Vasculitis Flashcards
What investigations would be done for presentation of:
- swollen joint, warm to touch, swelling feels boggy, no tenderness and erythema
- fatigue
- asthma
- Microscopy and cytology - help determine whether an inflammatory process is occurring
- FBC, U+E, LFT, CRP, PV - inflammatory process
- TFTs - hypothyroidism can present with joint pains and fatigue
- Multi-system disease - screen for IA/CTD/vasculitis; RF, anti-CCP antibody, ANA + ANCA
- Knee/chest X-RAY
- Urine dip
What types of large cell vasculitis are there?
Aorta > arteries
- Giant cell arteritis
- Takayasu’s arteritis
What types of medium vessel vasculitis are there?
Arteries > arterioles
- Kawasaki’s disease
- Polyarteritis nodosa (PAN)
What types of small vessel vasculitis are there?
Capillaries > venues > veins
- Microscopic polyangitis
- Eosinophilic granulomatosis with polyangitis (Churg-Strauss disease)
- Granulomatosis with polyangitis (Wegener’s)
- HSP
What is c-ANCA?
A positive c-ANCA reveals the anti-neutrophil cytoplasmic antibody is preferably targeting PR3, which if found mainly in the cytoplasm. Classically, Wegener’s/GPA is associated with c-ANCA.
What are the key features of Wegener’s?
- URT: nasal crusting, epistaxis, sinusitis
- LRT: dyspnoea, haemoptysis, cavitating lesions in the lung on XR
- Saddle nose deformity
- Vasculitic rash, eye involvement e.g. proptosis
- Cranial nerve involvement
- Renal biopsy: epithelial crescents in Bowman’s capsule
What is p-ANCA?
MPO (myeloperoxidase) and this is found more in the peri-nuclear region. It is most commonly found in microscopic polyangitis and eosinophilic granulomatosis with polyangitis.
What is the ACR criteria for EGPA?
- Asthma
- Eosinophilia of >10% in peripheral blood
- Paranasal sinusitis
- Pulmonary infiltrates
- Histological confirmation of vasculitis with extravascular eosinophils
- Mononeuritis multiplex or polyneuropathy
4/6 is high specificity and sensitivity
What is a common manifestation of vasculitis?
Glomerulonephritis - blood and protein in urine. If there is blood, send it off for red cell casts. These would indicate nephritic syndrome - proof of multi-system involvement.
What is crescenteric glomerulonephritis?
Serious complication of ANCA-associated vasculitides. Can present with both nephrotic and nephritic features clinically. Light microscopy shows crescent formation in the glomerulus. Immunosuppression is required for this.
What is the treatment progression for vasculitis?
- Rapid induction of immunosuppression with steroids (prednisolone or methylprednisolone) with 2nd immunosuppressant e.g. cyclophosphamide or rituximab
- Once remission achieved, steroid-sparing agent e.g. methotrexate, azathioprine or MMF
What do you need to consider with high dose steroids?
- Gastro-protection
- Bone protection
- Screen for diabetes
- Monitor BP/weight
What are common respiratory manifestations of EGPA?
- Asthma
- Haemoptysis
- Pneumonitis
- Allergic rhinitis
- Paranasal sinusitis
- Nasal polyps
What are common cardiac manifestations of EGPA?
- Pericardial effusion
- MI
- Myocarditis
What are common skin manifestations of EGPA?
- Purpura skin nodules
- Livedo reticularis