title: Renal monoclonal immunoglobulin deposition disease: a report of 64 patients from a single institution.
abstract: OBJECTIVE
  To better define the clinical-pathologic spectrum and prognosis of monoclonal immunoglobulin deposition disease (MIDD), this study reports the largest series.
  
  
  METHODS
  Characteristics of 64 MIDD patients who were seen at Mayo Clinic are provided.
  
  
  RESULTS
  Of 64 patients with MIDD, 51 had light chain deposition disease, 7 had heavy chain deposition disease, and 6 had light and heavy chain deposition disease. The mean age at diagnosis was 56 years, and 23 patients (36%) were ≤50 years of age. Clinical evidence of dysproteinemia was present in 62 patients (97%), including multiple myeloma in 38 (59%). M-spike was detected on serum protein electrophoresis in 47 (73%). Serum free light chain ratio was abnormal in all 51 patients tested. Presentation included renal insufficiency, proteinuria, hematuria, and hypertension. Nodular mesangial sclerosis was seen in 39 patients (61%). During a median of 25 months of follow-up (range, 1-140) in 56 patients, 32 (57%) had stable/improved renal function, 2 (4%) had worsening renal function, and 22 (39%) progressed to ESRD. The mean renal and patient survivals were 64 and 90 months, respectively. The disease recurred in three of four patients who received a kidney transplant.
  
  
  CONCLUSIONS
  Patients with MIDD generally present at a younger age than those with light chain amyloidosis or light chain cast nephropathy. Serum free light chain ratio is abnormal in all MIDD patients, whereas only three-quarters have abnormal serum protein electrophoresis. The prognosis for MIDD is improving compared with historical controls, likely reflecting earlier detection and improved therapies.
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Hilton 10-20, Rochester, MN 55905, USA. nasr.samih@mayo.edu
authors.forename: Samih H
authors.initials: SH
authors.lastname: Nasr
authors.forename: Anthony M
authors.initials: AM
authors.lastname: Valeri
authors.forename: Lynn D
authors.initials: LD
authors.lastname: Cornell
authors.forename: Mary E
authors.initials: ME
authors.lastname: Fidler
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Sethi
authors.forename: Vivette D
authors.initials: VD
authors.lastname: D'Agati
authors.forename: Nelson
authors.initials: N
authors.lastname: Leung
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D004586:Electrophoresis; D005260:Female; D005455:Fluorescent Antibody Technique; D006362:Heavy Chain Disease / Q000276:immunology; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000032:analysis*; D007147:Immunoglobulin Light Chains / Q000032:analysis*; D053208:Kaplan-Meier Estimate; D007668:Kidney / Q000276:immunology* / Q000503:physiopathology / Q000648:ultrastructure; D007674:Kidney Diseases / Q000276:immunology* / Q000401:mortality / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D016030:Kidney Transplantation; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D008910:Minnesota; D009101:Multiple Myeloma / Q000276:immunology; D015999:Multivariate Analysis; D010265:Paraproteinemias / Q000276:immunology* / Q000401:mortality / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D016016:Proportional Hazards Models; D012008:Recurrence; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains