Nishiya H, Yoshida H, Tomonari H, Hikita M, Shike T, Takeda Y, Kikuchi T, Kuriyama S, Sakai O
Internal Medicine, Saiseikai Central Hospital, Tokyo, Japan.
Nihon Jinzo Gakkai Shi. 1996 Jan;38(1):40-5.
We have encountered a 49-year-old female with persistent proteinuria and hematuria. Blood pressure, renal function, physical findings and chest X-p showed no abnormality, but blood tests disclosed mild thrombocytopenia, elevated serum ACE activity, serum lysozyme activity and serum IgA concentration. Abdominal echography and CT revealed multiple nodules in her spleen. In order to make a definite diagnosis and exclude the possibilities of malignant lymphoma or metastatic malignant tumor, splenectomy, and open renal biopsy were performed at the same time. On histological examinations, light microscopic appearance of the spleen was characterized by non-caseating granulomas compatible with sarcoidosis. Renal biopsy specimen showed diffuse proliferative glomerulonephritis with positive staining of IgA predominantly located in the mesangial area, compatible with IgA nephropathy. The present case may provide suggestive evidence for a link between sarcoidosis and IgA nephropathy in the pathogenesis. IgA nephropathy complicated by sarcoidosis is rare, and thus is of particular interest because common immunological abnormalities might be considered in the disease process of both diseases. We feel that despite a low index of suspicion, physicians must be alert to the possibility of IgA nephritis associated with sarcoidosis. The literature is reviewed regarding the relationship between IgA nephropathy and sarcoidosis.
我们遇到了一位49岁的女性,她患有持续性蛋白尿和血尿。血压、肾功能、体格检查及胸部X线检查均无异常,但血液检查发现轻度血小板减少、血清ACE活性升高、血清溶菌酶活性升高及血清IgA浓度升高。腹部超声和CT显示她的脾脏有多个结节。为了明确诊断并排除恶性淋巴瘤或转移性恶性肿瘤的可能性,同时进行了脾切除术和开放性肾活检。组织学检查显示,脾脏的光镜表现为符合结节病的非干酪样肉芽肿。肾活检标本显示弥漫性增生性肾小球肾炎,IgA染色阳性,主要位于系膜区,符合IgA肾病。本病例可能为结节病和IgA肾病在发病机制上的联系提供提示性证据。结节病合并IgA肾病很罕见,因此特别值得关注,因为两种疾病的病程中可能存在共同的免疫异常。我们认为,尽管怀疑指数较低,但医生必须警惕与结节病相关的IgA肾炎的可能性。本文就IgA肾病与结节病的关系进行了文献综述。