Steroid-responsive idiopathic glomerular capillary endotheliosis

Case report and literature review

Arohan Subramanya, Donald Houghton, Suzanne Watnick

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Glomerular capillary endotheliosis is a lesion of endothelial cell injury. Morphological characteristics are endothelial swelling with glomerular hypertrophy and a reduction in capillary lumen size. This lesion commonly is found in patients with thrombotic microangiopathy, but similar histopathologic characteristics have been reported in patients with other diseases. A previously healthy 39-year-old woman presented with progressive lower-extremity swelling and arthralgias for 1 week. She had no other symptoms and denied prior illness. Her examination was remarkable for hypertension and pitting edema. Urine showed dysmorphic red blood cells and proteinuria. Serum creatinine level increased from 1.1 to 2.0 mg/dL (97 to 177 μmol/L) during several weeks. She did not meet criteria for systemic lupus erythematosus. Other test results included a negative pregnancy test and normal complement levels. Additional workup was negative for other causes of glomerular capillary endotheliosis. She underwent 2 renal biopsies. The first showed marked endothelial cell swelling, and the second biopsy 2 months later showed disease progression. Both were consistent with glomerular capillary endotheliosis. Proteinuria and serum creatinine level elevation responded to methylprednisolone therapy within 1 week, recurred after steroid doses were tapered, and responded again after restarting steroid therapy with monthly cyclophosphamide infusions. The differential diagnosis for glomerular capillary endotheliosis is limited. Various causes have been implicated, such as dysregulation of vascular endothelial growth factor, abnormal collagen production, and endothelial abnormalities. We did not identify prior cases of idiopathic glomerular capillary endotheliosis in the literature. Idiopathic glomerular capillary endotheliosis may be a newly recognized entity potentially responsive to steroid and cytotoxic regimens.

Original languageEnglish (US)
Pages (from-to)1090-1095
Number of pages6
JournalAmerican Journal of Kidney Diseases
Volume45
Issue number6
DOIs
StatePublished - Jun 2005

Fingerprint

Steroids
Proteinuria
Creatinine
Endothelial Cells
Thrombotic Microangiopathies
Pregnancy Tests
Biopsy
Methylprednisolone
Arthralgia
Serum
Systemic Lupus Erythematosus
Cyclophosphamide
Hypertrophy
Vascular Endothelial Growth Factor A
Disease Progression
Lower Extremity
Edema
Differential Diagnosis
Collagen
Erythrocytes

Keywords

  • Glomerular capillary endotheliosis
  • Glomerulonephritis
  • Kidney diseases
  • Proteinuria

ASJC Scopus subject areas

  • Nephrology

Cite this

Steroid-responsive idiopathic glomerular capillary endotheliosis : Case report and literature review. / Subramanya, Arohan; Houghton, Donald; Watnick, Suzanne.

In: American Journal of Kidney Diseases, Vol. 45, No. 6, 06.2005, p. 1090-1095.

Research output: Contribution to journalArticle

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