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Crescentic Glomerulonephritis Workup

  • Author: Malvinder S Parmar, MB, MS; Chief Editor: Vecihi Batuman, MD, FACP, FASN  more...
 
Updated: Dec 21, 2014
 

Laboratory Studies

See the list below:

  • 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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Imaging Studies

See the list below:

  • 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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Procedures

See the list below:

  • Renal biopsy is usually required to diagnose RPGN types I, II, and III.
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Histologic Findings

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.

Glomerulonephritis, crescentic. Light microscopy ( Glomerulonephritis, crescentic. Light microscopy (200x hematoxylin and eosin stain): The normal renal architecture is lost. The glomerulus (*) is solid and hypercellular and surrounded by severe interstitial inflammatory infiltrate. Image courtesy of Suzanne Meleg-Smith, MD, Department of Pathology, Tulane University School of Medicine, New Orleans.
Glomerulonephritis, crescentic. Light microscopy ( Glomerulonephritis, crescentic. Light microscopy (25x hematoxylin and eosin stain): Compression of the glomerular tuft with a circumferential cellular crescent that occupies most of the Bowman space. Image courtesy of Madeleine Moussa, MD, FRCPC, Department of Pathology, London Health Sciences Centre, London, Ontario, Canada.
Glomerulonephritis, crescentic. Light microscopy ( Glomerulonephritis, crescentic. Light microscopy (200x periodic acid-Schiff stain): Bowman capsule (arrow) surrounds each glomerulus. The glomerular tuft (*) is distorted by a proliferation of epithelial cells (crescent), which replaces the urinary space. Image courtesy of Suzanne Meleg-Smith, MD, Department of Pathology, Tulane University School of Medicine, New Orleans.
Glomerulonephritis, crescentic. Light microscopy ( Glomerulonephritis, crescentic. Light microscopy (400x trichrome stain): The remnant of the glomerular tuft (*) is surrounded by the cellular crescent with abundant fibrin–red on trichrome stain. Interstitial edema separates the tubules, and scarce inflammatory cells are present. Image courtesy of Suzanne Meleg-Smith, MD, Department of Pathology, Tulane University School of Medicine, New Orleans.

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.

Glomerulonephritis, crescentic. Immunofluorescence Glomerulonephritis, crescentic. Immunofluorescence (25x): Anti–glomerular basement membrane characterized by the presence of linear immunoglobulin G deposit along the glomerular basement membrane. Image courtesy of Madeleine Moussa, MD, FRCPC, Department of Pathology, London Health Sciences Centre, London, Ontario, Canada.
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Contributor Information and Disclosures
Author

Malvinder S Parmar, MB, MS FRCP(C), FACP, FASN, Associate Professor, Department of Internal Medicine, Northern Ontario School of Medicine; Assistant Professor, Department of Medicine, University of Ottawa Faculty of Medicine; Consulting Physician, Timmins and District Hospital, Ontario, Canada

Malvinder S Parmar, MB, MS is a member of the following medical societies: American College of Physicians, American Society of Nephrology, Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ajay K Singh, MB, MRCP, MBA Associate Professor of Medicine, Harvard Medical School; Director of Dialysis, Renal Division, Brigham and Women's Hospital; Director, Brigham/Falkner Dialysis Unit, Faulkner Hospital

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FACP, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Renal Section, Southeast Louisiana Veterans Health Care System

Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, International Society of Nephrology

Disclosure: Nothing to disclose.

Additional Contributors

James H Sondheimer, MD, FACP, FASN Associate Professor of Medicine, Wayne State University School of Medicine; Medical Director of Hemodialysis, Harper University Hospital at Detroit Medical Center; Medical Director, DaVita Greenview Dialysis (Southfield)

James H Sondheimer, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Nephrology

Disclosure: Receive dialysis unit medical director fee (as independ ent contractor) for: DaVita .

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Glomerulonephritis, crescentic. Light microscopy (25x hematoxylin and eosin stain): Compression of the glomerular tuft with a circumferential cellular crescent that occupies most of the Bowman space. Image courtesy of Madeleine Moussa, MD, FRCPC, Department of Pathology, London Health Sciences Centre, London, Ontario, Canada.
Glomerulonephritis, crescentic. Light microscopy (200x hematoxylin and eosin stain): The normal renal architecture is lost. The glomerulus (*) is solid and hypercellular and surrounded by severe interstitial inflammatory infiltrate. Image courtesy of Suzanne Meleg-Smith, MD, Department of Pathology, Tulane University School of Medicine, New Orleans.
Glomerulonephritis, crescentic. Light microscopy (200x periodic acid-Schiff stain): Bowman capsule (arrow) surrounds each glomerulus. The glomerular tuft (*) is distorted by a proliferation of epithelial cells (crescent), which replaces the urinary space. Image courtesy of Suzanne Meleg-Smith, MD, Department of Pathology, Tulane University School of Medicine, New Orleans.
Glomerulonephritis, crescentic. Light microscopy (400x trichrome stain): The remnant of the glomerular tuft (*) is surrounded by the cellular crescent with abundant fibrin–red on trichrome stain. Interstitial edema separates the tubules, and scarce inflammatory cells are present. Image courtesy of Suzanne Meleg-Smith, MD, Department of Pathology, Tulane University School of Medicine, New Orleans.
Glomerulonephritis, crescentic. Immunofluorescence (25x): Anti–glomerular basement membrane characterized by the presence of linear immunoglobulin G deposit along the glomerular basement membrane. Image courtesy of Madeleine Moussa, MD, FRCPC, Department of Pathology, London Health Sciences Centre, London, Ontario, Canada.
 
 
 
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