title: [Renal manifestations of light chain associated diseases -  epidemiology and prognosis].
abstract: BACKGROUND
  An impaired renal function in light chain associated disorders may be caused by myeloma cast nephropathy (MCN) but also by AL-amyloidosis (AL-A) and monoclonal immundeposition disease (MIDD).
  
  
  METHODS
  In a monocentric, retrospective analysis, patients suffering from multiple myeloma (MM) (n = 392) requiring medical therapy, AL-A (n = 53) or MIDD (n = 12) diagnosed between 1996 and 2008 were evaluated for renal insufficiency. The different patient cohorts were compared in terms of their clinical course and outcome.
  
  
  RESULTS
  Renal insufficiency in MM-, AL-A- or MIDD-patients at the time of diagnosis was found in 45,5 % of the patients. MCN, AL-A and MIDD were found in 68, 25 and 6 %, respectively. Dialysis dependency was seen in 17 % of MCN, in 8 % of AL-A and in 50 % of MIDD patients. Signs of hypervolemia were the leading symptoms in MIDD/AL-A. The time between the occurence of first symptoms and diagnosis was as long as 52 weeks in patients with AL-A. Patients with renal involvement showed a reduced median survival of 17 compared with 77 months in patients with a normal renal function. Median survival was only 12 months in AL-A compared to 21 months in MCN. Stabilization of renal function after chemotherapy occurred only in MCN. Multivariate Cox regression analysis showed impaired renal function as independent risk factor (Hazard-Ratio 2,88 [2,06-4,0]. In terms of survival and kidney function, autologous stem cell transplantation (ASCT) was beneficial for patients with renal involvement.
  
  
  CONCLUSIONS
  Renal insufficiency is an independent risk factor in MM, AL-A and MIDD. Specific therapy, especially ASCT may improve prognosis in patients with renal insufficiency and could stabilize renal function in MCN-patients.
authors: Klinik für Innere Medizin II, Heinrich Braun Klinikum Zwickau. jens.gerth@hbk-zwickau.de
authors: J
authors: J
authors: Gerth
authors: H
authors: H
authors: Sigusch
authors: N
authors: N
authors: Illner
authors: M
authors: M
authors: Busch
authors: L-O
authors: LO
authors: Muegge
authors: T
authors: T
authors: Lehmann
authors: G
authors: G
authors: Wolf
mesh_terms: D000682:Amyloid / Q000097:blood; D003404:Creatinine / Q000097:blood; D003430:Cross-Sectional Studies; D005500:Follow-Up Studies; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood*; D007676:Kidney Failure, Chronic / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality* / Q000628:therapy; D009101:Multiple Myeloma / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality* / Q000628:therapy; D010265:Paraproteinemias / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality* / Q000628:therapy; D006435:Renal Dialysis; D012189:Retrospective Studies; D016019:Survival Analysis
chemical_list: D000682:Amyloid; D007147:Immunoglobulin Light Chains; C035222:amyloid protein AL; D003404:Creatinine