Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Krankenhausstrasse 12, 91054 Erlangen, Germany.
Ann Rheum Dis. 2010 Jun;69(6):1214-9. doi: 10.1136/ard.2009.120204. Epub 2009 Nov 23.
Hereditary haemochromatosis (HH) is a common autosomal recessive inherited disorder that frequently causes arthritis. The pathophysiology of musculoskeletal involvement is, however, unclear.
To analyse synovial tissue obtained at surgery from patients with HH arthropathy and compare it qualitatively and quantitatively with specimens from patients with rheumatoid arthritis (RA) and osteoarthritis (OA).
Synovial tissue from 15 patients with HH, 20 with RA and 39 with OA was obtained during surgery. A synovitis grading system was used to determine the severity of synovial inflammation. Using immunohistochemistry, synovial neovascularisation and infiltration of macrophages, neutrophils and lymphocytes were quantitatively assessed.
Synovitis in HH arthropathy largely resembles OA with mild infiltration of mononuclear cells and lymphocytes, formation of synovial microvessels and a low degree of synovial hyperplasia. While many features of HH arthropathy are reminiscent of OA, macrophage and especially neutrophil invasion is clearly more prominent in HH arthropathy than in primary OA and mimics features of RA. This finding was observed particularly in synovial tissue of HH samples with marked haemosiderin deposition.
The histological picture of the synovium in HH arthropathy largely resembles a process reminiscent of OA. Neutrophil invasion is, however, markedly increased in HH arthropathy, especially in joints with iron deposition. Accumulation of neutrophils may be crucial for the production of matrix enzymes, which enables cartilage degradation and more rapidly progressive articular damage.
遗传性血色素沉着症(HH)是一种常见的常染色体隐性遗传性疾病,常导致关节炎。然而,肌肉骨骼受累的病理生理学尚不清楚。
分析遗传性血色病性关节炎患者手术中获得的滑膜组织,并与类风湿关节炎(RA)和骨关节炎(OA)患者的标本进行定性和定量比较。
从 15 例 HH 患者、20 例 RA 患者和 39 例 OA 患者的手术中获得滑膜组织。使用滑膜炎分级系统来确定滑膜炎症的严重程度。通过免疫组织化学,定量评估滑膜新生血管形成和巨噬细胞、中性粒细胞和淋巴细胞的浸润。
HH 关节炎的滑膜炎与 OA 非常相似,单核细胞和淋巴细胞浸润轻微,形成滑膜微血管,滑膜增生程度低。虽然 HH 关节炎的许多特征类似于 OA,但巨噬细胞,尤其是中性粒细胞的浸润在 HH 关节炎中明显比原发性 OA 更明显,类似于 RA 的特征。这一发现尤其在 HH 样本中观察到,这些样本中滑膜组织有明显的含铁血黄素沉积。
HH 关节炎滑膜的组织学图像在很大程度上类似于 OA 的过程。然而,HH 关节炎中中性粒细胞浸润明显增加,尤其是在铁沉积的关节中。中性粒细胞的积累可能对基质酶的产生至关重要,这使软骨降解和更快速的关节损伤。