All::Rheumatology::Diseases::ANCA associated vasculitis
Intro
What are the common small-vessel vasculitides associated with Anti-neutrophil cytoplasmic antibodies (ANCA)?
Granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), and microscopic polyangiitis.
What are the common findings in ANCA associated vasculitis?
Renal impairment, respiratory symptoms (dyspnea, hemoptysis), systemic symptoms (fatigue, weight loss, fever), vasculitic rash (minority), and ear, nose, and throat symptoms (sinusitis).
What are the main types of ANCA, and which conditions are associated with each?
cANCA is associated with granulomatosis with polyangiitis, and pANCA is associated with eosinophilic granulomatosis with polyangiitis and others.
What are the target proteins for cANCA and pANCA?
cANCA targets Serine proteinase 3 (PR3), and pANCA targets myeloperoxidase (MPO).
What is the general approach to first-line investigations for ANCA associated vasculitis?
Urinalysis for haematuria and proteinuria, bloods for renal impairment, full blood count, CRP, and ANCA testing, and chest x-ray for potential lung lesions.
How should ANCA associated vasculitis be managed?
Managed by specialist teams for an exact diagnosis; mainstay of management is immunosuppressive therapy.