Bollow Matthias
Klinik für diagnostische und interventionelle Radiologie, Augusta-Kranken-Anstalt Bochum, Bergstraße 26, 44791, Bochum, Deutschland.
Ruhr-Universität Bochum, Bochum, Deutschland.
Radiologe. 2021 May;61(5):470-482. doi: 10.1007/s00117-021-00842-2. Epub 2021 Apr 1.
Progressive systemic scleroderma (PSS) and mixed connective tissue disease (MCTD) represent vasculitic autoimmune diseases from the group of collagenoses with manifestations in various organ systems such as the skin, the internal organs and the joints.
To present the atypical arthritis patterns of the hands in PSS and MCTD that differ from those in rheumatoid arthritis (RA) and psoriatic arthritis (PsA) in the context of clinical and serologic findings and in consideration of the classification of PSS and MCTD.
Narrative review based on the current literature on the subject from the radiological and rheumatological point of view.
In PSS, combinations of acral soft tissue atrophy, nonreactive acro-osteolysis, and interstitial calcifications can be visualized by projection radiography, which in the final stage can lead to a scleroderma claw hand. Digital pharmacoangiography of the hands can be used to reliably diagnose manifest vascular occlusions of the digital arteries. MCTD is characterized by various overlapping symptoms of at least two systemic autoimmune diseases and most frequently presents in the hand with symmetrical involvement of the PIP (proximal interphalangeal), MCP (metacarpophalangeal) and wrist joints with the manifestation of so-called "puffy fingers".
The presented morphological atypical arthritis patterns of the hands in PSS and MCTD differ considerably from the typical patterns in the hands in RA and PsA. MRI is useful to diagnose early stages and pharmacoangiography can be used to differentiate between temporary and manifest digital vascular occlusions.
进行性系统性硬化症(PSS)和混合性结缔组织病(MCTD)是胶原病组中的血管炎性自身免疫性疾病,在皮肤、内脏器官和关节等多个器官系统有表现。
结合临床和血清学检查结果,并考虑PSS和MCTD的分类,阐述PSS和MCTD中手部非典型关节炎模式,这些模式与类风湿关节炎(RA)和银屑病关节炎(PsA)中的手部关节炎模式不同。
从放射学和风湿病学角度,基于关于该主题的当前文献进行叙述性综述。
在PSS中,通过X线投影可显示肢端软组织萎缩、非反应性肢端骨质溶解和间质钙化的组合,在疾病终末期可导致硬皮病爪形手。手部数字药物血管造影可用于可靠诊断指动脉的明显血管闭塞。MCTD的特征是至少两种系统性自身免疫性疾病的各种重叠症状,最常见于手部,表现为近端指间关节(PIP)、掌指关节(MCP)和腕关节的对称性受累,并出现所谓的“腊肠指”。
PSS和MCTD中所呈现的手部形态学非典型关节炎模式与RA和PsA中手部的典型模式有很大差异。MRI有助于诊断早期阶段,药物血管造影可用于区分暂时性和明显的指血管闭塞。