Machado Natália Pereira, Camargo Cintia Zumstein, Oliveira Ana Cecília Diniz, Buosi Ana Letícia Pirozzi, Pucinelli Mário Luiz Cardoso, Souza Alexandre Wagner Silva de
Rheumatology Division, Universidade Federal de São Paulo, São Paulo, Brazil.
Sao Paulo Med J. 2010;128(5):306-8. doi: 10.1590/s1516-31802010000500012.
anti-glomerular basement membrane (anti-GBM) antibody syndrome is characterized by deposition of anti-GBM antibodies on affected tissues, associated with glomerulonephritis and/or pulmonary involvement. This syndrome has been described in association with other autoimmune disorders, but as far as we know, it has not been described in association with dermatomyositis and psoriasis.
a 51-year-old man with a history of dermatomyositis and vulgar psoriasis presented with a condition of sensitive-motor polyneuropathy of the hands and feet, weight loss of 4 kg, malaise and fever. On admission, he had been making chronic use of cyclosporin and antihypertensive drugs for three months because of mild arterial hypertension. Laboratory tests showed anemia and leukocytosis, elevated serum urea and creatinine and urine presenting proteinuria, hematuria, leukocyturia and granular casts. The 24-hour proteinuria was 2.3 g. Renal biopsy showed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG) deposits on the glomerular basement membrane by means of direct immunofluorescence, which were suggestive of anti-GBM antibodies. The patient was then treated initially with methylprednisolone and with monthly cyclophosphamide in the form of pulse therapy.
抗肾小球基底膜(anti-GBM)抗体综合征的特征是抗GBM抗体沉积于受累组织,伴有肾小球肾炎和/或肺部受累。该综合征已被描述与其他自身免疫性疾病相关,但据我们所知,尚未见与皮肌炎和银屑病相关的报道。
一名51岁男性,有皮肌炎和寻常型银屑病病史,出现手足感觉运动性多发性神经病、体重减轻4kg、全身不适和发热。入院时,由于轻度动脉高血压,他已长期使用环孢素和抗高血压药物三个月。实验室检查显示贫血和白细胞增多,血清尿素和肌酐升高,尿液出现蛋白尿、血尿、白细胞尿和颗粒管型。24小时蛋白尿为2.3g。肾活检显示新月体坏死性肾小球肾炎,通过直接免疫荧光法在肾小球基底膜上有线性免疫球蛋白G(IgG)沉积,提示抗GBM抗体。患者最初接受甲泼尼龙治疗,并每月进行一次环磷酰胺脉冲治疗。