Rapidly progressive glomerulonephritis secondary to anti-GBM disease associated with MPO-ANCA: a case report
Fecha
2023Autor
Resumen
Abstract
Background Anti-glomerular basement membrane (GBM) disease and ANCA-associated vasculitis (AAV) diseases are
rare. It is associated with variable renal manifestations and increased mortality, thus requiring early aggressive treat‑
ment to minimize adverse outcomes and improve prognosis.
Case presentation We present the case of a male patient with 1-month onset of asthenia, adynamia, oliguria, and
weight loss. Initial laboratory fndings were indicative of severe kidney dysfunction. The urinalysis showed active
sediment, but the urinary tract ultrasound was unaltered. As these fndings were consistent with rapidly progressive
glomerulonephritis, he received steroid pulses, and given the severity of the condition, renal replacement therapy
was initiated. Other diagnostic tests revealed MPO-ANCA antibody levels of 26 mg/dl, pANCAs 1/320, and anti-GBM of
8 mg/dl. Kidney biopsy evidenced necrotizing glomerulonephritis with extracapillary proliferation in 90% of the glo‑
meruli. The patient received plasma exchange (PE) therapy and intravenous (IV) cyclophosphamide (CYC) cycles; how‑
ever, he presented with severe alveolar hemorrhage requiring the completion of 21 PE sessions and 3 CYC boluses.
Pulmonary symptoms resolved, but the patient persisted dependent on dialysis. During the outpatient follow-up,
monthly CYC were prescribed until circulating antibody levels were normal; however, the patient did not recover full
kidney function and remained dependent on renal support.
Conclusions Anti-GBM and AAV diseases are rare; therefore, anti-GBM antibodies should be screened simultaneously
in patients with ANCA positive, especially in older patients, due to the early morbidity and mortality typical of antiGBM disease with comparable disease severity it represents.
Abstract
Abstract
Background Anti-glomerular basement membrane (GBM) disease and ANCA-associated vasculitis (AAV) diseases are
rare. It is associated with variable renal manifestations and increased mortality, thus requiring early aggressive treat‑
ment to minimize adverse outcomes and improve prognosis.
Case presentation We present the case of a male patient with 1-month onset of asthenia, adynamia, oliguria, and
weight loss. Initial laboratory fndings were indicative of severe kidney dysfunction. The urinalysis showed active
sediment, but the urinary tract ultrasound was unaltered. As these fndings were consistent with rapidly progressive
glomerulonephritis, he received steroid pulses, and given the severity of the condition, renal replacement therapy
was initiated. Other diagnostic tests revealed MPO-ANCA antibody levels of 26 mg/dl, pANCAs 1/320, and anti-GBM of
8 mg/dl. Kidney biopsy evidenced necrotizing glomerulonephritis with extracapillary proliferation in 90% of the glo‑
meruli. The patient received plasma exchange (PE) therapy and intravenous (IV) cyclophosphamide (CYC) cycles; how‑
ever, he presented with severe alveolar hemorrhage requiring the completion of 21 PE sessions and 3 CYC boluses.
Pulmonary symptoms resolved, but the patient persisted dependent on dialysis. During the outpatient follow-up,
monthly CYC were prescribed until circulating antibody levels were normal; however, the patient did not recover full
kidney function and remained dependent on renal support.
Conclusions Anti-GBM and AAV diseases are rare; therefore, anti-GBM antibodies should be screened simultaneously
in patients with ANCA positive, especially in older patients, due to the early morbidity and mortality typical of antiGBM disease with comparable disease severity it represents.
Keywords
URI
http://repositorio.mederi.com.co/handle/123456789/763https://link.springer.com/article/10.1186/s42269-023-01020-1
Colecciones
- Investigación clínica [389]