关键词: POEMS综合征, 肾脏损害, 病理

Abstract:

ABSTRACT Objective: To investigated the clinical and pathological features of renal involvement in patients with POEMS syndrome. Methodology: Twelve consecutive patients diagnosed as POEMS syndrome in a single institute were recruited in this study。Among them, 4 patients were performed renal biopsy. Their clinical and pathological characteristics of renal lesions were investigated. Results: They were 9 males and 3 females with a median age of 44.5 years old. The systemic manifestations included polyneuropathy in all cases, organomegaly in 11, at least one endocrine abnormality at time　of presentation in 10, a detectable monoclonal protein with serum immunofixation in 9, and skin changes documented in 12 cases. The renal involvements included initially reported edema in 11 cases, mild proteinuria in 8, microscopic hematuria in one, and the increasing serum creatinine in half of cases. Renal biopsy showed prominent glomerular changes which were mesangiolytic lesions with swelling and vacuolization of endothelial cells. Mesangial proliferation and thickening of the capillary wall with double contour were also observed on light microscopy. No immunoglobulins or light chains deposits were found on immunofluorescent microscopy. Conclusion: These observations suggest that chronic endothelial injury constitute the basic pathology of glomerular disease in patients with POEMS syndrome, while no specific clinical renal features. It is important for clinicians that POEMS should be considered a rare cause of renal disease.