Rheumatology International最新文献

Background: Patients with radiographic axial spondyloarthritis (r-axSpA) are at an increased risk of osteoporotic fractures compared to the general population.

Aim: To evaluate the clinical utility of trabecular bone score (TBS) compared with bone mineral density (BMD) for predicting major osteoporotic fractures (MOF) in patients with radiographic axial spondyloarthritis (r-axSpA).

Methods: A total of 63 patients with r-axSpA were included. At baseline, lumbar spine DXA, thoracic and lumbar spine X-rays, and basic demographic data were collected. Patients were followed for 3 years, after which new major osteoporotic fractures (MOF) and follow-up spine X-rays were recorded.

Results: During the observation period, 7 patients experienced MOF. Of these, 6 had been classified at high risk based on TBS (TBS ≤ 1.31). On the other hand, only 1 was identified as osteopenic and none as osteoporotic based on BMD T-scores. Strongest relative risk (RR) factors for MOF included a prior MOF (RR = 13.3; p < 0.001) and presence of syndesmophytes/ankylosis (RR = 2.0; p = 0.020). Low TBS (≤ 1.31) was associated with a RR of 4.5 (hence being only a trend due to low numbers). BMD T-score < - 1.0 seemed to be not helpful in this cohort (RR = 0.64).

Conclusion: TBS may provide greater clinical value than BMD in identifying r-axSpA patients at increased risk of MOF. Despite limited sample size, these findings highlight the potential of TBS as a complementary diagnostic tool in routine practice.

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.