title: Clinicopathological features and prognosis of Kimura's disease with renal involvement in Chinese patients.
abstract: OBJECTIVE
  Kimura's disease (KD) with renal involvement is a rare disease. Optimal treatments are still not well established. It is necessary to analyze clinicopathological features, treatment responses, and prognosis for improving KD diagnosis and treatment.
  
  
  METHODS
  Clinicopathological data, treatment responses, and prognosis were collected and analyzed retrospectively.
  
  
  RESULTS
  The patients consisted of 27 males and 2 females, with an average age of 35.5 ± 15.1 (13 - 61) years. 27 exhibited proteinuria ranging from 0.730 to 14.1 g/24 h (5.98 ± 3.40 g/24 h). Hypertension, renal insufficiency (serum creatinine (Scr) > 1.24 mg/dL), and microhematuria occurred in 4 (13.8%), 11 (37.9%), and 13 (44.8%) cases, respectively. Light microscopy (LM) identified mesangium proliferation, minimal change, focal and segmental glomerulosclerosis (FSGS), membranous glomerulonephritis, membranoproliferative glomerulonephritis (MPGN), and acute tubular necrosis in 14, 8, 3, 2, 1, and 1 cases, respectively. All were treated with Tripterygium wilfordii (TW), prednisone, leflunomide (LEF), tacrolimus (FK506), myophenolate mofetil (MMF), or renin-angiotensin system blockers (RASI). 26 patients were followed up for 1.60 - 108.7 months (39.6 ± 28.7). After treatments, urinary red blood cells (RBC) decreased in all. The amount of 24-hour urinary protein (24-hUPE) decreased in 24 patients. 22 reached complete remission (CR), 4 partial remissions (PR). The patients who did not relapse were younger than those who relapsed.
  
  
  CONCLUSIONS
  KD with renal involvement occurs predominantly among 35 - 50 year old Chinese patients with male predilection. The most common features are proteinuria, hypertension, micro hematuria with minimal change, and mesangial proliferative glomerulonephritis. Most were responsive to treatment, but could relapse. Gender, age, and hypertension are associated with KD recurrence. The prognosis is good mostly.
authors.forename: Ying
authors.initials: Y
authors.lastname: Chen
authors.forename: Jinquan
authors.initials: J
authors.lastname: Wang
authors.forename: Feng
authors.initials: F
authors.lastname: Xu
authors.forename: Caihong
authors.initials: C
authors.lastname: Zeng
authors.forename: Zhihong
authors.initials: Z
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D000328:Adult; D000796:Angiolymphoid Hyperplasia with Eosinophilia / Q000150:complications* / Q000188:drug therapy* / Q000503:physiopathology; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002681:China; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000473:pathology; D015432:Glomerulonephritis, Membranoproliferative / Q000150:complications / Q000473:pathology; D015433:Glomerulonephritis, Membranous / Q000150:complications / Q000473:pathology; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000473:pathology; D006417:Hematuria / Q000150:complications; D006801:Humans; D006973:Hypertension / Q000150:complications; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007555:Isoxazoles / Q000627:therapeutic use; D000077339:Leflunomide; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000627:therapeutic use; D008517:Phytotherapy; D028321:Plant Preparations / Q000627:therapeutic use*; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D011507:Proteinuria / Q000150:complications; D012008:Recurrence; D051437:Renal Insufficiency / Q000150:complications; D012189:Retrospective Studies; D016559:Tacrolimus / Q000627:therapeutic use; D029757:Tripterygium; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D007555:Isoxazoles; D028321:Plant Preparations; D000077339:Leflunomide; D009173:Mycophenolic Acid; D011241:Prednisone; D016559:Tacrolimus