Connective Tissue Disorders Flashcards
What is antiphospholipid syndrome (APS)?
Autoimmune disease
Causes increased risk of blood clotting
What are lab findings of people with APS?
+ve anti-cardiolipin antibodies
Lupus anticoagulant activity
Anti-beta2 glycoprotein
What are haematological findings of people with APS?
Arterial/venous thrombosis
What are reproductive signs of people with APS?
Spontaneous loss 10-34/40
OR
3 spontaneous losses
Are anti-cardiolipin antibodies only found in people with APS?
No
found in 1-5% of healthy people
Give 5 other features of APS
Livedo reticularis Thrombocytopenia Migraine Liebman-Sachs endocarditis Catastrophic APS
How is APS treated?
Life long anticoagulation
- warfarin
How is APS treated in pregnant women?
Aspirin + LMW heparin
- warfarin is teratogenic
What is Sjogren’s syndrome?
Autoimmune condition
Lymphocyte infiltration of exocrine glands
Causes sicca disorders (e.g. xeromastia, keratoconjunctivitis)
Name the objective test for ocular dryness. Describe.
Schirmer test
Filter paper placed beneath water line. Positive result if
Which antibodies come up positive in people with Sjogrens?
Anti Ro and La
SSA and SSB
What does a biopsy check for in Sjogrens?
Lymphocytic infiltrate of exocrine glands
Name 4 other manifestations of Sjogrens
Arthralgia
Fatigue
Skin and vaginal dryness
Parotid gland swelling
What is the peak age of develepment of primary Sjogrens syndrome?
40-60
What is the gender distribution of the prevalence of Sjogrens?
M:F
1:9
How is Sjogrens’ syndrome treated?
Eye drops
Saliva replacement /
Pilocarpine (saliva stimulant)
Hydroxychloroquine (joint pain and fatigue)
Steroids only for serious complications, which are v rare
What is Systemic Sclerosis?
Autoimmune condition
Fibroblast overactivity leads to overgrowth of connective tissue -> fibrosis + vasculopathy
Give 2 examples of Localised Scleroderma
Morphoea
Linear Scleroderma
Give 3 examples of
Diffuse Scleroderma
Limited Systemic sclerosis
Diffuse systemic sclerosis
Systemic sclerosis sine scleroderma
List the 6 key features of Limited Systemic sclerosis
CREST + P
Calcinosis Raynaud's Eosophageal dysmotility Sclerodactyly Telangectasia Pulmonary hypertension
Which antibody is found in people with Limited Systemic sclerosis
Anti-centromere antibodies
Which areas of the body are commonly affected by Limited systemic sclerosis?
Face
Hands
Forearms
Feet
What are the 4 key features of Diffuse systemic sclerosis?
Skin changes within a year of Raynauds
Trunk and acral skin involvment
Early significant organ involvement
Which antibody is found in people with Diffuse systemic sclerosis?
Anti-Scl-70 antibodies
What is the peak age of development of systemic sclerosis?
25-55
How are the sexes affected by systemic sclerosis?
M:F
1:4
How is Raynauds (associated with systemic sclerosis) treated?
Calcium channel blockers
iloprost
Bosentan
How is renal impairment (associated with systemic sclerosis) treated?
ACE inhibitors
How are GI symptoms (associated with systemic sclerosis) treated?
PPIs (against reflux)
How is interstitial lung disease (associated with systemic sclerosis) treated?
Immunosuppression
- cyclophosphamide
What is Mixed Connective Tissue Disease (MCTD)?
A condition whose features / symptoms are also seen in other connective tissue diseases
List the major criteria for diagnosing MCTD
Myositis Pulmonary involvement Raynaud's phenomenon Sclerodactyly Swollen hands
Which antibody is associated with the presence of MCTD?
Anti-RNP
What is polyarteritis nodosa (PAN)?
Unknown aetiology
Inflammation and fibrinoid necrosis of small/med arteries
Which major organs are tageted by PAN?
Kidneys
Heart
Liver
GIT
What are the clinical features of PAN?
Non-specific organ features
- hypertension
- haematuria
- abdominal pain
- melaena
- diarrhoea
How is PAN diagnosed?
Biopsy
Serum: pANCA +ve
