Puéchal X, Said G, Hilliquin P, Coste J, Job-Deslandre C, Lacroix C, Menkès C J
Hôpital Cochin, Université René Descartes, Paris, France.
Arthritis Rheum. 1995 Nov;38(11):1618-29. doi: 10.1002/art.1780381114.
To examine the clinicopathologic features of the noncompressive neuropathies in rheumatoid arthritis (RA).
We studied 32 patients with RA and peripheral neuropathy whose nerve and/or muscle biopsy specimens exhibited necrotizing vasculitis. Morphologic analysis of nerve specimens included light and electron microscopy studies and teased fiber preparation. Survival was evaluated, and the prognostic values of clinical, biologic, and pathologic features were assessed by Cox proportional hazards model. A prognostic assessment based on the significant variables was devised to estimate the probability of survival of any individual patient.
Epi- and/or perineurial vasculitis was observed with the same frequency in the 17 patients with sensory and motor deficit and the 15 patients with sensory neuropathies and was associated with axonal degeneration of an average of 77.7% of the nerve fibers. The mean followup was 7.2 years, and the overall survival rate at 5 years was 57%. A full prolonged remission of the vasculitis was observed in 53% of the patients; relapse occurred in 25%. The factors correlated with mortality, in decreasing order of significance, were clinical cutaneous vasculitis (P = 0.0003), neuropathy affecting 3 or 4 limbs (P = 0.03), and depressed level of C4 (P < 0.05). The prognostic assessment indicated a wide range of 5-year probabilities of survival, from < 1% to 93%.
Necrotizing vasculitis is responsible for the different patterns of noncompressive neuropathies in RA, including mononeuritis multiplex and distal symmetric sensory or sensorimotor neuropathy. Cutaneous vasculitis, multifocal neuropathy, and depressed C4 level were the 3 independent variables which best predicted mortality. We propose a prognostic assessment according to these variables, to stratify patients to receive more aggressive or less aggressive therapy.
研究类风湿关节炎（RA）中非压迫性神经病变的临床病理特征。
我们研究了32例患有RA和周围神经病变的患者，其神经和/或肌肉活检标本显示有坏死性血管炎。神经标本的形态学分析包括光镜和电镜研究以及神经纤维分离制片。评估生存率，并通过Cox比例风险模型评估临床、生物学和病理特征的预后价值。设计基于显著变量的预后评估方法以估计任何个体患者的生存概率。
在17例有感觉和运动功能障碍的患者以及15例有感觉神经病变的患者中，观察到神经外膜和/或神经束膜血管炎的频率相同，且与平均77.7%的神经纤维轴突变性相关。平均随访7.2年，5年总生存率为57%。53%的患者出现血管炎完全长期缓解；25%出现复发。与死亡率相关的因素，按显著性递减顺序依次为临床皮肤血管炎（P = 0.0003）、累及3或4个肢体的神经病变（P = 0.03）和C4水平降低（P < 0.05）。预后评估显示5年生存概率范围很广，从<1%到93%。
坏死性血管炎是RA中非压迫性神经病变不同模式的原因，包括多发性单神经炎和远端对称性感觉或感觉运动性神经病变。皮肤血管炎、多灶性神经病变和C4水平降低是最能预测死亡率的3个独立变量。我们根据这些变量提出一种预后评估方法，以便对患者进行分层，使其接受更积极或较不积极的治疗。
