological features with systemic juvenile idiopathic arthritis (sJIA), supporting a disease continuum and shared treatment approaches. The COVID-19 pandemic has impacted AOSD care, with SARS-CoV-2 infection and vaccination occasionally triggering disease flares, necessitating adaptive management strategies. Driven by innate immune dysregulation and overproduction of proinflammatory cytokines (IL-1, IL-6, IL-18), AOSD presents in systemic and articular phenotypes, with severe complications like macrophage activation syndrome (MAS), fulminant hepatitis, and parenchymal lung disease. Diagnosis, based on Yamaguchi or Fautrel criteria and biomarkers (ferritin, IL-18), is challenging due to nonspecific symptoms. Biologic therapies (anakinra, canakinumab, tocilizumab) achieve remission in 80-90% of systemic cases. This review synthesises diagnostic challenges, novel biomarkers (e.g., gasdermin D), and emerging therapies (e.g., IL-18 binding protein), emphasising precision medicine and future research needs.

Objective: N-of-1 trials constitute single-patient, randomised, crossover and often, double-blind clinical trials, where each patient serves as his/her own control. The implementation of n-of-1 trials propels us towards the practice of patient-centric medicine, while exhibiting multiple additional advantages for rheumatology, including the identification of the most appropriate treatment for each patient, improved response, outcomes and quality of life, fewer adverse events, and reduced economic costs. The design employs similar aspects to randomised clinical trials in order to maintain scientific rigor, while producing clinically relevant treatment outcomes, tailored to each patient.

Methods: For the purpose of this review, we searched PubMed and clinicaltrials.gov for n-of-1 trials or series conducted on patients with rheumatic diseases until August 2024.

Results: N-of-1 trials can facilitate clinical decisions and evaluate the efficacy of medications, lifestyle interventions, or adjuvant treatments (i.e. for pain), while focusing on disease-specific outcomes or comorbidities (cachexia, obesity, etc.). In this review, the advantages and limitations of n-of-1 trials in rheumatology are discussed and trials performed on patients with rheumatic diseases are presented.

Conclusions: Employing the n-of-1 design in everyday clinical practice consists of the epitome of patient-centred medicine, greatly benefiting patients and clinicians, facilitating deprescribing, and reducing the economic burden of pharmacotherapy.

Objective: To evaluate the potential of Matrix Metalloproteinase-9 (MMP-9) as a diagnostic marker for psoriatic arthritis (PsA) and to investigate the correlation between MMP-9 levels and PsA disease activity.

Patients and method: A total of 72 subjects participated in this cross-sectional study, consisting of 43 patients diagnosed with Psoriatic Arthritis (PsA) and 29 healthy control subjects. The Composite Psoriatic Disease Activity Index and Disease Activity in Psoriatic Arthritis were utilised to evaluate the disease activity levels in PsA patients. To measure serum levels of MMP-9 the quantitative sandwich enzyme-linked immunosorbent assay method was applied. Results: The mean age of PsA patients is 43.81 ± 12.82 years, with a mean BMI of 29.46 ± 5.91 kg/m², significantly higher than healthy subjects (p < 0.01). Oneway ANOVA indicates a significant difference in serum MMP-9 levels among active PsA, inactive PsA, and healthy controls [F(2, 68) = 21.15, p < 0.001]. Serum MMP-9 levels significantly differ between PsA groups and healthy controls (p < 0.001). Pearson correlation shows no link between serum MMP-9 levels and PsA activity. MMP-9 shows strong diagnostic potential for distinguishing PsA patients from healthy controls, with an AUC of 0.88 (p < 0.001).

Conclusions: This study demonstrated that MMP-9 shows promising potential as a diagnostic marker for PsA. but no significant correlation between serum MMP-9 levels and PsA disease activity. These findings highlight the need for further research involving a larger cohort of PsA patients to assess whether MMP-9 could play a complementary role in PsA diagnosis.

Introduction: High levels of stress among healthcare workers may impact the quality of care provided to patients. Chronic stress can lead to conditions such as fatigue and fibromyalgia. We aimed to assess stress levels and identify healthcare workers at risk for occupational burnout and fibromyalgia.

