Rheumatology International最新文献

The most important complication of familial Mediterranean fever (FMF) are AA amyloidosis and amyloid nephropathy. Amyloid nephropathy typically follows a slow, indolent course. However, "amyloid storm" is a recently described acute condition, characterized by sudden onset of severe proteinuria and rapidly progressive renal failure with markedly acute-phase reactants, progressing over days to weeks into end-stage renal disease (ESRD). Amyloid storm has been rarely reported in the literature. Most cases have occurred in patients with known FMF-related AA amyloid nephropathy. However, it has also been reported as the initial presentation of AA amyloidosis. No standardized treatment guidelines exist, but management has included supportive care, colchicine, and interleukin (IL)-1 blockade. This report presents the first case of amyloid storm associated with ulcerative colitis in the literature. The patient exhibited impaired oral intake and fatigue, alongside acute kidney injury, spot urine proteinuria of approximately 100 g/day, and elevated C-reactive protein levels (CRP 118 mg/L). The diagnosis of AA amyloidosis was made after a kidney biopsy. In addition to starting haemodialysis, the patient was treated with anakinra and colchicine given the clinical presentation indicative of an amyloid storm. Subsequently, the acute-phase reactants decreased and the patient's general health improved, but haemodialysis was still required. After the patient discontinued anakinra treatment, he was re-hospitalized with complaints of fatigue and weakness but died on day 71 after the amyloid storm diagnosis. In our literature review, we identified a total of 36 patients with amyloid storm, 29 of whom were from two separate original studies. According to our review, the frequency of FMF-associated amyloid storm is 5.5%. The majority of patients were male, and while FMF was the most common cause of AA amyloidosis, ankylosing spondylitis, non-FMF autoinflammatory diseases, and ulcerative colitis were among the other causes. Our analysis revealed that infections were the most common triggering factor of an amyloid storm. ESRD rates were found to be up to 67%, and mortality rates ranged from 33 to 67%. This case report aims to highlight the relatively new and little-known concept of amyloid storm, which, despite its rarity, discusses its clinical features, laboratory findings, treatment options, and outcomes based on our case as well as a few cases in the literature.

Temporal artery biopsy (TAB) and, more recently, temporal artery ultrasound are recommended for the diagnosis of giant cell arteritis (GCA). The inter-rater agreement for TAB is poorly reported, and agreement for ultrasound is variable. A prospective study, the ECHORTON study, evaluated a diagnostic strategy for GCA that used temporal artery ultrasound as the first-line diagnostic test and TAB for ultrasound-negative patients. Clinical expertise served as the reference method. We propose assessing the inter-rater agreement in interpreting TAB and ultrasound images collected in this study. From 2016 to 2020, 165 patients with high suspicion of GCA were enrolled in the ECHORTON study at four general hospitals and two university hospitals. Pathologists and vascular medicine experts independently reviewed TAB and ultrasounds, respectively. The TAB samples were stained with eosin and silver and classified as positive, negative, or equivocal for GCA. Ultrasound results were considered positive when halos were detected around the lumen of both temporal arteries using 9-4 to 18-6 MHz linear probes. This study involved double-blind analysis of 4384 sections from 77 TAB, and 5781 images from 132 ultrasound scans. Kappa coefficients were 0.75 [95% CI: 0.56-0.94] for temporal artery biopsy and 0.73 [95% CI: 0.56-0.90] for temporal artery ultrasound. The reproducibility of interpretations showed heterogeneity across centres, with agreement ranging from fair to excellent. Overall, both TAB and ultrasound demonstrated good inter-rater agreement for GCA diagnosis, though agreement levels varied from fair to excellent across hospitals. Trial registration: The ECHORTON study was registered in ClinicalTrials.gov under the number NCT02703922 on March 3, 2016.

