Stylianou Kostas, Maragkaki Eleftheria, Tzanakakis Michael, Stratakis Stavros, Gakiopoulou Hariklia, Daphnis Eugene
Nephrology Department, University Hospital of Heraklion, Heraklion, Greece.
Pathology Department, National and Kapodistrian University of Athens, Athens, Greece.
Case Rep Nephrol Dial. 2014 Dec 3;5(1):44-8. doi: 10.1159/000369924. eCollection 2015 Jan-Apr.
We present the case of a patient with IgG4-related disease, which manifested in an asynchronous manner as vitiligo, cholecystitis, sialadenitis, lymphadenopathy, facial palsy and kidney dysfunction. The patient underwent a renal biopsy, and a presumptive diagnosis of lupus nephritis was made due to compatible clinical and immunological findings. The biopsy revealed IgG4-related kidney disease with severe interstitial nephritis and membranous nephropathy. Corticosteroids treatment restored all disease manifestations. We bring this case to the attention of the nephrologists because of the protean, asynchronous, multisystemic nature of the disease that necessitates a multidisciplinary approach, a low threshold for kidney biopsy and a high index of suspicion for making the correct diagnosis and treatment.
我们报告一例IgG4相关性疾病患者，该疾病以白癜风、胆囊炎、涎腺炎、淋巴结病、面神经麻痹和肾功能不全等异步方式表现。患者接受了肾活检，由于临床和免疫学表现相符，初步诊断为狼疮性肾炎。活检显示为IgG4相关性肾病，伴有严重的间质性肾炎和膜性肾病。皮质类固醇治疗使所有疾病表现均得到恢复。我们将此病例提请肾脏病学家注意，因为该疾病具有多变、异步、多系统的性质，需要多学科方法、较低的肾活检阈值以及较高的怀疑指数才能做出正确的诊断和治疗。
