Vasculitis Flashcards
Define polymyalgia rheumatica (PMR)
A syndrome of sudden (<2wk) onset severe pain and stiffness in a limb girdle pattern (shoulders, neck, hips, and lumbar spine). N.B. no weakness Symptoms are worse in the morning, with duration >30 minutes. Always occurs in over 50yrs.
Name 2 differential diagnoses of Polymyalgia rheumatica (PMR)
Polymyositis: proximal muscle ache and weakness Myopathy: weakness, but no pain or stiffness PMR: proximal morning pain and stiffness, no weakness
Describe the investigations of Polymyalgia rheumatica
Raised ESR and/or CRP* are hallmark Serum ALP and GGT may be raised as markers of acute phase response Anaemia is often present
What is the management of Polymyalgia rheumatica?
Prednisolone 10-15mg/d PO + PPI Improvement should be seen within 2 weeks* R/V at 8 weeks
Define Giant cell arteritis
- Inflammatory granulomatous arteritis of temporal arteries
- Associated with polymyalgia rheumatica (50%).
- Typically seen in women over 50
Describe the clinical features of Giant cell arteritis
- Jaw and/or tongue claudication
- Severe headaches
- Scalp/temple tenderness e.g. when combing hair
- Tender swollen temporal or occipital arteries
- Changes in vision: curtain drop (amaurosis fugax)
What will investigations of Giant cell arteritis show?
- Raised ESR
- Very high CRP
- Temporal artery biopsy (within 1 week)
- Granulomatous infiltration
- Giant cells
Why must a temporal artery biopsy be >1cm when investigating Giant cell arteritis?
Giant cell arteritis can occur in a discontinuous pattern, so a long biopsy is needed to ensure it is not missed.
Why is Giant cell arteritis a medical emergency?
It may cause sudden, painless vision loss if the opthalmic artery is involved.
Start treatment immediately if suspected
Describe the diagnostic criteria for giant cell arteritis
3 of 5 of the following:
- Granulomatous inflammation
- Increased CRP and ESR
- Aged over 50
- New headache
- Temporal pain
Outline the management of Giant cell arteritis
- Prednisolone
- Uncomplicated (no claudication): 40mg
- Complicated: 60mg
- PPIs
- Bisphosphonates
Wean steroids when symptoms resolve and ESR falls
Define Takayasu’s arteritis
A rare large vessel arteritis more commonly seen in Japan. It involves the aortic arch and other major arteries.
What are the characteristic features of Takayasu’s arteritis?
Absence of peripheral pulses HTN
What is the treatment of Takayasu’s arteritis?
Corticosteroids for symptoms Surgical bypass of affected areas
Define Classical polyarteritis nodusa (PAN)
A rare medium vessel vasculitis that typically occurs in middle-aged men. Accompanied by severe systemic maifestations.
Describe the clinical features of Classical polyarteritis nodusa
Initial: Fever, malaise, weight loss, myalgia Acute organ infarction: -Neuro: Mononeuritis multiplex -GI: Pain mimicking acute cholecystitis (continuous RUQ), pancreatitis (epigastric/central radiating to back), or appendicitis (Central to RIF). GI haemorrhage -Renal: Haematuria and proteinuria -Cardiac: MI and heart failure -Skin: SC haemorrhage and gangrene. Livedo reticularis
What is the treatment of Classical polyartheritis nodusa?
Corticosteroids + immunsuppressive drugs e.g. Azathioprine
Define Kawasaki’s disease
An acute systemic medium vessel vasculitis supposedly triggered by infection, that mainly affects children under 5yr. Commonly seen in Japan.
Describe the diagnostic criteria of Kawasaki’s disease
Persistent fever (>5 days) plus 4 of the following: Bilateral conjunctival congestion after 2-4days Dry red lips and oral cavity Acute cervical lymphadenopathy Polymorphic rash Red oedematous palms and soles
Outline the management of Kawaski’s disease
Single high-dose IV immunoglobin to prevent CAD, followed by Aspirin 200-300mg daily
Describe the clinical features of small-vessel vasculitides (ANCA positive)
Small-vessel vasculitides (ANCA positive) include GPA. eosinophilic GPA, and microscopic polyangiitis. They show either a c-ANCA (GPA) or p-ANCA (eGPA or microscopic polyangiitis) pattern. Respiratory tract and kidneys are frequently involved. ESR is often markedly elevated (>100)
Describe the investigations of Granulomatosis with polyangiitis
c-ANCA pattern and granulomas on biopsy CXR: Multiple cavitating nodules, consolidation, ground glass opacification
Who typically gets Granulomatosis with polyangiitis?
Elderly Caucasian
Outline the presentation of Granulomatosis with polyangiitis
URT: Bloody nasal discharge with crusting Saddle-nose deformity Sinusitis Otitis media Diffuse alveolar haemorrhage: Cough Dyspnoea Pleurisy Glomerulonephritis
What is the treatment for Granulomatosis with polyangiitis?
Corticosteroids and cyclophosphamide Rituximab Methotrexate
How does microscopic polyangiitis differ from GPA?
GPA is associated with c-ANCA, features granulomas on biopsy. Microsopic is associated with p-ANCA, no granulomas on biopsy.
Describe the presentation of eosinophilic GPA
Presents in early adulthood with allergic rhinitis, difficult to control asthma, and peripheral blood eosinophilia. Later systemic involvement: Skin: Tender SC nodules, petechiae, purpura Neuro: Mononeuritis multiplex Heart Kidneys GI
How is eosinophilic GPA investigated?
CXR: migratory patchy opacification, nodules, effusion Biopsy: Eosinophilic infiltration, small-vessel vasculitis p-ANCA pattern
What is the treatment of eosinophilic GPA?
Corticosteroids Immunosuppressive agents if severe
Define Goodpasture syndrome
Autoimmune disease that damages the basement membranes in the lungs and kidneys.
Describe the presentation of Goodpasture syndrome
Characteristic triad of: Pulmonary haemorrhage Glomerulonephritis Presence of antibodies against basement membrane antigens
How is Goodpasture syndrome treated?
Plasmapheresis to remove circulating antibodies Prednisolone + cyclophosphamide to prevent further antibody production
