Abstract:

Ｏｂｊｅｃｔｉｖｅ：To explore the clinicopathologic features and prognosis in Chinese patients with light chain deposition disease (LCDD).
Ｍｅｔｈｏｄｏｌｏｇｙ：Forty five patients with LCDD fulfilled the inclusion criteria were recruited this retrospective study. Their clinical, pathologic and followup data were collected and analyzed. KaplanMeier estimate and Cox regression were used in the analysis of renal survival and prognostic factors respectively.
Ｒｅｓｕｌｔｓ：They were 28 males and 17 females with a mean age of 508 years old, and male/female ratio was 16∶1. 11 patients were simultaneously diagnosed as multiple myeloma (MM). In clinical findings, hypertension was presented in 800% of cases, renal insufficiency in 956%, anemia in 956%, nephroticrange proteinuria (≥35 g/24h) in 444% and microhematuria in 822%. Monoclonal Ig was detected in 262% of cases by serum immunofixation electrophoresis (sIFE). Serum free light chain (FLC) ratio was abnormal (κ/λ>165) in 844% of cases, and was markedly abnormal (κ/λ>8) in 311%. 333% of patients had hypocomplementemia C3. In histopathological observation, 800% of patients showed glomerular nodular lesions and the remaining patients (200%) displayed mild to moderate mesangial proliferation. Severe tubular atrophy and interstitial fibrosis were found in 533% of patients. 778% of cases had light chain related arteriole lesion on light microscopy. Immunofluorescence revealed deposits of light chain (956% κ light chain) linear along the GBMs and TBMs. A total of 40 patients had followedup for a mean time of 221 months, and 26 patients developed to ESRD. The mean renal survivals were 338 months. Univariate and multivariate COX analysis indicated the serum creatinine (SCr) (P=0005) and the level of urinary retinol binding protein (RBP) (P<0001) at renal biopsy predicting prognosis. Other clinical and histopathologic parameters did not correlate with renal survival.
Ｃｏｎｃｌｕｓｉｏｎ：LCDD was more prevalent in middleaged males and manifested by renal dysfunction, hypertension, anemia, abnormal serum FLC ratio, nephrotic proteinuria and microhematuria. The common pathologic features were deposition of κ light chain, nodular lesion, severe tubularinterstitial lesions and electron dense deposition along inner GBM and out layer TBM. LCDD patients had worse renal survival, and the independently prognostic factors were SCr and RBP.