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Idiopathic IgA nephropathy with segmental necrotizing lesions of the capillary wall
- Source :
- Kidney international. 59(2)
- Publication Year :
- 2001
-
Abstract
- Idiopathic IgA nephropathy with segmental necrotizing lesions of the capillary wall. Background Segmental glomerular necrosis has been described in the biopsy material in a minority of patients with idiopathic IgA nephropathy in the oldest studies on this disease, but this marker of active capillaritis has received little attention in the subsequent literature, and its significance and relevance for the clinical outcome is still unknown. Methods Thirty-five out of 340 patients (10.3%) biopsied in our division at the San Carlo Hospital since 1974 showed active segmental necrotizing lesions. The morphological features and the natural history of this group of patients were compared with those of a control group of 229 patients who had comparable serum creatinine and extent of glomerular sclerosis, but who lacked active segmental necrosis. Results Patients with the necrotic variant showed a significantly more marked extracapillary proliferation and interstitial accumulation of monocytes and T lymphocytes and, in the segmental areas of necrotizing and extracapillary lesions, infiltration of monocytes, deposition of fibrinogen, and expression of the adhesion molecule vascular cell adhesion molecule-1. No difference was found in the presenting clinical syndrome. The clinical course was frequently characterized by acute flareups, and the progression to end-stage renal failure was more frequent, although actuarial renal survival was not significantly worse ( P = 0.07). The aggressive treatment with steroids and cyclophosphamide, carried out in 20 of the 35 patients, has probably been beneficial, justifying the multicenter controlled trial that recently has been initiated. Conclusions Vasculitic lesions of the glomerular capillaries, with histologic and immunohistological features similar to those of Henoch-Schonlein purpura and antineutrophil cytoplasmic antibody-positive renal vasculitis, were found in 10% of patients with idiopathic IgAN. Clinical features at presentation did not differ from those of the other patients with IgAN, and despite of the more frequent occurrences of recurrent acute flareups, rapid progression to end-stage renal failure was a rare phenomenon, even in untreated patients.
- Subjects :
- Adult
Male
Pathology
medicine.medical_specialty
Renal glomerulus
Biopsy
Angiotensin-Converting Enzyme Inhibitors
Methylprednisolone
Nephropathy
Renal Circulation
chemistry.chemical_compound
Necrosis
Necrotizing Vasculitis
medicine
Humans
active capillaritis
Cyclophosphamide
Glucocorticoids
Kidney
Creatinine
Berger disease
endothelium injury
business.industry
Glomerulonephritis
Glomerulonephritis, IGA
medicine.disease
Capillaritis
Immunohistochemistry
Survival Analysis
Capillaries
medicine.anatomical_structure
chemistry
Nephrology
vasculitic damage
Disease Progression
Prednisone
Drug Therapy, Combination
Female
business
glomerulonephritis
Immunosuppressive Agents
Kidney disease
Subjects
Details
- ISSN :
- 00852538
- Volume :
- 59
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Kidney international
- Accession number :
- edsair.doi.dedup.....6e1989f44defacac38818e8b356cb2ce