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Flashcards in Connective Tissue Diseases Deck (42)
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1

underlying pathology behind systemic sclerosis

excessive collagen deposition causing skin and internal organ changes

2

Condition most associated with systemic sclerosis

Raynauds

3

3 phases of cutaneous involvedment in systemic sclerosis

oedematous, indurative, atrophic --> skin thickened and tight

4

Organs affected in systemic sclerosis

lungs, kidneys, gut

5

Affect of systemic sclerosis on the lungs

pulmonary fibrosis and hypertension

6

Effect of systemic sclerosis on the kidneys

increased hypertension --> renal crisis

7

Effect of systemic sclerosis on the gut

dysphagia, malabsorption, reflux, dysmotility

8

Systemic sclerosis can be split into...

limited and diffuse

9

Limited cutaneous sclerosis can be described by CREST, which stands for...

Calcinosis of the subcutaneous tissues, Raynauds, oEsophageal and gut dysmotility, Sclerodactyl, Telangiectasia

10

Where is skin involvement limited to in limited systemic sclerosis?

face, hands and feet

11

Autoantibody associated with limited systemic sclerosis?

Anti-centromere (70-80%), ANA

12

Diffuse systemic sclerosis is...

rapid and widespread skin changes with early and significant organ involvement

13

Autoantibody associated with diffuse systemic sclerosis?

Anti-scl70 (topoisermerase), ANA

14

Investigations for systemic sclerosis

auto-antibodies, organ screening (lungs, kidneys, heart)

15

Management of Raynauds in systemic sclerosis

Calcium channel blockers, iloprost (PGI2 analogue), bosentan (endothelin R antagonist)

16

Management of renal involvement in systemic sclerosis

ACE-Is (ramipril), ARBs (losartan)

17

Management of GI involvement in systemic sclerosis

PPIs - against reflux

18

Management of ILD in systemic sclerosis

immunosuppression - cyclophosphamide

19

main organs affected by SLE

skin, joints, kidneys, blood cells and nervous system

20

What is the pathogenic mechanism behind SLE?

due to defect in apoptosis, increased cell death but also a delay in clearance of dead cells leads to persistence of antigens i.e. dsDNA and thus immune complex production. antibody-antigen complexes are deposited in the basement membranes of skin and kidneys

21

The majority of SLE is in which group?

Women (11:1), 20-30 years

22

General presentation of SLE (get 5/6)

fatigue, weightloss, fever, arthralgia, myalgia, malar rash, discoid lupus, ulceration, lupus nephritis, pleurisy/pleural effusion, leukopaenia, anaemia, pericarditis, autoimmune hepatitis

23

Immunology for SLE diagnosis

ANA (95%, non-specific), anti-dsDNA (specific and varies with activity), anti-sm (specific but low sensitivity), C3/C4 (low when disease is active)

24

What investigations should be done for SLE (3)?

Bloods (anaemia, leucopaenia, thrombocytopaenia), urinalysis (nephritis), immunology

25

Management of SLE with skin and arthralgia

hydroxychlorquine, topic steroids and NSAIDs

26

Mechanism of action of hydroxychloroquine

blocks APCs

27

Management of SLE with organ involvement

immunosuppression with azathioprine or mycofenolate mofetil

28

Management of SLE with severe organ involvement

IV steroids and cyclophosphamide

29

Monitoring carried out with SLE

anti-dsDNA, BP, cholesterol, urinalysis

30

What is the pathogenic mechanism in sjogrens syndrome?

lymphocitic infiltrates form in exocrine organs