Objective: To report three kindreds of familial membranous nephropathy and describe its clinical and pathological characteristics.
Methods: 6 patients with renal biopsy proved familial membranous nephropathy from 3 kindreds were described. Their clinical and pathological data were compared with 33 patients who were also suffering from familial membranous nephropathy from 16 kindreds documented in PubMed and 30 sporadic patients who were collected in our hospital with membranous nephropathy.
Results: Compared with the patients documented in the literatures, the patients were older and the ratios of male and female patients in this series were equal. In both series, the pathological lesions were severe. Compared with sporadic patients with membranous nephropathy, the patients in familial form had severer pathological lesions. There were no differences among all patients in clinical manifestations, such as proteinuria, hypertension and renal function. The major clinical symptom in familial membranous nephropathy patients was nephritic syndrome.
Conclusions: The present study first reported the kindreds of familial membranous nephropathy in China. The clinical manifestations of familial membranous nephropathy in our patients, as well as in other familial cases reported to date, are similar with those in the non-familial membranous nephropathy patients, but the pathological changes in the familial cases were severer than those in the non-familial. To distinguish the familial form from idiopathic membranous nephropathy, it is essential to understand the genetic factors underlying this disease.