关键词: 嗜酸性粒细胞增多综合征, 特发性, 临床病理特征, 糖皮质激素

Abstract:

Ｏｂｊｅｃｔｉｖｅ：To explore the clinicopathologic features, treatment and prognosis of renal impairment in patients with idiopathic hypereosinophilic syndrome (IHES).
Ｍｅｔｈｏｄｏｌｏｇｙ：Eighteen patients with IHES were enrolled into this retrospective study. Their clinical, pathologic and followup data were investigated.
Ｒｅｓｕｌｔｓ：They were 13 male and 8 female with a median age of 36 years old (ranged from 9 to 67 y). They presented multiple organ dysfunctions including kidney (100%), skin (278%), lung (278%), gastrointestinal tract (278%) and peripheral nerve involvement (111%). The eosinophil count significantly increased (1 670～15 100/μl). Renal impairment was included nephrotic syndrome in 12 cases (667%), urinary abnormalities in 4 (222%), acute kidney injury in 2 (111%), and chronic renal insufficiency in 6 (333%). 15 cases underwent renal biopsy, the histological diagnosis was membranoproliferative glomerulonephritis in 3 cases, podocyte disease in 3, mesangial proliferative glomerulonephritis in 2, IgA nephropathy in 2, membranous nephropathy in 2, chronic interstitial nephritis in 2, and focal segmental sclerosis in one case. Eosinophil infiltrated into renal interstitium in 11 and into Glomerulus in 3) cases. Immunoglobulin or complement deposited in glomerulus in 12 cases. After treatment of glucocorticoid only or combination with other immunosuppressant, the eosinophil count was decreased. 15 patients were followedup, 14(933%) showed the urinary protein decreased or renal function recovered. When the glucocorticoid is discontinued, eosinophil increased(8 cases),urine protein increased(1) in some patients, even progression to endstage renal disease(1).
Ｃｏｎｃｌｕｓｉｏｎ：
IHES can induce renal impairmen.Nephrotic syndrome were common as clinical features. Pathologic types were various,infiltration of eosinophil were the common pathologic features. Glucocorticoid can improve the renal impairments. Prognosis were good in most of the patients.

Key words: hypereosinophilic syndrome, idiopathic, clinico-pathologic features, glucocorticoids