Kallenberg C G
University Hospital Groningen, Department of Clinical Immunology, The Netherlands.
Curr Opin Rheumatol. 1995 Nov;7(6):568-73. doi: 10.1097/00002281-199511000-00017.
Connective tissue diseases (CTDs) frequently present with one or only a few symptoms, which does not allow prompt diagnosis. Raynaud's phenomenon is one of those symptoms. However, only a minority of patients who present with Raynaud's phenomenon develop a CTD. Prognostic factors for the future development of CTD in such patients are older age at presentation, more severe Raynaud's phenomenon, the presence of antinuclear antibodies, and abnormal patterns on nailfold capillary microscopy. Some patients have overlapping symptoms of various CTDs. Mixed connective tissue disease (MCTD) is the prototype of such an overlapping syndrome. However, during follow-up, most patients with MCTD develop a specific CTD, either scleroderma, systemic lupus erythematosus, rheumatoid arthritis, or combinations of those illnesses. Primary pulmonary hypertension is one of the leading causes of death in MCTD. Its treatment is insufficient, although continuous prostacyclin infusion may provide some relief. New therapies such as nitric oxide and combined heart-lung transplantation in an early stage should be explored. The autoimmune response to small nuclear ribonucleoproteins, which is highly characteristic for MCTD, interestingly shows cross-reactivity with retroviral antigens, and the cooccurrence of human T cell lymphotropic virus type I and HIV infection with MCTD has been reported. This suggests that those viruses, possibly by molecular mimicry, play a role in the induction of the disease. Fibrotic conditions related to silicone exposure still evoke much interest. However, most recent data do not substantiate a role for silicone gel breast implants in the development of autoimmune CTDs.
结缔组织病（CTD）常常仅表现出一种或少数几种症状，这使得难以迅速做出诊断。雷诺现象就是其中一种症状。然而，仅有少数出现雷诺现象的患者会发展为CTD。此类患者未来发展为CTD的预后因素包括发病时年龄较大、雷诺现象更严重、存在抗核抗体以及甲襞毛细血管显微镜检查出现异常模式。一些患者具有多种CTD的重叠症状。混合性结缔组织病（MCTD）就是这种重叠综合征的典型代表。然而，在随访过程中，大多数MCTD患者会发展为一种特定的CTD，即硬皮病、系统性红斑狼疮、类风湿关节炎或这些疾病的组合。原发性肺动脉高压是MCTD的主要死亡原因之一。其治疗并不充分，尽管持续输注前列环素可能会提供一些缓解。应探索诸如一氧化氮和早期联合心肺移植等新疗法。对小核糖核蛋白的自身免疫反应是MCTD的高度特征性表现，有趣的是，它与逆转录病毒抗原存在交叉反应，并且已有报道人类嗜T细胞病毒I型和HIV感染与MCTD同时出现。这表明这些病毒可能通过分子模拟在疾病的诱发中起作用。与硅暴露相关的纤维化情况仍然备受关注。然而，最新数据并未证实硅凝胶乳房植入物在自身免疫性CTD发展中的作用。
