A pulmonary renal syndrome is diagnosed when the combination of a rapid progressive
glomerulonephritis (RPGN) and diffuse pulmonary hemorrhage (DAH) are present. Both
are severe disease manifestations that are associated with a considerable morbidity
and mortality. Most frequently, the underlying cause is a small vessel vasculitis
due to anti-neutrophil cytoplasmatic antibody (ANCA) associated vasculitis (AAV) or
anti-glomerular basement membrane disease. Other vasculitis’s and connective tissue
diseases such as Systemic Lupus Erythematosus and Rheumatoid Arthritis present the
more rare causes of this syndrome.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Transfusion and Apheresis ScienceAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Anti-Neutrophil cytoplasmatic autoantibody-associated glomerulonephritis and vasculitis.Am J Pathol. 1989; 135: 921-930
- Molecular architecture of the Goodpasture autoantigen in Anti-GBM Nephritis.N Engl J Med. 2010; 363: 4343-4354
- The effects of plasma exchange in patients with ANCA-associated vasculitis; an updated systematic review and meta-analysis.BMJ. 2022; 376e064604
- EUVAS). Long-term follow-up of patients with severe ANCAassociated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear.Kidney Int. 2013; 84 (European Vasculitis Study Group): 397-402
- PEXIVAS Investigators. Plasmaexchange and glucocorticosteroids in severe ANCA-associated vasculitis.N Engl J Med. 2020; 382: 622-631
- Recovery from Goodpasture’s syndrome after immunosuppressive treatment and plasmapheresis.Br Med J. 1975; 2: 252-254
- Therapy of anti-glomerular basement membrane antibody disease: analysis of prognostic significance of clinical, pathologic and treatment factors.Medicine. 1985; 64: 219-227
- Apheresis to treat systemic vasculitis.Jt Bone Spine. 2018; 85: 177-183
- Complement inhibition in ANCA-associated vasculitis.Front Immunol. 2022; 13888816
- Eculizumab and complement activation in anti-glomerular basement membrane disease.Kidney Int Rep. 2021; 6: 2713-2717
Article info
Publication history
Published online: February 24, 2023
Publication stage
In Press Journal Pre-ProofIdentification
Copyright
© 2023 Elsevier Ltd. All rights reserved.