Methods: We conducted a cross-sectional study among healthcare professionals in the Abu Dhabi region. Physicians, nurses and other healthcare workers were invited to take part in an anonymous survey via email. The questionnaire included validated scales for workplace stress as well as for diagnostic symptoms for fibromyalgia.

Results: Among the 254 respondents, the majority were females (73.2%) aged 20 to 59 years. Females reported higher stress levels than males (mean scores 6.7 vs 5.7; on a scale of 0 to 10), and physicians reported higher stress than nurses (mean scores 7.2 vs 5.8). More than half of the professionals were considering quitting due to work-related stress; highest among of resident physicians (65%). Fibromyalgia criteria were met by 28.3% of respondents, predominately females, with a significant association observed between higher stress levels and a fibromyalgia diagnosis.

Conclusions: High levels of stress were reported by healthcare professionals, especially women. Stress was associated with fatigue, fibromyalgia, and burnout leading to the intention of leaving clinical work. Workplace changes and stress reduction support programs are needed urgently to protect this vital workforce.

Rheumatoid arthritis (RA) is a chronic autoimmune inflammatory disease with substantial morbidity and socioeconomic burden. Early diagnosis and treatment are crucial to mitigate disease progression and preserve joint function. Current treatment strategies encompass non-pharmacological and pharmacological approaches, with disease-modifying antirheumatic drugs (DMARDs) being the cornerstone of pharmacotherapy. Novel immunomodulating drugs have revolutionised RA management by targeting specific cytokines or intracellular signalling pathways involved in disease pathogenesis. Evidence-based guidelines recommend biologics as second-line therapy for patients for whom conventional DMARDs have failed. While Tumour Necrosis Factor Inhibitors (TNFi) have traditionally been favoured, recent evidence suggests nuanced responses based on patient characteristics and treatment history are more effective. Moreover, Janus Kinase (JAK) Inhibitors emerge as a promising therapeutic option, demonstrating comparable efficacy to bDMARDs in clinical practice. Despite significant advancements, challenges in optimising RA treatment include variable treatment responses and safety concerns. Future research aims to refine treatment strategies, personalise therapeutic approaches, and elucidate disease mechanisms to improve outcomes for RA patients. The evolving landscape of immunomodulating drugs offers diverse therapeutic options for RA management. This article provides a comprehensive review of RA therapy, focusing on novel immunomodulating drugs.

Background: Anti-transcription intermediary factor 1-gamma (anti-TIF1γ) antibodies are closely associated with Inflammatory myositis (IIM) and cancer-associated myositis.

Objective: Description of clinical characteristics of anti-TIF1γ(+) IIM patients in a Greek population.

Material & methods: Retrospective analysis with 113 IIM cases between 2001 and 2024 was performed and clinical and laboratory data were collected. Disease manifestations and outcomes were compared between anti-TIF1γ-positive and -negative groups.

Results: Twenty patients (17.7%) were anti-TIF1γ(+), of which 70% were women. The mean age was 64.8 ± 12.5 years vs 59.61 ± 12.81 of anti-TIF1γ(-) patients (p>0.05). Anti-TIF1γ was strongly associated with Dermatomyositis (DM) (95%, p < 0.001) and more severe cutaneous involvement (mean CDASI=27.35 ± 15.01 vs 14 ± 12.25 p =0.0015). Malignancy was significantly more frequent in the anti-TIF1γ(+) group (60% vs. 20.4%, p = 0.001), with an odds ratio of 5.84 (95% CI 2.09-16.31). Logistic regression identified anti-TIF1γ positivity as independent predictor of malignancy. Interstitial Lung Disease was uncommon among anti-TIF1γ(+) cases (15%, p = 0.004), while dysphagia was far more prevalent (55% vs. 22.6%, p = 0.001). Muscle power (MMT-8score) and CPK levels did not differ significantly, and survival was lower in anti-TIF1γ(+) patients (55.7% vs. 82.6% p<0.001), associated with malignancy.

Conclusions: In our cohort, anti-TIF1γ antibodies define a distinct IIM subset marked by severe skin disease, high malignancy risk, and poorer survival, supporting comprehensive cancer screening and tailored immunosuppressive treatment. This study describes this phenotype in a Greek cohort, aligning with international evidence and highlighting the need for collaborative studies.