Brickman C M, Tsokos G C, Balow J E, Lawley T J, Santaella M, Hammer C H, Frank M M
J Allergy Clin Immunol. 1986 May;77(5):749-57. doi: 10.1016/0091-6749(86)90424-0.
Occasional reports have appeared linking hereditary angioedema (HAE) with autoimmune diseases. We have systematically evaluated 157 patients for manifestations of autoimmunity. Nineteen of these patients (12%) had clinical immunoregulatory diseases including glomerulonephritis (five patients), Sjögren's syndrome (three), inflammatory bowel disease (three), thyroiditis (two), systemic lupus erythematosus (one), drug-induced lupus (one), rheumatoid arthritis (one), juvenile rheumatoid arthritis with IgA deficiency (one), incipient pernicious anemia (one), and sicca syndrome (one). All eight patients with HAE who developed an autoimmune disease with a known human histocompatibility antigen association developed a disease associated with their histocompatibility antigen haplotype (p = 0.014). Although only four patients developed Sjögren's syndrome or sicca syndrome, an additional nine manifested part of the sicca complex. We also found patients with HAE with features suggestive of an immune-based abnormality. These features included idiopathic pancreatitis (three patients), Raynaud's disease (two), partial lipodystrophy (one), chronic chorioretinitis (one), and alopecia universalis (one).
偶尔有报道将遗传性血管性水肿（HAE）与自身免疫性疾病联系起来。我们系统评估了157例患者的自身免疫表现。其中19例患者（12%）患有临床免疫调节疾病，包括肾小球肾炎（5例）、干燥综合征（3例）、炎症性肠病（3例）、甲状腺炎（2例）、系统性红斑狼疮（1例）、药物性狼疮（1例）、类风湿关节炎（1例）、伴有IgA缺乏的青少年类风湿关节炎（1例）、早期恶性贫血（1例）和干燥综合征（1例）。所有8例发生与已知人类组织相容性抗原相关的自身免疫性疾病的HAE患者，所患疾病均与其组织相容性抗原单倍型相关（p = 0.014）。虽然只有4例患者发生了干燥综合征或干燥综合征，但另有9例表现出部分干燥综合征相关症状。我们还发现一些HAE患者具有提示基于免疫异常的特征。这些特征包括特发性胰腺炎（3例）、雷诺病（2例）、部分脂肪营养不良（1例）、慢性脉络膜视网膜炎（1例）和全秃（1例）。
