Clinical and pathological study of Kimura's disease with renal involvement.
OBJECTIVE
Our objective was to retrospectively analyze the clinical and histological features of 8 Chinese patients with Kimura's disease and renal involvement.


METHODS
Eight male patients with Kimura's disease admitted to Jinling Hospital from 1998 to 2006 were included. The diagnoses were confirmed by lymphoid biopsy. Peripheral blood eosinophil, CD4+ and CD8+ T lymphoid cell count and total serum IgE level were examined. Renal biopsy, IL-4 expression cells and IgE-positive cell counts in renal tissue were performed. Follow-up data were recorded in detail.


RESULTS
Six patients were observed with the onset symptoms of subcutaneous mass or enlarged lymph nodes, and the other 2 with edema. Constitutional symptoms included bronchial asthma, enteritis, eczema, neuritis and nephrotic syndrome. Renal biopsy revealed mesangial proliferation with or without IgA deposition under immunofluorescence (n=6) and membranous nephropathy (n=2). Tubulointerstitial infiltration of eosinophils was found in 6 patients. IL-4-positive cells in renal interstitium were detected in 5 cases. IgE-positive cells were negative in all cases. Fusion of the epithelial foot process was observed under electron microscopy in patients with mesangial proliferation. All patients were sensitive to treatment with systemic prednisone, but 4 patients had a renal or extrarenal relapse after the dose tapered. Three patients who presented with solitary masses and underwent lymphoidectomy or mass excision were free from renal and extrarenal relapse. All patients had normal renal function at last follow-up.


CONCLUSIONS
Mesangial proliferation, eosinophilic infiltration and podocyte confusion are prominent histological features of this cohort of patients. Patients are sensitive to prednisone therapy but apt to relapse. Lymphoidectomy can be helpful to prevent relapse.
Liu
C
C
Research Institute of Nephrology, Jinling Hospital, Nanjing University School of Medicine, Nanjing, Jiangsu - China.
Hu
W
W
Chen
H
H
Tang
Z
Z
Zeng
C
C
Liu
Z
Z
Li
L
L
D000293:Adolescent; D000328:Adult; D000796:Angiolymphoid Hyperplasia with Eosinophilia / Q000150:complications* / Q000276:immunology / Q000473:pathology; D001706:Biopsy; D016516:CD4-CD8 Ratio; D049109:Cell Proliferation; D005500:Follow-Up Studies; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood*; D007150:Immunohistochemistry; D015847:Interleukin-4 / Q000096:biosynthesis*; D007668:Kidney / Q000378:metabolism / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index
D015847:Interleukin-4; D007073:Immunoglobulin E