Renal Monoclonal Immunoglobulin Deposition Disease: A Report of 64 Patients from a Single Institution

被引:194
作者
Nasr, Samih H. [1 ]
Valeri, Anthony M. [4 ]
Cornell, Lynn D.
Fidler, Mary E.
Sethi, Sanjeev
D'Agati, Vivette D. [5 ]
Leung, Nelson [2 ,3 ]
机构
[1] Mayo Clin, Div Anat Pathol, Dept Lab Med & Pathol, Rochester, MN 55905 USA
[2] Mayo Clin, Div Nephrol, Rochester, MN 55905 USA
[3] Mayo Clin, Div Hematol, Rochester, MN 55905 USA
[4] Columbia Univ, Coll Phys & Surg, Div Nephrol, New York, NY USA
[5] Columbia Univ, Coll Phys & Surg, Dept Pathol, New York, NY USA
来源
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY | 2012年 / 7卷 / 02期
关键词
HEAVY-CHAIN DEPOSITION; LIGHT-CHAIN; NODULAR GLOMERULOSCLEROSIS; SPECTRUM; FEATURES;
D O I
10.2215/CJN.08640811
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
Background and objectives To better define the clinical-pathologic spectrum and prognosis of monoclonal immunoglobulin deposition disease (MIDD), this study reports the largest series. Design, setting, participants, & measurements 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. Clin J Am Soc Nephrol 7: 231-239, 2012. doi: 10.2215/CJN.08640811
引用
收藏
页码:231 / 239
页数:9
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