Light Chain Deposition Disease with Renal Involvement: Clinical Characteristics and Prognostic Factors

Claudio Pozzi, Marco D'Amico, Giovanni B. Fogazzi, Simona Curioni, Franco Ferrario, Sonia Pasquali, Giacomo Quattrocchio, Cristiana Rollino, Siro Segagni, Francesco Locatelli

Research output: Contribution to journalArticle

Abstract

Background: Light chain deposition disease (LCDD) is characterized by the tissue deposition of monotypical immunoglobulin light chains (LCs). The aim of this study was to investigate its clinical characteristics and prognostic factors. Methods: Multicenter study of LCDD with renal and patient survival analyses. Results: Sixty-three cases were studied (age: 58 ± 14.2; males: 63.5%; κ/λ deposition: 68/32%; underlying disorders: multiple myeloma [MM] 65%, lymphoproliferative disorders 3%, idiopathic 32%). Ninety-six percent presented with renal insufficiency (acute, 52%; chronic, 44%), and 84% with proteinuria >1 g/d. During the follow-up, 36 patients reached uremia (incidence rate: 23.7/100 patient-years) and 37 died (17.5/100 patient-years). The factors independently associated with a worse renal prognosis were age (relative risk [RR], 1.05; 95% confidence interval [CI], 1.009. to 1.086) and serum creatinine at presentation (RR, 1.24; 95% CI, 1.02 to 1.5). Those independently associated with a worse patient survival were age (RR, 1.06; 95% CI, 1.03 to 1.1), MM (RR, 2.75; 95% CI, 1.22 to 6.2), and extrarenal LC deposition (RR, 2.24; 95% CI, 1.15 to 4.35). While κ-LC deposition was more frequently associated with nodular sclerosing glomerulopathy, histological parameters were not predictors of renal/patient prognosis. The survival of the uremic patients undergoing dialysis was similar to that of patients not reaching uremia. Conclusion: LCDD is characterized by renal insufficiency with proteinuria and has a severe prognosis. Apart from age, the prognostic factors identified were degree of renal insufficiency at presentation affecting the renal prognosis, underlying hematologic disorder and extrarenal LC deposition affecting the patient prognosis. Dialysis is worth performing in uremic LCDD patients.

Original languageEnglish
Pages (from-to)1154-1163
Number of pages10
JournalAmerican Journal of Kidney Diseases
Volume42
Issue number6
DOIs
Publication statusPublished - Dec 2003

Keywords

  • Autologous stem cell transplantation
  • Chemotherapy
  • Diagnosis
  • Doxorubicin
  • Light chain deposition disease (LCDD)
  • Renal insufficiency
  • Survival

ASJC Scopus subject areas

  • Nephrology

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  • Cite this

    Pozzi, C., D'Amico, M., Fogazzi, G. B., Curioni, S., Ferrario, F., Pasquali, S., Quattrocchio, G., Rollino, C., Segagni, S., & Locatelli, F. (2003). Light Chain Deposition Disease with Renal Involvement: Clinical Characteristics and Prognostic Factors. American Journal of Kidney Diseases, 42(6), 1154-1163. https://doi.org/10.1053/j.ajkd.2003.08.040