[10] Vasculitis Flashcards
What are the classification of vasculitis?
Large vessel
Medium vessel
Small vessel
Give two examples of large vessel vasculitis
Giant Cell Arteritis
Takayasu’s arteritis
Give two examples of medium vessel vasculitis
Polyarteritis nodosa
Kawasaki disease
How can small vessel vasculitides be further classified?
pANCA
cANCA
ANCA -ve
Give two examples of cANCA small vessel vasculitis
Churg-Strauss
Microscopic polyangiits
Give an example of a cACNA small vessel vasculitis
Wegener’s granulomatosis
Give 4 examples of a ANCA -ve small vessel vasculitis
Henoch-Schonlein purpura
Goodpasture’s disease
Cryoglobulinaemia
Cutaneous leukocytoclastic vasculitis
What is giant cell arteritis also known as?
Temporal arteritis
Who is giant cell arteritis common in?
Elderly
Who is giant cell arteritis rare in?
<55
What % of cases of giant cell arteritis are associated with polymyalgia rheumatica?
50%
What are the features of giant cell arteritis?
Systemic signs Headache Temporal artery and scalp tenderness Jaw claudication Amaurosis fugax Prominent temporal arteries, with or without pulsation
What are the systemic signs of giant cell arteritis?
Fever
Malaise
Fatigue
What action should be taken if you suspect a patient has giant cell arteritis?
Do an ESR, and start prednisolone 40-60mg/day PO
What may be found on blood tests in giant cell arteritis?
Greatly increased ESR and CRP
Increased ALP
Decreased Hb
Increased platelets
What kind of anaemia is present in giant cell arteritis?
Normocytic normochromic
How quickly should a temporal artery biopsy be done in suspected giant cell arteritis?
Within 3 days
How should giant cell arteritis be managed after starting steroids?
Taper steroids gradually
Give PPI and alendronate cover
What should the tapering of steroids be guided by in giant cell arteritis?
Symptoms and ESR
How long should PPI and alendronate be given in giant cell arteritis?
Usually 2 year course
When does polymyalgia rheumatica present?
> 50 years old
How does polymyalgia rheumatica present?
Severe pain and stiffness in shoulders, neck, and hips
Describe the onset of symptoms in polymyalgia rheumatica?
Sudden/subacute onset
Are the symptoms of polymyalgia rheumatica symmetrical?
Yes
When is the pain of polymyalgia rheumatica worse?
In the morning
Is there associated weakness in polymyalgia rheumatica?
No
What other features may be present in polymyalgia rheumatica?
Mild polyarthritis
Tenosynovitis
Carpal tunnel syndrome
Systemic signs
What systemic signs may be present in polymyalgia rheumatica?
Fatigue
Fever
Weight loss
What % of patients with polymyalgia rheumatica develop giant cell arteritis?
15%
What is found on investigation in polymyalgia rheumatica?
Increased ESR and CRP
Increased plasma viscosity
Increased ALP
Normal CK
How is polymyalgia rheumatica managed?
Same as giant cell arteritis
Where is Takayasu’s arteritis found?
Japan - rare outside of Japan
What gender is more commonly affected wiht Takayasu’s arteritis?
Females
What age is Takayasu’s arteritis most common in?
20-40 years
What are the features of Takayasu’s arteritis?
Weak pulses in upper extremities
Visual disturbance
Hypertension
Constitutional symptoms
What are the constitutional symptoms of Takayasu’s arteritis?
Fever
Fatigue
Weight loss
Is polyarteritis nodosa common in UK?
No, is rare
What it the male to female ratio of polyarteritis nodosa?
M:F = 2:1
What age group is most commonly affected with polyarteritis nodosa?
Young adults
What infection is polyarteritis associated with?
Hepatitis B
What are the features of polyarteritis nodosa?
Rash
Hypertension
Melaena and abdominal pain
Constitutional symptoms
How is polyarteritis nodosa managed?
Prednisolone and cyclophosphamide
What is Kawasaki’s disease?
A childhood PAN variant
What are the features of Kawasakis disease?
5-day fever Bilat non-purulent conjunctivitis Oral mucositis Cervical lymphadenopathy Polymorphic rash Extremity changes Coronary artery aneurysms
What extremity changes are seen in Kawasaki’s disease?
Erythema and desquamation
How is Kawasaki’s disease managed?
IVIg and aspirin
What happens in Wegener’s granulomatosis?
Necrotising granulomatous inflammation and small vessel vasculitis, which particularly affects the URT, LRT, and kidneys
What are the URT features of Wegener’s granulomatosis?
Chronic sinusitis
Epistaxis
Saddle-nose deformity
What are the LRT features of Wegener’s granulomatosis?
Cough
Haemoptysis
Pleuritis
What are the renal features of Wegener’s granulomatosis?
Rapidly progressive glomerulonephritis
What are the skin features of Wegener’s granulomatosis?
Palpable purpura
What are the ocular features of Wegener’s granulomatosis?
Conjunctivitis
Keratitis
Uveitis
What investigations are done in Wegener’s granulomatosis?
Urine dipstick
CXR
What are the features of Churg-Strauss?
Late-onset asthma
Eosinophilia
Granulomatous small-vessel vasculitis
What does the granulomatous small-vessel vasculitis lead to in Churg-Strauss?
Rapidly progressive glomerulonephritis
Palpable purpura
GIT bleeding
What autoantibody is found in Wegener’s granulomatosis?
cANCA
What autoantibody is found in Churg-Strauss?
pANCA
What drug might Churg-Strauss be associated with?
Montelukast
What are the features of microscopic polyangiitis?
Rapidly progressive glomerulonephritis
Haemoptysis
Palpable purpura
What autoantibody is found in microscopic polyangiitis?
pANCA
What is produced in Goodpastures?
Anti-glomerular basement membrane antibodies
What are the features of Goodpastures syndrome?
Rapidly progressive glomerulonephritis
Haemoptysis
What is found on CXR in Goodpastures?
Bilateral lower zone infiltrates (haemorrhage)
How is Goodpastures managed?
Immunosuppression and plasmapheresis
What is produced in simple cryoglobulinaemia?
Monoclonal IgM
What is simple cryoglobulinaemia secondary to?
Myeloma
CLL
Waldenstroms
What does simple cryoglobulinaemia cause?
Hyperviscosity
What does the hyperviscosity in simple cryoglobulinaemia lead to?
Visual disturbance Bleeding from mucous membranes Thrombosis Headaches Seizures
What % of cases of cryoglobulinaemia are mixed?
80%
What is produced by mixed cryoglobulinaemia?
Polyclonal IgM
What is mixed cryoglobulinaemia secondary to?
SLE
Sjorgens
Hep C
Mycoplasma
What does mixed cryoglobulinaemia lead to?
Immune complex disease
What does the immune complex disease in mixed cryoglobulinaemia lead to?
Glomerulonephritis
Palpable purpura
Arthralgia
Peripheral neuropathy