POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal M protein, and skin lesion) is a rare multisystemic disease of unknown cause with varying clinical manifestations. Amyloidosis-associated POEMS Syndrome is also rare condition. We experienced a 63-year-old female who had been suffered from edema and tingling sensation of low extremities. She also had a marked demyelination, axonal degeneration and regeneration of the nerve, hypoaldosteronism, hyperprolactinemia, decreased sexual hormone, monoclonal gammopathy (IgG lambda type), skin change and ascites. The renal biopsy revealed homogenous mesangial widening with a few cell nuclei at the glomerular capillary, and it showed positive apple-green birefringence in Congo-red stain. It's the first report that shows the deposition of amyloid in patients with POEMS syndrome in Korea. Edema and M-spike were improved by use of prednisolone, melphalan, fludrocortisone and colchicine.
Amyloid ; Amyloidosis* ; Ascites ; Axons ; Biopsy ; Birefringence ; Capillaries ; Cell Nucleus ; Colchicine ; Demyelinating Diseases ; Edema ; Extremities ; Female ; Fludrocortisone ; Humans ; Hyperprolactinemia ; Hypoaldosteronism ; Korea ; Melphalan ; Middle Aged ; Paraproteinemias ; POEMS Syndrome* ; Prednisolone ; Regeneration ; Sensation ; Skin

BACKGROUND: The manifestation of Membranoproliperative glomerulonephritis (MPGN) is variable from asymptomatic hematuria, nephrotic syndrome to advanced chronic kidney disease. The etiology, pathogenesis, and treatment of MPGN are unclear. The incidence of idiopathic MPGN is also rare in Korea, and the clinical course was rarely studied. So we investigated clinical course of idiopathic MPGN type 1. METHODS: From March 1990 to November 2004, renal biopsy was done in about 1500 patients in Dong-A universty hospital. Among them, 16 patients were diagnosed as idiopathic MPGN. Fourteen patients were observed over 6 months. We analyzed clinical data of these patients retrospectively. RESULTS: Male to female ratio of total 16 patients is 2.2:1. Mean age of patients was 37.6 years (14-76 years) at the time of diagnosis. Thirteen cases (81%) had nephrotic range proteinuria, 8 cases (50%) had hypertention, 12 cases (75%) had edema, and 4 cases (25%) had decreased renal fuction (serum creatinine>1.2 mg/dL) at the time of diagnosis. Average observation time was 55.7+/-37.4 (6-122)months. Six cases ended up with progressive renal failure. Three cases achieved complete remission. Renal survival time for 50% of cases was 92 months. In our study, edema and decreased renal fucntion at the time of diagnosis were related with later development of progressive renal failure (p<0.05). Of the total 14 cases, 4 cases ware treated conservatively, 6 cases were treated with anti-platelet agents, and 4 cases were treated with anti-platelet agents and prednisone. In conservative treatment group, 1 case ended up with progressive renal failure and 1 case achieved complete remission. In anti-platelet agents treatment goup, 4 cases ended up progressive renal failure. In combined anti-platelet agents and prednisone treatment group, 1 case ended up progressive renal failure and 2 cases achieved complete remission. However, the effect of each treatment is inconclusive because the number of the patients are too small. CONCLUSIONS: We suspect that deceased renal function and edema at the time of diagnosis may be risk factor predicting progressive renal failure in patients with idiopathic MPGN type 1.
Biopsy ; Diagnosis ; Edema ; Female ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative* ; Hematuria ; Humans ; Incidence ; Korea ; Male ; Nephrotic Syndrome ; Prednisone ; Proteinuria ; Renal Insufficiency ; Renal Insufficiency, Chronic ; Retrospective Studies ; Risk Factors