Knee flexion contracture (FC), or loss of passive knee extension (KE), is common in knee osteoarthritis (OA) and linked to worse pain, function, and earlier joint replacement. Its relationship with structural progression over time on magnetic resonance imaging (MRI) remains unclear. This study evaluated whether baseline loss of knee extension was associated with longitudinal structural changes on MRI. A retrospective cohort analysis was conducted using data from 1018 participants (1131 knees) from an Osteoarthritis Initiative (OAI) sub-cohort. Baseline KE was measured using a goniometer. Structural changes were assessed annually over four years using the MRI Osteoarthritis Knee Score (MOAKS). Associations between baseline KE loss and longitudinal MOAKS outcomes were evaluated using ANCOVA, adjusting for demographic, radiographic and clinical covariates. Baseline KE loss correlated with subsequent worsening of MOAKS-evaluated pathologies in central structures, including meniscal hypertrophy (Year 1 [p = 0.001]; Year 4 [p = 0.042]), medial meniscal extrusion (Year 3 [p = 0.02]; Year 4 [p = 0.03]), lateral meniscal extrusion (Year 4 [p = 0.04]), and larger tibial bone marrow lesion (BML) size (Year 3 [p = 0.01]). Baseline KE loss also correlated with improved MOAKS scores in anterior and central structures, with reduced lateral meniscal extrusion (Year 3 [p = 0.004]; Year 4 [p = 0.046]), tibial BML size (Year 1 [p = 0.03]; Year 2 [p = 0.02]; Year 4 [p = 0.015]), tibial BML number (Year 2 [p = 0.006]; Year 4 [p = 0.01]), and tibial cystic BML percentage (Year 1 [p = 0.04]; Year 2 [p = 0.01]; Year 4 [p = 0.01]) and femur (Year 2 [p = 0.01]). Baseline KE was associated with structural changes in knee OA over four years including worse central meniscal scoring, but better BML scores and meniscal extrusion in the anterior compartments. Lost KE in those with OA may predict longitudinal patterns of regional structural progression.

Despite numerous reports of finger pain by young healthy smartphone users, no study to date evaluated the impact of hand joint diseases on smartphone use. The study assessed the prevalence and determinants of smartphone-related pain and discomfort in patients with hand osteoarthritis (HOA), rheumatoid arthritis (RA), or psoriatic arthritis (PsA). In this monocentric cross-sectional study, patients with HOA, RA, or PsA completed a dedicated survey assessing smartphone use, pain, paraesthesia, and discomfort. Clinical, radiographic, and patient-reported outcomes were collected. Associations with smartphone-related symptoms were evaluated using univariate and multivariable logistic regression, reporting odds ratios (OR) and 95% confidence intervals (95%CI). A total of 377 patients were included (174 RA, 102 PsA, 101 HOA; mean age 61.2 years; 73.2% women). Among the 343 smartphone users, 25-30% reported pain or discomfort, with significantly higher prevalence in HOA patients (40-45%, p<0.001). Symptoms were more frequent in women (p<0.05) and were associated with higher VAS pain (OR 1.36, 95%CI 1.22-1.52), higher VAS disease activity (OR 1.32, 95%CI 1.17-1.48), greater tender joint count (OR 1.15, 95%CI 1.06-1.24), and longer daily smartphone use (p<0.05). Radiographic erosions and deformities were not significantly associated. Functional scores (FIHOA, HAQ, Cochin) correlated with symptoms. Smartphone-related pain and discomfort are common in rheumatic hand diseases, especially HOA, and are linked to patient-reported disease activity and functional impairment. These findings highlight the interest to include digital device use in functional assessments of hand arthritis.

Social networks are widely used in the healthcare system for education, research, and professional networking. However, its adoption and impact in Central Asia, particularly among medical professionals, remain underexplored. This study assesses social media usage patterns, motivations, and perceived challenges among healthcare professionals in Kazakhstan, with a focus on professional engagement, information-seeking behaviours, and the need for structured training. A cross-sectional online survey was conducted among healthcare professionals in Kazakhstan from November 2022 to January 2023 after extensive pilot-testing by a multi-professional team. The questionnaire comprised multiple-choice and open-ended questions, and Likert scale answers to explore, social media preferences for professional engagement, and future possibilities. Among 147 respondents (M:F ratio = 1:1.17, median age = 32 years), social media adoption was nearly universal (97.96%, n = 144). The primary reasons for usage were knowledge acquisition (81.94%, n = 118), skill development (79.16%, n = 114), and maintaining social connections (68.05%, n = 98). YouTube and Instagram were the most frequently accessed platforms. Despite the perceived professional utility of social media, 75% (n = 108) of respondents felt overwhelmed, and only 65.27% (n = 94) considered it a secure means of communication.69% (n = 99) had never attended training on optimal social media use for professional growth, and 98.61% (n = 142) expressing willingness to participate in future trainings. The social media landscape among healthcare professionals in Kazakhstan reveals nuanced patterns of platform utilisation. The perceived utility of these platforms is tempered by acknowledged challenges, highlighting a critical need for structured guidance and comprehensive professional training frameworks tailored to the unique digital communication environment of Central Asian healthcare settings.