Sissons J G, West R J, Fallows J, Williams D G, Boucher B J, Amos N, Peters D K
N Engl J Med. 1976 Feb 26;294(9):461-5. doi: 10.1056/NEJM197602262940902.
Investigation of the serum complement system in 25 patients with various forms of lipodystrophy showed no abnormality in three patients with total lipodystrophy; a single patient with limb lipodystrophy had evidence of activation of the classical complement pathway. However, of the 21 patients with partial lipodystrophy, 17 had low serum C3, with normal C4 and C2, concentrations, accompanied in 14 by a serum C3 splitting factor indistinguishable from nephritic factor, suggesting activation of the alternative pathway. These abnormalities occurred in 10 patients without clinically overt renal disease. Seven patients had overt nephritis; renal biopsies obtained in six showed mesangiocapillary (membranoproliferative) nephritis in all. Thus, the majority of patients with partial lipodystrophy have hypocomplementemia. Although nephritis may not invariably develop, the high rate of mesangiocapillary nephritis in these patients suggests that complement activation via the alternative pathway predisposes to the development of this form of glomerular disease.
对25例各种类型脂肪营养不良患者的血清补体系统进行研究发现，3例全身脂肪营养不良患者无异常；1例肢体脂肪营养不良患者有经典补体途径激活的证据。然而，在21例部分脂肪营养不良患者中，17例血清C3降低，C4和C2浓度正常，其中14例伴有与肾炎因子难以区分的血清C3裂解因子，提示替代途径激活。这些异常出现在10例无明显临床肾病的患者中。7例有明显肾炎；6例患者的肾活检均显示为系膜毛细血管性（膜增生性）肾炎。因此，大多数部分脂肪营养不良患者有补体血症。虽然肾炎不一定会发生，但这些患者中系膜毛细血管性肾炎的高发生率表明，通过替代途径激活补体易导致这种形式的肾小球疾病的发生。
