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[Rapidly progressive glomerulonephritis in macroscopic periarteritis nodosa. 7 cases]
R Cevallos1, L Guillevin, B Jarrousse
1Service de Médecine Interne, Université Paris-Nord, Bobigny.
Abstract:
Rapidly progressive glomerulonephritis (RPGN) is rarely associated with macroscopic polyarteritis nodosa (PAN), as seen in this series of 7 out of 235 patients. The clinical symptoms of PAN were as follows: myalgias 6; fever 1; arthralgias and mononeuropathy multiplex 5; cutaneous vasculitis 3; arterial hypertension 4, 3 of which were malignant. The mean proteinuria was 2.7 g/24 h; creatininemia 458 microM/l; microscopic hematuria was present in 4 of the 7 patients; only 1 patient was anuric. Markers of hepatitis B virus were absent in all cases. Arteriography revealed microaneurysms and renal infarcts in 6 patients and distal arterial stenosis in one. Renal biopsies from all 7 patients demonstrated extracapillary glomerulonephritis, which was associated with tubulointerstitial fibrosis in one. Necrotizing vasculitis lesions were associated in 5 cases. Immunofluorescence was positive in 5 cases. The association of RPGN and PAN exists and may be underestimated due to the lack of systematic angiographic examinations during RPGN and renal biopsies in PAN patients with renal involvement.
Insights
Rapidly progressive glomerulonephritis (RPGN) rarely occurs with polyarteritis nodosa (PAN). This study highlights their association, suggesting it may be underdiagnosed due to insufficient diagnostic testing.
Area of Science:
- Nephrology
- Rheumatology
- Pathology
Background:
- Polyarteritis nodosa (PAN) is a systemic vasculitis that can affect renal arteries.
- Rapidly progressive glomerulonephritis (RPGN) is a severe form of kidney injury characterized by rapid loss of kidney function.
Observation:
- This series examined 7 patients with both RPGN and PAN, a rare clinical association.
- Clinical manifestations included myalgias, arthralgias, mononeuropathy multiplex, cutaneous vasculitis, and severe arterial hypertension.
- Laboratory findings revealed significant proteinuria, elevated creatinine, and hematuria, with one patient being anuric.
Findings:
- Arteriography showed microaneurysms and renal infarcts in most patients.
- Renal biopsies confirmed extracapillary glomerulonephritis and necrotizing vasculitis lesions.
- Hepatitis B virus markers were negative in all cases.
Implications:
- The co-occurrence of RPGN and PAN is likely underestimated.
- Systematic angiographic examinations and renal biopsies are crucial for diagnosing this association.
- Early recognition and diagnosis are vital for appropriate management of patients with RPGN and PAN.