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Vasculitis presentation final
- 1. VASCULITIS DJ Williamson Western Australian Department of Health
- 5. AGE AT ONSET Young Middle-age Old Henoch-Schönlein purpura Granulomatosis with polyangiitis Giant cell arteritis Takayasu arteritis (female) Microscopic polyangiitis Behçet disease Eosinophilic granulomatosis with polyangiitis Kawasaki disease Polyarteritis nodosa Cogan syndrome Hypersensitivity vasculitis Eales’ disease
- 7. OCULAR Orbital pseudo-tumour Episcleritis Scleritis Uveitis Retinal vasculitis Anterior ischaemic optic neuropathy Diplopia
- 8. SKIN & NAILS Palpable purpura Livedo reticularis Digital infarcts Splinter haemorrhages Erythema nodosum
- 10. AIRWAYS Sinusitis Upper airway Pulmonary infiltrates Diffuse alveolar haemorrhage semanticscholar.org
- 11. VASCULAR EVENT Stroke Retinal vascular occlusion Mesenteric infarction Mononeuropathy
- 13. DIFFERENTIAL DIAGNOSES Infection (Hepatitis, HIV, Syphilis, IE, TB, Weil’s) Atherosclerosis (Cholesterol embolism) Thromboembolism (APS, TTP) Connective tissue diseases (IgG4 disease) Drugs (Cocaine, Hydralazine) Malignancy Careful history, including drugs Screen for infection (including TB) Serology for CTDs (ANA, ENA, C’)
- 14. PATHOGENESIS
- 15. PATHOGENESIS Neutrophils have a central role C5a receptor inhibition
- 16. DIAGNOSIS Standardised clinical assessment Laboratory results Imaging Biopsy Cornec et al (2016) Nature Rev Rheum
- 17. p-ANCA: perinuclear staining pattern (MPO) c-ANCA: cytoplasmic staining pattern (PR3) Atypical: other staining pattern (Other) Recommended to check both indirect immunofluorescence and ELISA, at least initially
- 18. p-ANCA: MPA, EPGA > GPA IBD, PSC, RA, SLE c-ANCA: GPA > MPA > EPGA Atypical: PBC, PSC, RA, SLE Atypical autoantibodies directed against elastase in cocaine-induced midline destructive disease
- 19. DISEASE ASSOCIATIONS ANCA GPA MPA EGPA cANCA (PR3) 80% 40% 10% pANCA (MPO) 15% 50% 60% Negative 5% 10% 30% ANCA specificity predicts pattern of organ involvement False Positives common in infections such as tuberculosis, malaria and leprosy
- 20. PATTERNS
- 21. MPA Middle age Kidneys Systemic Skin Lungs Patient with AAV Mouse + anti-MPO Xiao, Hu, Falk, Jennette (2015)
- 23. EGPA Long prodrome, steroid reduction Asthma Eosinophilia Pulmonary infiltrates
- 24. POLYARTERITIS NODOSA Treat HBV or HCV if present Prognosis worse if old Prognosis worse if cardiac, GI, renal disease Often one episode but 5y relapse is 25% Necrotising vasculitis of medium-sized arterioles Renal microaneurysms
- 25. IGA VASCULITIS (HSP) Child or teenager Palpable purpura Arthralgia Abdominal pain (intussusception) Hypertension Haematuria (nephrotic syndrome) Early relapse Granular IgA and C3 in walls of small blood vessels Fibrinoid necrosis Palpable purpura
- 26. BEHÇET DISEASE More severe in young males Recurrent mucocutaneous ulceration Inflammatory eye disease Asymmetric non-erosive polyarthritis Arterial and venous disease Pathergy No diagnostic test
- 27. TAKAYASU ARTERITIS ~ 20% of vasculitis in India Young women Lower abdominal aorta Renovascular hypertension ‘Pulseless disease’ Imaging for diagnosis Imaging for monitoring (?) Vascular intervention and AVR Indian Takayasu Clinical Activity Score Mandal et al (2015) Ind J Rheumatol Angiography CT Angiography Doppler Ultrasound
- 28. GCA Elderly Headache Diplopia Visual loss Polymyalgia rheumatica Halo sign: disappears with Rx ‘Gold standard’ but often negative in large vessel disea
- 30. TREATMENT • Prednisolone • Urgent high doses (~1 mg/kg) if temporal arteritis • Monitor with symptoms and ESR/CRP • Significant side effects in ~80% • Wean over months • ‘Steroid-sparing’ drugs • Some evidence for Methotrexate but not strong • Only ~ 20% remain in remission with steroids alone • Biologics • TNF blockers not effective • Anti-IL-6 (Tocilizumab) recently approved • Bone protection • Bisphosphonates, calcium and vitamin D Steroids Steroid-sparing drugs Biologics Bone protection
- 31. ANCA-ASSOCIATED VASCULITIS 80% one year mortality without treatment Induce remission with minimal toxicity Maintain remission and minimise damage
- 32. REMISSION INDUCTION • Cyclophosphamide • IV Pulses 15 mg/kg over ~ 3 months • Reduced dose compared to oral induction • Counselling • Prednisolone • IV Pulses of Methylprednisolone 1 g/d x3 • Oral Prednisolone 1 mg/kg tapering over ~ 3 months • Plasma exchange • Severe renal failure • Anti-CD20 MAb (Rituximab/Mabthera) • Infusion 375 mg /week x4 • Alternative to Cyclophosphamide (eg if PR3 ANCA) Regime depends on severity
- 33. REMISSION MAINTENANCE • Azathioprine • 2 mg/kg/d • Check TPMT levels if available (?) • Prednisolone • 10-15 mg/d • Significant adverse effects • Anti-CD20 MAb (Rituximab/Mabthera) • Remission induction after relapse & maintenance • Preferable to more Cyclophosphamide / Azathioprine • Risk of hypogammaglobulinemia • Methotrexate • More likely to relapse • Greater requirement for steroids • Can be continued if used for induction • Avoid with Bactrim (sometimes used for nasal disease) Relapse in 70% over 10 years More common if PR3 ANCA Eliminate Staph aureus from nose
- 34. MONITORING Inflammatory markers ? Autoantibodies ? Novel biomarkers ?? Composite disease activity scores ? Composite damage indices ? Imaging ??
- 35. CO-MORBIDITIES • Infection • PJP prophylaxis while on Cyclophosphamide • Latent TB reactivation • Immunization • Cardiovascular • Vascular risk factors • Hypertension • Renal impairment • RRT and transplantation • Metabolic • Osteoporosis • Diabetes • Cancer • Urothelial cancer if exposed to Cyclophosphamide • Lymphoma and skin cancer Now major cause of morbidity & mortality
- 36. EGPA • Improved remission induction • Increased time to first relapse • Reduced steroid use Anti-interleukin 5 mAb biozentrum.uni-wuerzburg.de EGPA • Improved remission induction • Increased time to first relapse • Reduced steroid use Anti-interleukin 5 mAb biozentrum.uni-wuerzburg.de
- 37. SUMMARY