Connective Tissue Disease Flashcards
What are connective tissue diseases?
diseases with spontaneous over activity of the immune system
Which condition is often known as the great imitator?
SLE
Who does SLE affect more, M or F?
F
What causes SLE?
- Immune complexes deposited in wall of blood vessel
- presence of immune complexes activates complement and attracts inflammatory cells eg neutrophils
- enzymes released from neutrophils cause damage to endothelial cells of basement membrane
What cutaneous features occur in SLE?
photosensitivity
malar rash
discoid lupus erythematosus
What Jaccoud’s arthritis?
deforming arthropathy present in SLE
What endocarditis may be present in SLE?
Libman Sach
What haematological features are there of SLE?
lymphadenopathy
leucopenia
anaemia
thrombocytopenia
What tests should be done for suspected SLE?
FBC ANA dsDNA ENA complement levels
In how many SLE patients is ANA positive?
95%
When should an ANA test be taken seriously?
if other antinuclear antibodies are positive
How often is anti double stranded DNA antibody positive for patients with SLE?
60%
Which antibody is highly specific for SLE?
anti double stranded DNA antibody
What else may anti double stranded DNA antibody be associated with?
lupus nephritis
Drug treatment for moderate SLE?
Moderate disease: oral steroids, azatrioprine, methotrexate
Drug treatment for mild SLE?
NSAIDs + hydroxychloroquine for maintenance
Topiccal steroids for skin
Drug treatment for severe/acute SLE?
Acute flare up: IV Cyclophosphamide + High dose prednisolone
Rituximab
What is arterial/venous thrombosis, pregnancy loss associated with?
Anti-phospholipid syndrome
What is responsible for 15% of cases of recurrent foetal loss and 20% of recurrent thrombosis in young people?
anti phospholipid syndome
Treatment of Anti-phospholipid syndrome?
thrombosis (life long anticoagulation)
pregnancy loss - aspirin and heparin during pregnancy
attention to vascular risk factors
What does Sjogren’s syndrome involve?
lymph infiltration of exocrine glands, causing xerostomia and keratoconjunctivitis sicca
What are some manifestations of Sjogren’s?
fatigue, arthralgia, dry mouth/skin/vagina, neonatal complete heart block, Raynauds, salivary swelling
Treatment for Sjogren’s?
eye drops, punctual plugs saliva replacement pilocarpine hydroxychloroquine Steroids and immunosupression
What is systemic sclerosis?
vasculopathy (raynauds)
fibrosis, can be localized or systemic
What does the acronym CREST stad for in systemic sclerosis?
calcinosis, Raynauds, esophageal dysmotility, sclerodactylyl, telangiectasia
Treatments for sclerosis?
calcium channel blockers Prostacyclin ACE Is Prednisolone Immunosuppression Bosenta, Sildenafil
What is MCTD?
mixed connective tissue disease
What antibody is used for MCTD?
anti-RNP
