Rheumatology - Core Topics Flashcards
What is giant cell arteritis (GCA)?
Systemic and chronic vasculitis of the medium and large arteries, typically presenting with symptoms affecting the temporal arteries (ie. temporal arteritis).
What is the key complication of giant cell arteritis?
Vision loss - occlusive arteritis can result in anterior ischaemic optic neuropathy.
Risk factors for giant cell arteritis.
- increasing age
- comorbid polymyalgia rheumatica
- HLA-DR4 genetic predisposition
Presentation of giant cell arteritis.
- unilateral headache around temple / forehead
- scalp tenderness
- jaw claudication
- blurred or double vision
- sight loss
May be systemic symptoms, for example fever, myalgia and fatigue.
How is giant cell arteritis diagnosed?
- clinical presentation
- raised ESR
- temporal artery biopsy findings
Full blood count may show a normocytic anaemia and thrombocytosis (raised platelets).
Liver function tests can show a raised alkaline phosphatase
C reactive protein is usually raised
Duplex ultrasound of the temporal artery shows the hypoechoic halo sign
What histological finding in typical of temporal artery biopsy in giant cell arteritis?
Multinucleated giant cells
How is giant cell arteritis managed?
Immediate commencement of prednisolone (60mg OD) before confirmation of diagnosis to reduce the risk of permanent sight loss.
Once the diagnosis is confirmed they will need to continue high dose steroids (40-60mg) until the symptoms have resolved. They then need to slowly wean off the steroids. This can take several years.
Other medications:
Aspirin 75mg daily decreases visual loss and strokes
Proton pump inhibitor (e.g. omeprazole) for gastric prevention while on steroids
Referrals:
Vascular surgeons for a temporal artery biopsy in all patients with suspected GCA
Rheumatology for specialist diagnosis and management
Ophthalmology review as an emergency same day appointment if they develop visual symptoms
What is polymyalgia rheumatica?
An inflammatory condition that causes pain and stiffness in the shoulders, pelvic girdle and neck.
There is a strong association with giant cell arteritis.
Who is most commonly affected by polymyalgia rheumatica?
- older adults (>50yrs)
- women
- more common in caucasians
Presentation of polymyalgia rheumatica.
- bilateral shoulder pain
- bilateral pelvic girdle pain
- worse with movement
- interferes with sleep
- stiffness for >45minutes in the morning
- carpel tunnel syndrome
What examination findings would be supportive of polymyalgia rheumatica?
- decreased ROM of shoulders, neck and hips
- normal muscle strength
- muscle tenderness
What are some differentials for polymyalgia rheumatica?
- osteoarthritis
- rheumatoid arthritis
- systemic lupus erythematous (SLE)
- cervical spondylosis
How is polymylagia rheumatica diagnosed?
Typically history and exam, supported by raised inflammatory markers:
ESR, CRP and plasma viscosity raised. Note normal inflammatory markers does NOT exclude PMR.
Additional investigations to consider:
Anti-nuclear antibodies (ANA) for systemic lupus erythematosus
Anti-cyclic citrullinated peptide (anti-CCP) for rheumatoid arthritis
Urine Bence Jones protein for myeloma
Chest xray for lung and mediastinal abnormalities
How is polymyalgia rheumatica managed?
15mg OD prednisolone usually effective, sometimes for up to 18 months, followed by a gradual reducing regime.
Methotrexate can be steroid-sparing in relapsing PMR.
What is systemic lupus erythematous (SLE)?
An inflammatory autoimmune connective tissue disease, affecting multiple organs and systems due to inadequate T cell suppressor activity, with increased B cell activity.
What is the leading cause of premature death in SLE?
Cardiovascular disease and infection.
Pathophysiology of SLE.
Anti-nuclear antibodies causes the immune system to target proteins within cell nuclei, generating an inflammatory response.
This inflammation is inappropriate and chronic, causing problems for many tissues within the body.
Presentation of SLE.
SOAP BRAIN
Serositis (pleurisy, pericarditis)
Oral ulcers
Arthritis
Photosensitive malar rash
Blood disorders (low WCC, haemolytic anaemia)
Renal involvement (glomerulonephritis)
Autoantibodies (ANA positive)
Immunological tests (low complements)
Neurologic disorder (seizures, psychosis)
How is SLE investigated?
- autoantibodies (ANA, anti-dsDNA)
- FBC (anaemia of chronic disease)
- C3 and C4 decreased
- CRP and ESR raised
- immunoglobulins raised
- urinalysis for proteinuria
- renal biopsy
Which autoantibodies are associated with SLE?
- anti-nuclear antibodies (ANA)
- anti-dsDNA antibodies
- anti-Ro
- Anti-La
What are some possible complications of SLE?
- CVD (leading cause of death_
- infection (secondary to immunosuppressants)
- anaemia of chronic disease
- pericarditis
- interstitial lung disease
- recurrent miscarriage
VTE is particularly associated with which syndrome occurring secondary to SLE?
Anti-phospholipid antibodies
How is SLE managed?
- sun protection
- CVD risk reduction
- NSAIDs and steroids (prednisolone) for flares
- hydroxychloroquine is first line for mild SLE
More aggressive immunosuppressants (e.g. methotrexate) and biological therapies (e.g. rituximab) are used in more severe SLE.
What is Raynaud’s phenomenon?
A condition caused by vasospasm of the digits, causing painful colour changes in response to a cold stimulus.
White - inadequate blood flow
Blue - venous stasis
Red - re-warming hyperaemia
What are some causes of Raynaud’s phenomenon?
- SLE
- cervical rib
- beta blockers