Sero-positive inflammatory arthritis (excluding RA) Flashcards
Define sero-positive inflammatory arthritis
Give three examples
Inflammatory arthropathies that test positive for rheumatoid factor
Includes:
- Rheumatoid arthritis
- Systemic lupus erythematosus (SLE)
- Sjorgren’s syndrome
- Bacterial and viral infections eg. septic arthritis
What is Systemic lupus erythematosus (SLE)?
A multi-systemic autoimmune disease featuring autoantibodies against a variety of autoantigens (ANA).
Insufficient phagocytosis of these ANAs causes secretion of pathogenic autoantibodies, resulting in a tissue damage.
Name three risk factors for SLE
- Female (9:1) - especially pre-menopausal
- Aged 20-40
- Afro-Caribbean and Asian ethnicity
- HLA B8, DR2, DR3 associated
- FHx in 1o relatives
- Epstein-Barr virus
Drug-induced lupus
UV light - can trigger flares
Name three causes of drug-induced lupus
How does drug-induced lupus differ from typical SLE?
- Hydralazine: HTN vasodilator
- Isoniazid
- Procainamide: Class Ia antiarrhythmic
- Penicillamine: Wilson’s disease treatment
- Anti-TNF
Drug-induced lupus is usually mild as it does not affect the kidneys or CNS. 95% positive for anti-histone antibodies
Give five presenting features of systemic lupus erythematosus
High variable presentation between patients.
- Non-specific symptoms of fatigue, malaise, myalgia, fever
- Symmetrical small joint arthralgia
- Photosensitive malar/butterfly rash; Discoid rash; alopecia
- Recurrent pleurisy and pleural effusions, ILD
- Pericarditis; Libman-Sacks endocarditis
- Lupus nephritis; end-stage renal disease
- Depression, psychosis, epilepsy, migraines, cerebellar ataxia, aseptic meningitis, stroke
- Sjogren’s (15%), retinal vasculitis
- Mouth ulcers
How is systemic lupus erythematosus diagnosed?
4 of 11 of the following: SOAP BRAIN MD
- Serositis: Pleurisy or pericarditis
- Oral ulcers
- Arthritis: Non-erosive, 2+ peripheral joints
- Photosensitivity
- Blood disorders: Leukopenia, lymphopenia, thrombocytopenia
- Renal (lupus nephritis): Proteinuria (>0.5g/d) or cellular casts
- ANAs (95%)
- Immune: Anti-dsDNA (70%), anti-Sm, or anti-pl antibodies
- Neurological: Seizures or psychosis
- Malar rash
- Discoid rash
What markers are used to monitor progression of SLE?
- Raised Anti-dsDNA
- Reduced C3 and C4
- Raised ESR
Request three investigations for suspected SLE
- FBC: Leukopenia, lymphopenia, thrombocytopenia
- Normal CRP and raised ESR
- U+Es; Urinalysis: Proteinuria and cellular casts
- ANA; Anti-dsDNA; Anti-Sm (most specific)
- Anti-phospholipid: associated with antiphospholipid syndrome
Outline the management of SLE
- Avoid excessive exposure to sunlight
- Reduce increased cardiovascular risk
-
Many respond well to NSAIDs, and do not need steroids or immunosuppresive agents
- Maintenance: NSAIDs, hydroxychloroquine (retinal tests), azathioprine
- Cutaneous lupus: Topical steroids
- Severe flares: Urgent IV cyclophosphamide + high-dose prednisolone/single dose IM long-acting steroid
- Lupus nephritis: ACEi
- If glomerulonephritis on Bx, high-dose prednisolone + cyclophosphamide/mycophenolate
What is anti-phospholipid syndrome?
- Autoimmune coagulation disorder
- Thrombosis and/or recurrent miscarriages
- Persistently positive tests for anti-phospholipid antibodies
Describe the clinical features of anti-phospholipid syndrome
- Coagulation defect eg. DVT; stroke
- Livedo reticularis: reticular cyanotic discolouration
- Recurrent miscarriage
- Thrombocytopenia: increased APTT
How is anti-phospholipid syndrome diagnosed?
Both:
- Hx of thrombosis and/or recurrent miscarriage
- One positive blood test:
- Anticardiolipin test
- Lupus anticoagulant test
- Anti B2-glycoprotein I test
Describe the management of anti-phospholipid syndrome
Treat if patient has had at least one thrombosis
- Low-dose aspirin or warfarin (Target INR 2-3)
- If pregnant, give aspirin and LMWH in early gestation
- Does not prevent pre-eclampsia or poor foetal growth
Define Sjogren’s syndrome
- A chronic inflammatory autoimmune disorder
- Lymphocytic infiltration and fibrosis of exocrine glands
- esp. lacrimal and salivary glands
What is primary Sjogren’s syndrome?
Syndrome of dry eyes
In the absence of RA or any autoimmune disease
Anti-Ro (90% in primary, 10% in secondary)