Systemic vasculitis Flashcards
What is the general management of systemic vasculitis?
Conservative Management: Patient counselling, Control CV RF
Medical therapy
- Treat complications (organ damage, angioplasty, bypass etc)
- Induce remission w/ high dose steroid therapy w/ bisphosphonate & Omeprazole coverage
- Consider adjunctive immunosuppressants if non-responsive to steroid therapy
- Ultimately aiming for a steroid-free remission
What are the types of large vessels vasculitis(aorta and its tributaries)?
- Giant cell arteritis (GCA),/ Polymyalgia rheumatic (PMR)
- Takayasu arteritis (TKA)
What are the types of medium vessels vasculitis (medium and small arteries and arterioles)?
- Polyarteritis nodosa (PAN)
- Kawasaki’s disease
What are the types of medium small vasculitis (ANCA +ve)?
- p-ANCA: Microscopic polyangiitis (MPA), Churg-Strauss (ie Eosinophilic Granulomatosis w polyangiitis)
- C-ANCA: Wegener’s granulomatosis (GWP; ie Granulomatosis with Polyangiitis)
What are the types of medium small vasculitis (ANCA -ve) (small arteries, arterioles, venules and capillaries) ?
- Henoch-Schonlein purpura
- Cutaneous leukocytoclastic vasculitis
- Essential cryoglobulinemia
What are the types of variable vessel vasculitis (affects vessel of any size & type)?
Behcet’s Disease
What are the common organ specific manifestations of giant cell arteritis?
- thickened temporal artery
- eye: sudden blindness
- jaw claudication
- pain in upper arms and legs
- rarely coronary artery or large vessel involvement
What are the common organ specific manifestations of Takayasu arteritis?
- Aortic insufficiency
- Inflammation of aorta and large branches
- reduced blood pressure and weak pulse in one arm compared to another
- renal dysfunction
What are the common organ specific manifestations of Polyarteritis nodosa ?
- coronary arteritis
- digital vasculitis and gangrene
- rash e.g. livdeo reticularis
- neuropathy e.g. foot drop
- abdominal pain and hemorrhage
- glomerulonephritis
What are the common organ specific manifestations of Kawasaki’s disease?
- painful swollen cervical lymph nodes
- dry sore mouth
- conjunctivitis
- coronary arteritis
- red swollen palms and soles
- polymorphic rash anywhere on the body
What are the common organ specific manifestations of Microscopic polyangititis ?
- ENT symptoms
- Pulmonary fibrosis
- Glomerulonephritis
- Rash
- Peripheral neuropathy
What are the common organ specific manifestations of Churg- Strauss granulomatosis ?
- Late onset asthma
- Eosinophilia in blood
- Glomerulonephritis
- Rash
- Peripheral neuropathy
What are the common organ specific manifestations of Wegener’s granulomatosis?
- ENT symptoms e.g. nose bleeds and deafness
- Pulmonary fibrosis or hemorrhage
- Glomerulonephritis
- Neuropathy