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Year 4: Rheumatology > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

what is Sjogrens syndrome

A

autoimmune condition characterised by lymphocytic infiltration of exocrine glands

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2
Q

what are the symptoms of Sjogrens syndrome

A

sicca symptoms - dry eyes + mouth arthralgia fatigue vaginal dryness parotid gland swelling

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3
Q

what conditions can Sjogrens be secondary too

A

RA SLE

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4
Q

Complications of sjogrens

A

interstitial lung disease peripheral neuropathy increased lymphoma risk

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5
Q

what is diagnosis of Sjogrens based on (3 things)

A
  1. ocular dryness - schrimer’s test 2. positive anti-ro + anti-la 3. typical salivary gland biopsy findings
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6
Q

what medication can stimulate saliva production in Sjogrens? what is the main side effect?

A

pilocarpine - flushing main side effect

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7
Q

treatment of arthralgia + fatigue in Sjogrens syndrome

A

hydroxychlorquine

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8
Q

what is systemic sclerosis

A

multi-system autoimmune disease characterised by microvascular inflammatory damage and fibrosis of skin + internal organs

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9
Q

what are the two classifications of systemic sclerosis

A

limited diffuse

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10
Q

differences between limited and diffuse systemic sclerosis

A

limited - skin fibrosis confined to hands, forearms, face, feet. Late organ involvement diffuse - rapid skin changes, involvement of trunk. Early organ involvement

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11
Q

antibody associated with limited systemic sclerosis

A

anti-centromere

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12
Q

antibody associated with diffuse systemic sclerosis

A

Anti-Scl 70

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13
Q

skin features of systemic sclerosis

A

Calcinosis

Reynauds (often first sign)

Esophageal dysmotility - reflux, dysphagia, strictures

Sclerodactyly

Telangectasia

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14
Q

organ involvement in systemic sclerosis

A

pulmonary hypertension, pulmonary fibrosis

renal AKI

right sided heart failure du to pulmonary HTN

GI malabsorption

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15
Q

Treatment of scleroderma in systemic sclerosis

A

immunosuppression

  • cyclophosphamide
  • methotrexate
  • mycophenolate
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16
Q

treatment of reynauds

A

calcium channel blockers (amlodopine) first line

PDE-5 inhibitors (sildenafil) second line

17
Q

treatment of oesophageal dysmotility in systemic sclerosis

A

PPI

Prokinetic agent e.g. metoclopramide

18
Q

treatment of renal involvement in systemic sclerosis

A

ACEi

19
Q

treatment of pulmonary HTN in systemic sclerosis

A

PDE-5 inhibitor (sildenafil)

endothelin receptor antagonist (bosentan)

20
Q

what antibody is associated with mixed connective tissue disease

A

Anti-RNP

21
Q

Presentation of anti-pospholipid syndrome

A

recurrent venous / aterial thrombosis

recurrent fetal loss

22
Q

Anti-phospholipid syndrome increases risk of what?

A

stroke / MI

23
Q

What type of endocarditis is associated with anti-pospholipid syndrome

A

Libman- Stacks : endocarditis not caused by bacteria

24
Q

common cutaneous feature of anti-pospholipid syndrome

A

livedo reticularis

25
Q

antibodies associated with anti-pospholipid syndrome

A

lupus anti-coagulant

anti-cardiolipin

26
Q

treatment of anti-pospholipid syndrome

A

wafarin

low-molecular weight heparin (e.g. dalteparin) in pregnancy

27
Q

what is Ehlers-Danlos syndrome

A

an inherited connective tissue disease characterised by defective collagen synthesis

  • autosomal dominant
28
Q

features of ehlers-danlos

A

joint hypermobility

hyperextensible skin

tissue fragility - mucosal bleeing/organ rupture

29
Q

what is marfans syndrome

A

autosomal dominant inhertited connective tissue disease due to defective fibrillin 1

30
Q

features of marfans syndrome

A

tall, thin stature

long fingers

hypermonbile joints

arthralgia

pes planus

high arched palate

31
Q

heart murmur in ehlers danlos

A

aortic regurgitation

32
Q

Cardiac problems in marfans syndrome

A

dilatation of aortic sinuses - aortic aneursym, aortic regurgitation, aortic dissection

mitral valve prolapse

Year 4: Rheumatology (11 decks)

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