Connective Tissue Disease Flashcards
what is Sjogrens syndrome
autoimmune condition characterised by lymphocytic infiltration of exocrine glands
what are the symptoms of Sjogrens syndrome
sicca symptoms - dry eyes + mouth arthralgia fatigue vaginal dryness parotid gland swelling
what conditions can Sjogrens be secondary too
RA SLE
Complications of sjogrens
interstitial lung disease peripheral neuropathy increased lymphoma risk
what is diagnosis of Sjogrens based on (3 things)
- ocular dryness - schrimer’s test 2. positive anti-ro + anti-la 3. typical salivary gland biopsy findings
what medication can stimulate saliva production in Sjogrens? what is the main side effect?
pilocarpine - flushing main side effect
treatment of arthralgia + fatigue in Sjogrens syndrome
hydroxychlorquine
what is systemic sclerosis
multi-system autoimmune disease characterised by microvascular inflammatory damage and fibrosis of skin + internal organs
what are the two classifications of systemic sclerosis
limited diffuse
differences between limited and diffuse systemic sclerosis
limited - skin fibrosis confined to hands, forearms, face, feet. Late organ involvement diffuse - rapid skin changes, involvement of trunk. Early organ involvement
antibody associated with limited systemic sclerosis
anti-centromere
antibody associated with diffuse systemic sclerosis
Anti-Scl 70
skin features of systemic sclerosis
Calcinosis
Reynauds (often first sign)
Esophageal dysmotility - reflux, dysphagia, strictures
Sclerodactyly
Telangectasia
organ involvement in systemic sclerosis
pulmonary hypertension, pulmonary fibrosis
renal AKI
right sided heart failure du to pulmonary HTN
GI malabsorption
Treatment of scleroderma in systemic sclerosis
immunosuppression
- cyclophosphamide
- methotrexate
- mycophenolate
treatment of reynauds
calcium channel blockers (amlodopine) first line
PDE-5 inhibitors (sildenafil) second line
treatment of oesophageal dysmotility in systemic sclerosis
PPI
Prokinetic agent e.g. metoclopramide
treatment of renal involvement in systemic sclerosis
ACEi
treatment of pulmonary HTN in systemic sclerosis
PDE-5 inhibitor (sildenafil)
endothelin receptor antagonist (bosentan)
what antibody is associated with mixed connective tissue disease
Anti-RNP
Presentation of anti-pospholipid syndrome
recurrent venous / aterial thrombosis
recurrent fetal loss
Anti-phospholipid syndrome increases risk of what?
stroke / MI
What type of endocarditis is associated with anti-pospholipid syndrome
Libman- Stacks : endocarditis not caused by bacteria
common cutaneous feature of anti-pospholipid syndrome
livedo reticularis
antibodies associated with anti-pospholipid syndrome
lupus anti-coagulant
anti-cardiolipin
treatment of anti-pospholipid syndrome
wafarin
low-molecular weight heparin (e.g. dalteparin) in pregnancy
what is Ehlers-Danlos syndrome
an inherited connective tissue disease characterised by defective collagen synthesis
- autosomal dominant
features of ehlers-danlos
joint hypermobility
hyperextensible skin
tissue fragility - mucosal bleeing/organ rupture
what is marfans syndrome
autosomal dominant inhertited connective tissue disease due to defective fibrillin 1
features of marfans syndrome
tall, thin stature
long fingers
hypermonbile joints
arthralgia
pes planus
high arched palate
heart murmur in ehlers danlos
aortic regurgitation
Cardiac problems in marfans syndrome
dilatation of aortic sinuses - aortic aneursym, aortic regurgitation, aortic dissection
mitral valve prolapse
