Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Hilton 10-20, Rochester, MN 55905, USA.
Clin J Am Soc Nephrol. 2012 Feb;7(2):231-9. doi: 10.2215/CJN.08640811. Epub 2011 Dec 8.
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.
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.
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.
为了更好地定义单克隆免疫球蛋白沉积病（MIDD）的临床病理谱和预后，本研究报告了最大的系列。
设计、地点、参与者和测量方法：提供了在梅奥诊所就诊的 64 例 MIDD 患者的特征。
在 64 例 MIDD 患者中，51 例为轻链沉积病，7 例为重链沉积病，6 例为轻链和重链沉积病。诊断时的平均年龄为 56 岁，23 例（36%）≤50 岁。62 例（97%）患者存在蛋白血症的临床证据，其中 38 例（59%）为多发性骨髓瘤。47 例（73%）患者的血清蛋白电泳检测到 M 峰。51 例患者均检测到血清游离轻链比值异常。临床表现包括肾功能不全、蛋白尿、血尿和高血压。39 例（61%）患者可见结节性系膜硬化。在 56 例患者中，中位数为 25 个月（范围 1-140）的随访期间，32 例（57%）患者肾功能稳定/改善，2 例（4%）患者肾功能恶化，22 例（39%）进展为终末期肾病。平均肾脏和患者生存率分别为 64 个月和 90 个月。接受肾移植的 4 例患者中有 3 例疾病复发。
与轻链淀粉样变性或轻链 casts 肾病相比，MIDD 患者一般在更年轻的年龄出现。所有 MIDD 患者的血清游离轻链比值均异常，而只有四分之三的患者血清蛋白电泳异常。与历史对照相比，MIDD 的预后正在改善，这可能反映了更早的发现和更好的治疗。
