Crescentic Glomerulonephritis Workup
- Author: Malvinder S Parmar, MB, MS; Chief Editor: Vecihi Batuman, MD, FACP, FASN more...
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CBC count may show leukocytosis and anemia.
Erythrocyte sedimentation rate (ESR) is usually elevated.
Blood urea nitrogen (BUN) and serum creatinine levels are usually elevated.
Electrolytes, especially the serum potassium level, are consistent with the degree of renal failure.
Urinalysis shows modest proteinuria (1-4 g/d), microscopic hematuria, RBCs, and RBC and WBC casts.
Proteinuria could be quantified by timed (24-h collection) or spot urine protein/creatinine ratio.
- Rarely, urine findings may be minimal.
- An absence of active urine sediment does not exclude a diagnosis of RPGN.
Complement levels (C3 and C4) are within reference ranges in patients with RPGN types I and III and may be decreased in patients with RPGN type II.
Circulating anti-GBM antibodies are detected in plasma of patients with RPGN type I, but this finding is neither 100% sensitive nor 100% specific for type I.
ANCAs: Typically, myeloperoxidase (MPO)–ANCA (previously known as perinuclear ANCA [p-ANCA]) is observed in 80-90% of patients with RPGN type III pauci-immune, but neither MPO-ANCA nor PR3-ANCA is 100% specific for type III.
Antinuclear antibody findings are usually negative (unless lupus related).
Serum cryoglobulin levels may be elevated in cryoglobulinemias and may be falsely negative.
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Abdominal ultrasound is used to assess renal size and echogenicity and to exclude obstruction.
Chest x-ray films are indicated for patients with suspected Goodpasture syndrome and vasculitides and to help manage pulmonary renal syndromes.
Sinus x-ray films and/or CT scans may show evidence of sinusitis in patients with GPA (Wegener granulomatosis).
Chest CT scans may show reticulonodular infiltrate, even when chest radiographic findings are normal and often mimics a neoplastic process.
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Renal biopsy is usually required to diagnose RPGN types I, II, and III.
Idiopathic RPGN is classified as follows:
Type I (anti-GBM disease with or without lung hemorrhage) - Linear deposits of IgG (5-20%)
Type II (immune complex-mediated disease) - Granular deposits of immunoglobulins by immunofluorescence and electron-dense deposits by electron microscopy (15-30%)
Type III (pauci-immune glomerulonephritis) - Little or no deposits observed by immunofluorescence or electron microscopy (55%)
The normal renal architecture is lost, and the glomerulus is solid, hypercellular, and surrounded by severe interstitial mononuclear cell infiltrate (see the first image below) is observed in patients with severe types. The glomerular tuft is distorted by proliferation of epithelial cells crescents that occupy most of the Bowman space as shown in the second, third, and fourth images below.
Anti-GBM disease is characterized by linear IgG deposits as depicted below. Endocapillary proliferation, if prominent, suggests the presence of infection. Segmental or diffuse endocapillary necrosis suggests underlying vasculitis.
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