ANCA-associated vasculitis
Jump to navigation
Jump to search
Etiology
- Wegener's granulomatosis (granulomatosis with polyangiitis)
- microscopic polyangiitis
- Churg-Strauss disease (eosinophilic granulomatosis with polyangiitis)
Pathology
- see anti-neutophil cytoplasmic antibody (ANCA)
Clinical manifestations
- pauci-immune glomerulonephritis
- pulmonary hemorrhage, hemoptysis
- epistaxis
- peripheral neuropathy, mononeuritis multiplex[1]
Laboratory
- c or central type cANCA in serum (Wegener's granulomatosis)
- p or peripheral type pANCA in serum (Churg-Strauss disease, microscopic polyangiitis, other)
- cocaine-associated vasculitis both cANCA in serum & pANCA in serum
Radiology
Complications
- incidence of stroke is higher than in the general population (RR 1.8),
- RR= 3.2 in patients < 65 years[13]
- high platelet count at diagnosis is associated with increased risk[13]
Management
- combination therapy (Wegener's granulomatosis, microscopic polyangiitis)
- rituximab 375 mg/m2/week (4 doses) in combination with glucocorticoids preferred induction for severe disease[10]
- induction & maintenance of remission[4][6]
- if cyclophosphamide is contraindicated, not tolerated, or is otherwise unacceptable (NICE) [NGC]
- reduced-dose prednisolone (0.5 mg/kg/day) non-inferior to high-dose prednisolone (1.0 mg/kg/day)[9]
- methotrexate in combination with glucocorticoid preferred induction for less severe disease[10]
- rituximab 375 mg/m2/week (4 doses) in combination with glucocorticoids preferred induction for severe disease[10]
- combination therapy (Churg-Strauss syndrome)
- glucocorticoid plus cyclophosphamide or rituximab is recommended to induce remission in severe disease
- glucocorticoid plus mepolizumab or methotrexate, azathioprine of mycophenolate mofetil to induce remission in less severe disease
- other modalities
- plasmapheresis[5]
- cyclophosphamide
- 2 mg/kg/day PO for one year after induction of remission
- monthly cyclophosphamide is less toxic, but also less effective
- azathioprine 1-2 mg/kg/day:
- useful in maintaining remissions induced by cyclophosphamide
- use in conjunction with prednisone as needed[1]
- prednisone 1 mg/kg PO QD for 4 weeks followed by slow taper, subsequent conversion to alternate day therapy for 6-9 months
- daratumumab (Darzalex) inhibits CD38-positive plasma cells that may linger after anti-CD20 (rituximab) treatment[12]
- prognosis
- rule out infection prior to initiating immunosuppressive therapy
- immunosuppressive therapy reduces risk of ESRD or death from 51% to 31%[1]
More general terms
More specific terms
- allergic angiitis & granulomatosis of Churg-Strauss; eosinophilic granulomatosis with polyangiitis (EGPA)
- ANCA-associated glomerulonephritis
- cocaine-associated vasculitis
- granulomatosis with polyangiitis; Wegener's granulomatosis
- microscopic polyangiitis
Additional terms
References
- ↑ Jump up to: 1.0 1.1 1.2 1.3 1.4 Medical Knowledge Self Assessment Program (MKSAP) 16,17,18 American College of Physicians, Philadelphia 2012,2015,2018.
- ↑ Jayne DR, Gaskin G, Rasmussen N et al Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. 2007 Jul;18(7):2180-8. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17582159
- ↑ Bosch X et al, Antineutrophil cytoplasmic antibodies. Lancet 2006, 368:404 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16876669
- ↑ Jump up to: 4.0 4.1 Specks U et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med 2013 Aug 1; 369:417 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/23902481 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMoa1213277
- ↑ Jump up to: 5.0 5.1 Gaffo AL. Diagnostic approach to ANCA-associated vasculitides. Rheum Dis Clin North Am. 2010 Aug;36(3):491-506 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20688246
- ↑ Jump up to: 6.0 6.1 Stone JH, Merkel PA, Spiera R et al Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010 Jul 15;363(3):221-32 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20647199
- ↑ Jump up to: 7.0 7.1 Rutgers A, Sanders JS, Stegeman CA, Kallenberg CG. Pauci-immune necrotizing glomerulonephritis. Rheum Dis Clin North Am. 2010 Aug;36(3):559-72 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20688250
- ↑ Poulton CJ, Nachman PH, Hu Y et al Pathways to renal biopsy and diagnosis among patients with ANCA small-vessel vasculitis. Clin Exp Rheumatol. 2013 Jan-Feb;31(1 Suppl 75):S32-7. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23343774 Free PMC Article
- ↑ Jump up to: 9.0 9.1 Furuta S, Nakagomi D, Kobayashi Y et al Effect of Reduced-Dose vs High-Dose Glucocorticoids Added to Rituximab on Remission Induction in ANCA-Associated Vasculitis. A Randomized Clinical Trial. JAMA. 2021;325(21):2178-2187 PMID: https://www.ncbi.nlm.nih.gov/pubmed/34061144 https://jamanetwork.com/journals/jama/fullarticle/2780489
- ↑ Jump up to: 10.0 10.1 10.2 Chung SA, Langford CA, Maz M et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol 2021 Aug; 73:1366-1383. PMID: https://www.ncbi.nlm.nih.gov/pubmed/34235894 https://onlinelibrary.wiley.com/doi/10.1002/art.41773
- ↑ Hunter RW, Welsh N, Farrah TE et al. ANCA associated vasculitis. BMJ. 2020;369:m1070 PMID: https://www.ncbi.nlm.nih.gov/pubmed/32291255 PMCID: PMC7179255 Free PMC article https://www.bmj.com/content/369/bmj.m1070
- ↑ Jump up to: 12.0 12.1 Rixecker TM, Leppervpm, Mang S et al Daratumumab for a Patient With Refractory Antineutrophil Cytoplasmatic Antibody- Associated Vasculitis. JAMA Intern Med. Published online April 10, 2023 PMID: https://www.ncbi.nlm.nih.gov/pubmed/37036724 https://jamanetwork.com/journals/jamainternalmedicine/fullarticle/2803783
- ↑ Jump up to: 13.0 13.1 13.2 Tabakovic D, Smith R, Jayne D, Mohammad AJ. High Risk of Stroke in ANCA-associated Vasculitis. A Population-Based Study. Rheumatology. 2023;62(8):2806-2812 PMID: https://www.ncbi.nlm.nih.gov/pubmed/36440920 PMCID: PMC10393431 Free PMC article https://www.medscape.com/s/viewarticle/995641
- ↑ Wallace ZS, Miloslavsky EM. Management of ANCA associated vasculitis. BMJ. 2020 Mar 18;368:m421. PMID: https://www.ncbi.nlm.nih.gov/pubmed/32188597 Review.
- ↑ Nakazawa D, Masuda S, Tomaru U, Ishizu A. Pathogenesis and therapeutic interventions for ANCA-associated vasculitis. Nat Rev Rheumatol. 2019 Feb;15(2):91-101. PMID: https://www.ncbi.nlm.nih.gov/pubmed/30542206 Free article. Review.
- ↑ Kallenberg CG. Key advances in the clinical approach to ANCA-associated vasculitis. Nat Rev Rheumatol. 2014 Aug;10(8):484-93. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24980139 Review.