Axford J S, Bomford A, Revell P, Watt I, Williams R, Hamilton E B
Department of Rheumatology Research, University College and Middlesex School of Medicine, London, UK.
Arthritis Rheum. 1991 Mar;34(3):357-61. doi: 10.1002/art.1780340314.
Genetic hemochromatosis, a disorder of iron metabolism, results in the deposition of massive amounts of iron in the tissues. Arthropathy is one of a number of clinical features associated with the disease. Characteristic radiographic features in the wrist and hand have been reported, and an increased incidence of severe hip disease has been observed. In this study, hip radiographs of 112 patients with genetic hemochromatosis and arthritis were reviewed, and histologic examination of 2 femoral heads was performed. Twenty-eight of the 112 patients (25%) had evidence of arthritis of the hip joint. In 23 (82%) of the 28 patients, this feature was thought to be associated with osteoarthritis; 2 of these patients had an atypical arthropathy associated with radiolucency of the femoral head and histologic features of atypical stripping of the cartilage from the subchondral bone. These atypical features were not thought to be due to avascular necrosis, pyrophosphate-associated arthropathy, apatite-associated deposition arthritis, or osteoarthritis, but may be typical of genetic hemochromatosis and possibly the result of increased susceptibility to shearing forces at the bone-cartilage interface. In 5 of the 28 patients (18%), chondrocalcinosis was the sole abnormal finding on radiography. Ten of the 28 patients eventually required hip surgery, which confirms the severity of the hip disease associated with genetic hemochromatosis.
遗传性血色素沉着症是一种铁代谢紊乱疾病,会导致大量铁在组织中沉积。关节病是与该疾病相关的众多临床特征之一。已有报道称手腕和手部具有特征性的放射学表现,并且观察到严重髋关节疾病的发病率有所增加。在本研究中,对112例患有遗传性血色素沉着症和关节炎患者的髋关节X光片进行了回顾,并对2个股骨头进行了组织学检查。112例患者中有28例(25%)有髋关节关节炎的证据。在这28例患者中的23例(82%),该特征被认为与骨关节炎有关;其中2例患者有一种非典型关节病,伴有股骨头透亮区以及软骨从软骨下骨非典型剥脱的组织学特征。这些非典型特征被认为并非由缺血性坏死、焦磷酸相关关节病、磷灰石相关沉积性关节炎或骨关节炎所致,而可能是遗传性血色素沉着症的典型表现,并且可能是骨 - 软骨界面处剪切力易感性增加的结果。28例患者中有5例(18%),放射学检查的唯一异常发现是软骨钙质沉着症。28例患者中有10例最终需要进行髋关节手术,这证实了与遗传性血色素沉着症相关的髋关节疾病的严重性。