Clinical and morphologic spectrum of renal involvement in idiopathic hypereosinophilic syndrome

Clin Exp Nephrol. 2021 Mar;25(3):270-278. doi: 10.1007/s10157-020-02012-5. Epub 2021 Jan 4.

Abstract

Background: To explore the clinical and pathological features of renal lesions in patients with kidney involvement in idiopathic hypereosinophilic syndrome (IHES).

Methods: The demographic, clinical, and pathological characteristics and the treatment and follow-up data were analyzed.

Results: We identified 18 patients with IHES and renal involvement. Eleven patients presented with nephrotic syndrome, and 6 patients had impaired renal function. 15 patients underwent renal biopsy, and the pathological findings included the following: membranoproliferative glomerulonephritis in 3 patients; minimal-change disease in 3; mesangial proliferative nephritis in two; IgA nephropathy in 2; membranous nephropathy in two; chronic interstitial nephritis in two; focal segmental sclerosis in one; and eosinophil infiltration into the renal interstitium in 11 and into the glomerulus in 3. After treatment with glucocorticoids, the eosinophil count decreased. 15 patients were followed up, and 14 showed a decrease in urinary protein or renal function recovery. When glucocorticoids were discontinued, eosinophil increased (8 cases), urine protein increased (1 case), and 1 patient progressed to end-stage renal disease.

Conclusions: Nephrotic syndrome with or without renal insufficiency is the main clinical manifestation. A wide spectrum of renal lesions can be observed in patients with IHES. Eosinophil infiltration into the renal interstitium was common in these patients. Most patients have a good prognosis after glucocorticoid therapy.

Keywords: Clinicopathological features; Glucocorticoid; Hypereosinophilic syndrome; Idiopathic.

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Aged
  • Biopsy
  • Female
  • Glomerulonephritis, IGA / drug therapy
  • Glomerulonephritis, IGA / etiology
  • Glomerulonephritis, IGA / pathology
  • Glomerulonephritis, Membranoproliferative / drug therapy
  • Glomerulonephritis, Membranoproliferative / etiology
  • Glomerulonephritis, Membranoproliferative / pathology
  • Glomerulonephritis, Membranous / drug therapy
  • Glomerulonephritis, Membranous / etiology
  • Glomerulonephritis, Membranous / pathology
  • Glomerulosclerosis, Focal Segmental / drug therapy
  • Glomerulosclerosis, Focal Segmental / etiology
  • Glomerulosclerosis, Focal Segmental / pathology
  • Humans
  • Hypereosinophilic Syndrome / complications
  • Hypereosinophilic Syndrome / drug therapy
  • Hypereosinophilic Syndrome / pathology*
  • Kidney / drug effects
  • Kidney / pathology*
  • Kidney Diseases / drug therapy
  • Kidney Diseases / etiology
  • Kidney Diseases / pathology*
  • Male
  • Middle Aged
  • Nephritis, Interstitial / drug therapy
  • Nephritis, Interstitial / etiology
  • Nephritis, Interstitial / pathology
  • Nephrosis, Lipoid / drug therapy
  • Nephrosis, Lipoid / etiology
  • Nephrosis, Lipoid / pathology
  • Nephrotic Syndrome / drug therapy
  • Nephrotic Syndrome / etiology
  • Nephrotic Syndrome / pathology
  • Treatment Outcome
  • Young Adult

Substances

  • Adrenal Cortex Hormones