Department of Medicine, University of California San Diego, San Diego, California.
Department of Molecular Medicine, The Scripps Research Institute, La Jolla, California.
Haemophilia. 2019 Sep;25(5):851-858. doi: 10.1111/hae.13802. Epub 2019 Jun 14.
Evidence suggests that toxic iron is involved in haemophilic joint destruction.
To determine whether joint iron deposition is linked to clinical and imaging outcomes in order to optimize management of haemophilic joint disease.
Adults with haemophilia A or haemophilia B (n = 23, ≥ age 21) of all severities were recruited prospectively to undergo assessment with Hemophilia Joint Health Scores (HJHS), pain scores (visual analogue scale [VAS]) and magnetic resonance imaging (MRI) at 3T using conventional MRI protocols and 4-echo 3D-UTE-Cones sequences for one affected arthropathic joint. MRI was scored blinded by two musculoskeletal radiologists using the International Prophylaxis Study Group (IPSG) MRI scale. Additionally, UTE-T2* values of cartilage were quantified. Correlations between parameters were performed using Spearman rank correlation. Two patients subsequently underwent knee arthroplasty, which permitted linking of histological findings (including Perl's reaction) with MRI results.
MRI scores did not correlate with pain scores or HJHS. Sixteen joints had sufficient cartilage for UTE-T2* analysis. T2* values for cartilage correlated inversely with HJHS (r = -0.81, P < 0.001) and MRI scores (r = -0.52, P = 0.037). This was unexpected since UTE-T2* values decrease with better joint status in patients with osteoarthritis, suggesting that iron was present and responsible for the effects. Histological analysis of cartilage confirmed iron deposition within chondrocytes, associated with low UTE-T2* values.
Iron accumulation can occur in cartilage (not only in synovium) and shows a clear association with joint health. Cartilage iron is a novel biomarker which, if quantifiable with innovative joint-specific MRI T2* sequences, may guide treatment optimization.
有证据表明，有毒铁参与了血友病性关节破坏。
确定关节铁沉积与临床和影像学结果是否相关，以便优化血友病性关节疾病的管理。
前瞻性招募了所有严重程度的血友病 A 或血友病 B 成人（n=23，年龄≥21 岁），对其进行评估，包括血友病关节健康评分（HJHS）、疼痛评分（视觉模拟评分 [VAS]）和使用常规 MRI 方案和 4 回波 3D-UTE-Cone 序列的 3T 磁共振成像（MRI），以评估一个受累的关节病关节。MRI 由两位肌肉骨骼放射科医生使用国际预防研究组（IPSG）MRI 量表进行盲法评分。此外，还对软骨的 UTE-T2* 值进行了量化。使用 Spearman 秩相关分析参数之间的相关性。随后有两名患者接受了膝关节置换术，这使得可以将组织学发现（包括 Perl 反应）与 MRI 结果联系起来。
MRI 评分与疼痛评分或 HJHS 无相关性。16 个关节有足够的软骨进行 UTE-T2分析。软骨的 T2 值与 HJHS（r=-0.81，P<0.001）和 MRI 评分（r=-0.52，P=0.037）呈负相关。这出乎意料，因为在骨关节炎患者中，UTE-T2* 值随着关节状况的改善而降低，这表明铁是存在的，并导致了这种影响。软骨的组织学分析证实了软骨细胞内的铁沉积，与低 UTE-T2* 值相关。
铁积累可发生在软骨（不仅在滑膜中），并与关节健康有明显关联。软骨铁是一种新的生物标志物，如果可以通过创新的关节特异性 MRI T2* 序列进行定量，可能有助于指导治疗优化。
