CLINICOPATHOLOGICAL CORRELATIONS IN LUPUS NEPHRITIS

Abstract

In the past 6 years 28/31 children with SLE have had lupus nephritis. Biopsy showed diffuse proliferative glomerulonephritis (DPGN) in 17, focal glomerulonephritis (FGN) in 5 and membranous glomerulonephritis (MGN) in 6.At the time of diagnosis of nephritis, children with DPGN had evidence of associated extrarenal disease (22/22) and 5/17 with DPGN had the nephrotic syndrome. MGN presented with nephrotic syndrome (4/6). Renal function was depressed in DPGN (5/17), FGN (0/4), and MGN (1/6). Active disease was associated with ↑ANA and ↓c'4, followed by ↓c'3, and ↑ split products of fibrin. Serological changes occurred prior to onset of clinical symptoms. Prednisone (2 mg/kg) and Azathioprine (2 mg/kg) produced complete or partial remission in DPGN (14/ 17), MGN (5/6) and FGN (1/1). Maintenance therapy consisted of Prednisone (20 mg q.o.d.) and Azathioprine (2 mg/kg q.d.). Repeat biopsy in DPGN showed moderate progression 6/9, MGN change 1/9, and improvement 2/9. Progressive biopsy change was noted in MGN (1/1). Increased frequency and prolonged duration of recurrences were associated with progressive biopsy changes of DPGN. Renal function remained or returned to normal or baseline in all patients, including those with progressive biopsy changes. During follow-up the nephrotic syndrome has developed in DPGN (10/17), FGN (0/5) and MGN (6/6). Death has occurred in patients with DPGN (4) and FGN (1).