eMedicine Specialties > Nephrology > Glomerular Diseases

Glomerulonephritis, Crescentic: Differential Diagnoses & Workup

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

Updated: Sep 25, 2008

Differential Diagnoses

Acute Renal Failure
Nephritis, Interstitial
Glomerulonephritis, Acute
Thrombotic Thrombocytopenic Purpura
Hemolytic-Uremic Syndrome
Hypertension
Hypertension, Malignant

Workup

Laboratory Studies

  • 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, perinuclear ANCA (p-ANCA) is observed in 80-90% of patients with RPGN type III pauci-immune, but neither p-ANCA nor 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.

Imaging Studies

  • 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 Wegener granulomatosis.
  • Chest CT scans may show reticulonodular infiltrate, even when chest radiographic findings are normal.

Procedures

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

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 Media file 2) 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 (see Media file 1 and Media files 3-4).

Anti-GBM disease is characterized by linear IgG deposits (see Media file 5). Endocapillary proliferation, if prominent, suggests the presence of infection. Segmental or diffuse endocapillary necrosis suggests underlying vasculitis.

More on Glomerulonephritis, Crescentic

Overview: Glomerulonephritis, Crescentic
Differential Diagnoses & Workup: Glomerulonephritis, Crescentic
Treatment & Medication: Glomerulonephritis, Crescentic
Follow-up: Glomerulonephritis, Crescentic
Multimedia: Glomerulonephritis, Crescentic
References
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References

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Further Reading

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Keywords

crescentic glomerulonephritis, idiopathic crescentic glomerulonephritis, idiopathic rapidly progressive glomerulonephritis, RPGN, GN, renal failure, kidney failure, acute nephritic syndrome, anti–glomerular basement membrane disease, anti-GBM disease, glomerulopathy, glomerular filtration rate, GFR, antineutrophil cytoplasmic antibody, ANCA, azotemia, oliguria, Goodpasture syndrome, Wegener granulomatosis, systemic lupus erythematosus, SLE, poststreptococcal glomerulonephritis, PSGN, membranoproliferative glomerulonephritis, MPGN

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Contributor Information and Disclosures

Author

Malvinder S Parmar, MB, MS, FRCP(C), FACP, FASN, Assistant Professor (VPT), Faculty of Medicine, University of Ottawa; Associate Professor, Department of Internal Medicine, Northern Ontario School of Medicine; Consulting Physician, Timmins and District Hospital, Ontario, Canada
Malvinder S Parmar, MB, MS, FRCP(C), FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Nephrology, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

James H Sondheimer, MD, Director of Hemodialysis Unit, Harper Hospital; Associate Professor, Department of Internal Medicine, Division of Nephrology, Wayne State University School of Medicine
James H Sondheimer, MD is a member of the following medical societies: American College of Physicians and American Society of Nephrology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ajay K Singh, MB, MRCP, MBA, Associate Professor of Medicine, Director of Dialysis, Department of Medicine, Harvard Medical School; Clinical Chief of Renal Division, Brigham and Women's Hospital
Disclosure: Nothing to disclose.

CME Editor

Rebecca J Schmidt, DO, FACP, FASN, Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine
Rebecca J Schmidt, DO, FACP, FASN is a member of the following medical societies: American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation, Renal Physicians Association, and West Virginia State Medical Association
Disclosure: Abbott Grant/research funds Speaking and teaching; Genzyme Honoraria Consulting; Roche Honoraria Consulting

Chief Editor

Vecihi Batuman, MD, FACP, FASN, Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Medicine Service, 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, and International Society of Nephrology
Disclosure: Nothing to disclose.

 
 
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