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
What are common renal manifestations of EGPA?
- Crescenteric glomerulonephritis
- HTN
- Renal failure
What are common neurological manifestations of EGPA?
- Mononeuritis multiplex
- Strokes
What are common eye and gastro manifestations of EGPA?
- Anterior uveitis
- Mesenteric infarction
- Bowel perforation
How does GCA present?
- > 60yrs
- Temporal headache
- Palpable temporal artery with absent pulsation
- Jaw claudication
- Scalp tenderness
- Sometimes systemic - fever and lethargy
- Visual disturbances: double vision, blurring, amaurosis fugax
What is Kawasaki disease?
- Commonly seen in children, important to catch due to serious complications such as coronary aortic aneurysms (echocardiogram screening)
- Persistent fever (>5 days)
- Lymphadenopathy
- Strawberry tongue
- Bilateral non-infective conjunctivitis, red eyes
- Desquamating rash
- Red palms and soles of feet which later peel
What is Polyarteritis Nodosa?
- Associated with HepB and more common in males
- Medium sized arteries which lead to aneurysms which can accumulate along the vessels forming a “rosary sign”
- Systemic: fever, malaise, arthralgia, weight loss
- Hypertension
- Mononeuritis multiplex, sensorimotor polyneuropathy
- Testicular pain
- Livedo reticularis
- Haematuria, renal failure
- P-ANCA
What is Takayasu’s disease?
- Large vessel vasculitis
- Causes absent pulses in the branches of the aorta leading to lack of pulses in upper limbs
- Can affect the kidneys
- Systemic: malaise, headache
- Unequal BP in upper limbs
- Carotid bruit
- Intermittent claudication
- Aortic regurgitation
- Management: steroids
What is the management for GPA?
- Steroids
- Cyclophosphamide
- Plasma exchange
- Median survival - 8-9yrs
What is the treatment for GCA?
- High dose glucocorticoids should be started before the temporal artery biopsy comes back
- Prednisolone if no visual loss
- IV methylprednisolone if there is evolving visual loss
- If there is vision changes they should be seen the same day by an ophthalmologist
- Bone protection with bisphosphonates as long tapering course of steroids
