Abstract

Anti-neutrophil cytoplasm antibodies (ANCA) are associated with small vessel vasculitides (AASV) affecting the lungs and kidneys. Structured clinical assessment using the Birmingham Vasculitis Activity Score and Vasculitis Damage Index should form the basis of a treatment plan and be used to document progress, including relapse. Severe disease with organ or life threatening manifestations needs cyclophosphamide or rituximab, plus high dose glucocorticoids, followed by lower dose steroid plus azathioprine, or methotrexate. Additional plasmapheresis is effective for very severe disease, reducing dialysis dependence from 60 to 40% in the first year, but with no effect on mortality or long-term renal function, probably due to established renal damage. In milder forms of ANCA-associated vasculitis, methotrexate, leflunomide, or mycophenolate mofetil are effective. Mortality depends on initial severity: 25% in patients with renal failure or severe lung hemorrhage; 6% for generalized non-life threatening AASV but rising to 30–40% at 5 years. Mortality from GPA is four times higher than the background population. Early deaths are due to active vasculitis and infection. Subsequent deaths are more often due to cardiovascular events, infection, and cancer. We need to improve the long-term outcome, by controlling disease activity but also preventing damage and drug toxicity. By contrast, in large vessel vasculitis where mortality is much less but morbidity potentially greater, such as giant cell arteritis (GCA) and Takayasu arteritis, therapeutic options are limited. High dose glucocorticoid results in significant toxicity in over 80%. Advances in understanding the biology of the vasculitides are improving therapies. Novel, mechanism based therapies such as rituximab in AASV, mepolizumab in eosinophilic granulomatosis with polyangiitis, and tocilizumab in GCA, but the lack of reliable biomarkers remains a challenge to progress in these chronic relapsing diseases.

Highlights

  • The systemic vasculitides are a complex set of overlapping conditions whose natural history has been significantly modified by current therapies but continue to challenge patients and clinicians

  • For patients with large vessel vasculitis such as giant cell arteritis (GCA) or Takayasu arteritis, the primary treatment is glucocorticoids, but as we identify disease mechanisms, we should be able to use targeted therapies, avoiding the use of high doses of steroids, which result in very significant toxicity in over 80% [31]

  • Less effective than azathioprine as a maintenance agent, this drug has a place in management of Anti-neutrophil cytoplasm antibodies (ANCA) vasculitis

Read more

Summary

Raashid Ahmed Luqmani *

NDORMS, Rheumatology Department, Nuffield Orthopaedic Centre, University of Oxford, Oxford, UK. Severe disease with organ or life threatening manifestations needs cyclophosphamide or rituximab, plus high dose glucocorticoids, followed by lower dose steroid plus azathioprine, or methotrexate. Additional plasmapheresis is effective for very severe disease, reducing dialysis dependence from 60 to 40% in the first year, but with no effect on mortality or long-term renal function, probably due to established renal damage. Mortality depends on initial severity: 25% in patients with renal failure or severe lung hemorrhage; 6% for generalized non-life threatening AASV but rising to 30–40% at 5 years. We need to improve the long-term outcome, by controlling disease activity and preventing damage and drug toxicity. Mechanism based therapies such as rituximab in AASV, mepolizumab in eosinophilic granulomatosis with polyangiitis, and tocilizumab in GCA, but the lack of reliable biomarkers remains a challenge to progress in these chronic relapsing diseases

INTRODUCTION
For most other forms of systemic
Hair loss
Plasmapheresis Induction
Increased risk of sepsis especially if combined with cyclophosphamide
Induction Maintenance
Nausea Diarrhea Mouth ulcers Hair loss Cytopenia Liver dysfunction Hypertension
Kawasaki disease is the main form of immunoglobulin
Resistant disease
Findings
DISCUSSION

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call

Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.