Department of Pathology, UT Southwestern Medical Center, Dallas, Texas, 75390, USA.
Long School of Medicine, UT Health San Antonio, San Antonio, Texas, 78229, USA.
Acta Neuropathol Commun. 2018 Feb 20;6(1):11. doi: 10.1186/s40478-018-0512-6.
Terminal complement complex deposition in endomysial capillaries detected by a C5b-9 immunostain is considered a diagnostic feature for dermatomyositis. However, we found widespread microvascular C5b-9 reactivity in a substantial subset of muscle biopsies with denervation changes, and in nerve biopsies of peripheral neuropathies, particularly in patients with diabetes. It is unclear whether the presence of C5b-9 deposition signifies active immune-mediated vascular injury that requires immune suppression therapy. We retrospectively identified 63 nerve biopsies in patients with a documented history of diabetes, 26 of which had concomitant muscle biopsies, as well as 54 control nerve biopsies in patients without a documented diabetes history, 18 of which had concomitant muscle biopsies. C5b-9 immunostain was performed on all cases. 87% of the nerve biopsies and 92% of the muscle biopsies from diabetic patients showed microvascular C5b-9 reactivity, compared to 34% and 50% in non-diabetic patients. The differences were statistically significant (p < 0.0001 for nerve and p = 0.002 for muscle). The C5b-9 reactivity was generally proportional to the extent of microvascular sclerosis in diabetic patients, but unrelated to inflammation or vasculitis. C5b-9 deposition in micro-vasculature in both muscle and nerve is therefore a common feature in patients with diabetic neuropathies and may have diagnostic utility. Precaution needs to be taken before using muscle capillary C5b-9 reactivity as evidence of myositis.
肌内膜毛细血管中末端补体复合物沉积被认为是皮肌炎的一个诊断特征,用 C5b-9 免疫染色可检测到。然而,我们发现大量神经病变患者的肌肉活检中存在广泛的微血管 C5b-9 反应性,包括神经活检,尤其是糖尿病患者。目前尚不清楚 C5b-9 沉积是否表明存在需要免疫抑制治疗的活性免疫介导的血管损伤。我们回顾性地确定了 63 例有明确糖尿病病史的患者的神经活检,其中 26 例同时进行了肌肉活检,54 例无明确糖尿病病史的患者的神经活检,其中 18 例同时进行了肌肉活检。所有病例均进行了 C5b-9 免疫染色。与非糖尿病患者相比,糖尿病患者的神经活检(87%)和肌肉活检(92%)显示微血管 C5b-9 反应性,而非糖尿病患者的神经活检(34%)和肌肉活检(50%)。差异具有统计学意义(神经:p < 0.0001;肌肉:p = 0.002)。在糖尿病患者中,C5b-9 反应性通常与微血管硬化的程度成正比,但与炎症或血管炎无关。因此,糖尿病神经病变患者的肌肉和神经微血管中 C5b-9 沉积是常见特征,可能具有诊断价值。在将肌肉毛细血管 C5b-9 反应性作为肌炎的证据之前,需要谨慎。