Rheumatology (1) Flashcards
What’s SLE?
- multisystemic autoimmune inflammatory disease
Pathology: auto Abs to a variety of autoantigens → formation and deposition of immune complexes
Demographics of SLE
- Prev: 0.2%
- Sex: F>>M=9:1
- Age: child-bearing age
- Genetic: ↑ in Afro-Caribs and Asians
Symptoms of SLE (with mnemonic)
A RASH POINts an MD
- Arthritis
- Non-erosive, involving peripheral joints
- Jaccoud’s: reducible deforming arthropathy
- Renal
- proteinuria and ↑BP
- ANA (+ve in 95%)
- Serositis
- Pleuritis: pleuritic pain, dyspnoea, effusion
- Pericarditis: chest pain relieved by sitting forwards
- Haematological
- AIHA (autoimmune haemolytic anaemia)
- ↓WCC
- Photosensitivity
- Oral ulcers
- Immune phenomenon
- Anti-dsDNA
- Anti-Sm
- Anti-phospholipid
- Neurological
- Seizures, psychosis
- Malar Rash
- Facial erythema sparing the nasolabial folds
- Discoid Rash
- Erythema → pigmented hyperkeratotic papules →
atrophic depressed lesions
- Mainly affects face and chest
(2) features of symptoms in SLE
- Relapsing, remitting history
- Constitutional symptoms: fatigue, wt. loss, fever, myalgia
Immunology of SLE (Ix)
- 95% ANA+ve
- dsDNA is very specific (sensitivity 60%)
- 30% ENA+ve: Ro, La, Sm, RNP
- Anticardiolipin Abs → false +ve syphilis serology
How to monitor disease activity in SLE?
- Anti-dsDNA titres
- Complement: ↓C3, ↓C4
- ↑ESR
Other Ix
- Bloods: FBC, U+E, CRP, clotting (usually normal)
- Urine: stix, PCR
Drug-Induced Lupus
- (4) causative drugs
- antibody
- symptoms
- prognosis
Causes: procainamide, phenytoin, hydralazine, isoniazid
- Anti-histone Abs in 100%
- Mostly skin and lung signs
- Disease remits if drug stopped
Management of SLE
- severe flares
- cutaneous symptoms
- maintenance (joint and skin symptoms; lupus nephritis)
In specialist SLE and lupus nephritis clinics
Severe Flares: Acute SLE
(AIHA, nephritis, pericarditis or CNS disease)
- High-dose prednisolone + IV cyclophosphamide
Cutaneous Symptoms
- topical steroids and prevent sun cream
Maintenance
- For joint and skin symptoms
NSAIDs and hydroxychloroquine ± low-dose steroids
- Lupus Nephritis
- Proteinuria: ACEi
- Aggressive GN: immunosuppression
Rx Complications
↑ risk of osteoporosis and CV disease
Classification of the anti-phospholipid syndrome (2)
- Primary: 70%
- Secondary to SLE: 30%
Antibodies in anti-phospholipid syndrome (2)
- anti-cardiolipin
- lupus anticoagulant
Features of anti-phospholipid syndrome (mnemonic)
CLOTs: venous (e.g. DVT) and arterial (e.g. stroke)
- Coagulation defect: ↑APTT
- Livido reticularis
- Obstetric complications: recurrent 1st-trimester abortion
- Thrombocyotpenia
Management of anti-phospholipid syndrome
- Low-dose aspirin
- Warfarin if recurrent thromboses: INR 3.5
Epidemiology of Systemic Sclerosis
- F>M=3:1
- 30-50yrs
- Limited Systemic Sclerosis: 70%
- Diffuse Systemic Sclerosis: 30%
Features of Limited Systemic Sclerosis (mnemonic)
Limited Systemic Sclerosis
( CREST syndrome)
- Calcinosis
- Raynaud’s
- Esophageal and gut dysmotility → GORD
- Sclerodactyly
- Telangiectasia
- Skin involvement limited to face, hands and feet
- Beak nose
- Microstomia (small mouth)
- Pulmonary HTN in 15%
Symptoms of Diffuse Systemic Sclerosis
Diffuse Systemic Sclerosis
- Diffuse skin involvement
- Organ fibrosis
- GI: GORD, aspiration, dysphagia, anal incontinence
- Lung: fibrosis and PHT
- Cardiac: arrhythmias and conduction defects
- Renal: acute hypertensive crisis (commonest
cause of death)
