Summary
Definition
History and exam
Key diagnostic factors
- reduced urine output
- hemoptysis
- edema
Other diagnostic factors
- male gender
- age 20 to 30 years or 60 to 70 years
- shortness of breath
- cough
- fever
- nausea
- crackles on lung examination
Risk factors
- HLA-DRB1 or DR4
Diagnostic investigations
1st investigations to order
- renal function testing
- renal biopsy
- antiglomerular basement membrane (anti-GBM) antibody titer
- antineutrophil cytoplasmic antibodies (ANCA)
- serum complement (C3 and C4)
- antinuclear antibody
- cryoglobulins
- hepatitis panel
- antistreptolysin O titer
- clotting screen
Treatment algorithm
reversible renal disease or any pulmonary involvement
irreversible renal disease
Contributors
Authors
Richard G. Phelps, BChir
Senior Lecturer and Honorary Consultant in Nephrology
University of Edinburgh and Edinburgh Royal Infirmary
Edinburgh
UK
Disclosures
RGP has helped Genzyme develop educational materials for neurologists regarding the increased risk of Goodpasture disease with alemtuzumab use. RGP has published an article on post-alemtuzumab nephritis in which employees of Genzyme were co-authors.
Acknowledgements
Dr Richard G. Phelps would like to gratefully acknowledge Professor Andre A. Kaplan and Dr Michael S. Gersch, previous contributors to this topic. AAK has received honoraria in 2013 to speak about plasma exchange from Gambro and Alexion (Gambro manufactures products for plasma exchange and Alexion manufactures a complement cascade inhibitor). MSG declares that he has no competing interests.
Peer reviewers
Dorin-Bogdan Borza, PhD
Assistant Professor of Medicine
Division of Nephrology
Department of Medicine
Vanderbilt University School of Medicine
Nashville
TN
Disclosures
DBB declares that she has no competing interests.
Differentials
- Systemic lupus erythematosus
- Granulomatosis with polyangiitis (Wegener)
- Microscopic polyangiitis
More DifferentialsGuidelines
- Guidelines on the use of therapeutic apheresis in clinical practice
- KDIGO clinical practice guideline for glomerulonephritis
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