Clinicopathological characteristics of familial SLE patients with lupus nephritis.

This study aimed to analyze the clinicopathological characteristics of familial SLE patients with lupus nephritis (LN). A total of 136 Chinese patients with lupus nephritis diagnosed by renal biopsy, including 34 familial patients and 102 sporadic cases, were recruited. Their demographic information, age of onset, disease duration, clinical features, laboratory data and histological manifestations were compared. The first-degree relatives (17 sibling cases and eight parent-offspring cases) of familial SLE patients were primarily affected. The prevalence of fever, rash and arthritis in familial subjects was higher than that in sporadic subjects. Familial patients had lower platelet counts and higher low-density lipoprotein. In familial patients, class V lupus nephritis was less common. After adjusted with the Benjamini and Hochberg procedure, however, fever was the only feature occurring significantly and more frequently in familial patients (58.8% vs 26.5%, P = 0.001). Moreover, SLEDAI and other clinicopathological features did not differ significantly between the two groups. Multivariate analysis showed that a higher prevalence of fever was an independent predictor of familial SLE. Most clinicopathological features in familial SLE patients were not significantly different from those in sporadic patients; the severity of LN in familial SLE patients was similar to that of sporadic cases. Thus, familial SLE may not be a different clinical entity.