RAPIDLY PROGRESSING

IGA NEPHROPATHY - how to

Abstract Category:
Endocrine and
treat?
metablolic disorders C. A. Correia, J. Freitas , A. Martins, J. Oliveira, J. Almeida
A-1513
Centro Hospitalar São João, Porto, Portugal
Introduction
IgA nephropathy (IgAN) is the most common glomerulonephritis in the Western world and typically presents
with a single or recurrent episodes of visible hematuria, usually following an upper respiratory infection, or with
mild proteinuria. The rapidly progressing and crescentic subtype is quite uncommon.

Case description

1 week Last days

• fatigue, lethargy,
bilateral lower • reporting brown
extremity swelling, • shortness of breath, emesis 4 - 5
nausea. mid sternal times/day • no color changes or
exertional chest pain dysuria
worsened on
inspiration
2 weeks 2 days

Clinical findings
• In the emergency department, examination The biopsy
demonstrated a significant hypertension specimen shows
proliferation of
194/109mmHg the mesagium,
with IgA deposits
Laboratory studies on immunofluore
scence and electr
• Tests showed anemia 8.2/25.3, metabolic acidosis pH on microscopy.
7.22, HCO3 16, PCO2 40, anion gap 18, and BUN/Cr
192/15.1. U/A showed 300 mg/dL of protein and
large hemoglobin but no gross hematuria. Cellular/fibrous crescents,
fibrinoid necrosis, and global
Laboratory and imaging studies sclerosis

• An autoimmune screen was performed: ANA,

ENAs, Anti- DNA, ANCAs and antiphospholipid
antibodies, which were all negative

Laboratory and imaging studies Emergent

dialysis
• The patient’s proteinogram showed no alteration
and serology for CMV, EB, HCV, HBV and HIV
was negative. Chest x-ray was normal.
• Echocardiogram with LVH

Glucocorticoids
+cyclophospha
mide followed
by azathioprine
Glucocorticoids
?
plus
azathioprine

Discussion
Despite IgAN is the most common glomerulonephritis in the Western world, a particular rapid progression to ESRD is
uncommon (< 10%) . In this patient, the rapid clinical deterioration and histopathological presence of cellular/fibrous
crescents, fibrinoid necrosis, and global sclerosis were suggestive of active glomerular injury in a background of
cortical chronic injury. In particular, the treatment of crescentic, rapidly progressive glomerulonephritis in patients with
IgAN has not been evaluated in randomized trials.

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