Young Amber, Namas Rajaie, Dodge Carole, Khanna Dinesh
Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA; University of Michigan Scleroderma Program, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
Division of Occupational and Physical Therapy, University of Michigan, Ann Arbor, MI, USA.
Curr Treatm Opt Rheumatol. 2016 Sep;2(3):252-269. doi: 10.1007/s40674-016-0052-9. Epub 2016 Jul 19.
Systemic sclerosis (SSc) is an autoimmune disease initially recognized by hand involvement due to characteristic Raynaud's phenomenon (RP), puffy hands, skin thickening, and contractures resembling claw deformities. SSc contributes to hand impairment through inflammatory arthritis, joint contractures, tendon friction rubs (TFRs), RP, digital ulcers (DU), puffy hands, skin sclerosis, acro-osteolysis, and calcinosis. These manifestations, which often co-exist, can contribute to difficulty with occupational activities and activities of daily living (ADL), which can result in impaired quality of life. However, despite this knowledge, most diagnostic and treatment principles in SSc are focused on visceral manifestations due to known associations with morbidity and mortality. Treatment of inflammatory arthritis is symptom based and involves corticosteroids ≤10mg daily, methotrexate, tumor necrosis factor inhibitors, tocilizumab, and abatacept. Small joint contractures are managed by principles of occupational hand therapy and rarely surgical procedures. TFRs may be treated similar to inflammatory arthritis with corticosteroids. All patients with RP and DU should keep digits covered and warm and avoid vasoconstrictive agents. Pharmacologic management of RP begins with use of calcium channel blockers, but additional agents that may be considered are fluoxetine and phosphodiesterase 5 (PDE5) inhibitors. DU management also involves vasodilators including calcium channel blockers and PDE5 inhibitors; bosentan has also been shown to prevent DU. In patients with severe RP and active DU, intravenous epoprostenol or iloprost can be used and surgical procedures, such as botulinum injections and digital sympathectomies, may be considered. For those with early diffuse cutaneous SSc needing immunosuppression for skin sclerosis, methotrexate or mycophenolate mofetil can be used, but the agent of choice depends on co-existing manifestations, such as inflammatory arthritis and/or lung involvement. Various pharmacologic agents for calcinosis have been considered but are generally ineffective; however, surgical options, including excision of areas of calcinosis, can be considered. Overall management of hand impairment for all patients with SSc should include occupational hand therapy techniques such as range of motion exercises, paraffin wax, and devices to assist in ADL. Thus, treatment options for the various manifestations contributing to hand impairment in SSc are limited and often modestly efficacious at best. Robust studies are needed to address the manifestations of SSc that contribute to hand impairment.
系统性硬化症（SSc）是一种自身免疫性疾病，最初因特征性雷诺现象（RP）、手部肿胀、皮肤增厚以及类似爪形畸形的挛缩而被发现手部受累。SSc通过炎性关节炎、关节挛缩、肌腱摩擦音（TFRs）、RP、指端溃疡（DU）、手部肿胀、皮肤硬化、肢端骨质溶解和钙质沉着导致手部功能障碍。这些常常并存的表现会导致职业活动和日常生活活动（ADL）困难，进而导致生活质量受损。然而，尽管了解这些情况，但由于已知与发病率和死亡率相关，SSc的大多数诊断和治疗原则都集中在内脏表现上。炎性关节炎的治疗以症状为基础，包括每日≤10mg的皮质类固醇、甲氨蝶呤、肿瘤坏死因子抑制剂、托珠单抗和阿巴西普。小关节挛缩通过职业手部治疗原则进行处理，很少采用外科手术。TFRs的治疗方法可能与炎性关节炎类似，使用皮质类固醇。所有患有RP和DU的患者都应保持手指保暖，避免使用血管收缩剂。RP的药物治疗首先使用钙通道阻滞剂，但也可考虑其他药物，如氟西汀和磷酸二酯酶5（PDE5）抑制剂。DU的治疗也包括血管扩张剂，如钙通道阻滞剂和PDE5抑制剂；波生坦也已被证明可预防DU。对于患有严重RP和活动性DU的患者，可使用静脉注射依前列醇或伊洛前列素，也可考虑外科手术，如肉毒杆菌素注射和指交感神经切除术。对于那些因皮肤硬化需要免疫抑制治疗的早期弥漫性皮肤型SSc患者，可使用甲氨蝶呤或霉酚酸酯，但选择何种药物取决于并存的表现，如炎性关节炎和/或肺部受累。已经考虑了各种治疗钙质沉着的药物，但通常效果不佳；然而，可以考虑手术选择，包括切除钙质沉着区域。所有SSc患者手部功能障碍的总体管理应包括职业手部治疗技术，如关节活动度练习、石蜡疗法以及辅助ADL的器具。因此，针对导致SSc患者手部功能障碍的各种表现的治疗选择有限，而且充其量往往效果一般。需要进行有力的研究来解决导致SSc患者手部功能障碍的各种表现。
