MSK - Vasculitis Flashcards
What is Aspirin exacerbated respiratory disease (AERD)?
Also called aspirin-induced asthma
What is included in Samter’s triad relating to AERD?
Samter’s Triad:
- Nasal polyps
- Asthma
-
Aspirin sensitivity - respiratory symptoms exacerbated by NSAIDs e.g. aspirin
- This is a hypersensitivity reaction as opposed to a true allergic reaction
Urea:Creatinine ratio can be used to screen for renal pathologies. What are the Urea:creatinine ratios for the following:
- Post-renal disease
- Renal disease or mixed pathology
- Pre-renal disease
- < 40 = Post-renal disease
- 40-100 = Renal disease or mixed pathology
- > 100 = Pre-renal disease
Which 5 systems of the body are commonly affected by the various types of vaculitis?
- Joints
- Lungs
- Skin
- Kidenys
- Nerves
Vasculitis is often catagorised as large, medium and small vessel. Split the following types of vasculitis into these catagories.
- Microscopic polyangiitis
- Kawasaki’s disease
- Polyarteritis nodosa
- Giant cell arteritis
- Granulomatosis with polyangiits (Wegener’s ganulomatosis)
- Takayasu’s arteritis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
Large vessel vasculitis:
- Giant cell arteritis
- Takayasu’s arteritis
Medium vessel vasculitis:
- Polyarteritis nodosa
- Kawasaki’s disease
Small vessel vasculitis:
- Microscopic polyangiitis
- Granulomatosis with polyangiits (Wegener’s ganulomatosis)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
What is the ANCA antibody?
What are the types of ANCA?
ANCA is an antibody (anti-neutrophil cytoplasmic antibody) used in the diagnosis of autoimmune disease
The two main types of ANCA are:
- C-ANCA - cytoplasmic ANCA
- P-ANCA - perinuclear ANCA
What are the most common targets for:
1) C-ANCA
2) P-ANCA
- C-ANCA commonest target = PR3 (proteinase 3) which is mostly found in the cytoplasm (hence the name C-ANCA)
- P-ANCA commonest target = MPO (myeloperoxidase) which is mostly found in the peri-nuclear region (hence the name P-ANCA)
What conditions are associated with +ve:
- C-ANCA
- P-ANCA
C-ANCA:
- Granulomatosis with polyangiitis (Wegner’s granulomatosis) - 90%
- Microscopic polyangiitis (30%)
P-ANCA:
- Immune crescentic (rapidly progressive) glomerulonephritis (80%)
- Can present with nephritic or nephrotic clinical features
- Microscopic polyangiits (50-75%)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (60%)
- Primary sclerosing cholangitis (60-80%)
- Granulomatosis with polyangiitis (25%)
ANCA can also be +ve in the following:
- IBD (UC > Crohn’s)
- Connective tissue disorders: RA, SLE, Sjogren’s
- Autoimmune hepatitis
What is the name of the classic diagnostic criteria for diagnosing eosinophilic granulomatosis with polyangiitis (EGPA) i.e. Churg-Strauss - and what does it involve?
Lanham diagnostic criteria for EGPA
Triad of:
- Asthma
- Eosinophilia
- Multi-organ involvement
Other symptoms:
- Mononeuritis multiplex
- Paranasal sinisitus
The updated ACR criteria (6 things) for diagnosing EGPA include what?
ACR criteria: 4/6 = good sensitivity + specificity
- Asthma
- Eosinophilia ( >10% of total WBC)
- Neuropathy
- Pulmonary infiltrates, non-fixed
- Paranasal sinus abnormalities e.g. pain, tenderness, radiographic opacification
- Extravascular eosinophils
What does the treatment pathway look like for
AAV (ANCA-associated vasculitis)?
-
Rapid induction of immunosuppression (especially in multi-organ involvement)
- E.g. steroids (prednisolone or methylprednisolone) + 2nd immunosuppressant e.g. cyclophosphamide or rituximab
- Once remission is induced –> switch to steroid-sparing agent for long-term treatment
- E.g. methotrexate, azathioprine or mycophenolate mofetil (MMF)
When starting patients on high-dose steroids what other factors do you need to consider?
- Gastro-protection e.g. PPI - due to ↑ risk of peptic ulcer (+/- perf), dyspepsia, oesophageal ulcer
- Bone protection e.g. bisphosphonates - due to risk of osteoporosis
- Screen for diabetes - HbA1c 1 month after initiation –> then every 3 months, close monitoring of diabetic pts
- Monitor blood pressure/weight - weight management advice + anti-hypertensives if necessary
What is EGPA?
It is eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) - it is an ANCA associated small-medium vessel vasculitis
What are the main features of EGPA (Churg-Strauss) ?
Features:
-
Ophthalmology
- anterior uveitis
-
Nervous system
- mononeuritis multiplex (inflammation of ≥ 2 nerves, often in unrelated parts of the body) and strokes
-
Upper resp tract
- allergic rhinitis (hayfever), paranasal sinusitis + nasal polyps
-
Lower resp tract
- asthma, haemoptysis and pneumonitis
-
Heart
- pericardial effusion, MI and myocarditis (cardiac involvement is a negative prognostic marker)
-
Gastroenterology
- oesophagitis, gastritis, cholecystitis, mesenteric infarction and bowel perforation
-
Skin
- purpura skin nodules and livedo reticularis
-
Renal
- crescentic (rapidly progressive) glomerulonephritis, HTN and renal failure
Which drug classicaly causes an exacerbation of asthma symptoms in someone with EGPA?
Leukotriene receptor antagonists
E.g. montelukast
- Associated with worsening asthma symptoms in those with EGPA
- Mechanism unclear - thought to occur due to facilitating systemic glucocorticoid withdrawel or
