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Flashcards in Connective Tissue Disease Deck (50):
1

What are connective tissue diseases characterised by?

presence of spontaneous over-activity of the immune systerm

2

Who does SLE affect?

M:F 1:9; higher in asians, afro-americans, afro-caribbeans, hispanic americans

3

How do hormones affect SLE incidence?

increased in those with higher oestrogen exposure- contraceptives and HRT

4

What environmental factors can increase SLE?

virsues; UV light; silica dust

5

What is the pathogenesis of SLE?

loss of immune regulation; defective apoptosis; necrotic cells release nuclear material which act as potential auto-antigens and autoimmunity results from extended exposure to nuclear and intra-cellular antigens

6

What causes renal disease in SLE?

deposition of immune complexes in mesngium; activate complement which attracts leucocytes which release cytokines; perpetuates inflam which causes necrosis and scarring over time

7

What are the general symptoms seen with SLE?

fever; malaise; poor appetite; wt loss; fatiuge

8

What are the mucocuntanoues features?

photosensitivty; malar rash (spares the naso-labial folds); discoid lupus erythematosus (may scar); subacute cutaneous lupus; painless mouth ulcers; alopecia

9

What are the MSK features of SLE?

non-deforming polyarthritis/polyathralgia; myopathy- weakness, myalgia and myosistis

10

What are the serositis features of SLE?

pericarditis; pleurisy; pleural effusion; pericardial effusion

11

What are the features of renal disease clinically?

proteinuria of >500mg in 24 hours; red cell casts

12

What test should always be done in pts with SLE?

urinalysis

13

What are the neurological features seen in SLE?

depression/psychosis; migraneous headache; seizures; cranial or peripheral neuropathy; mononeuritis mulitplex

14

What are the haemotological features of SLE?

lymphadenopathy; leucopenia; lymphopenia; haemolytic anaemia; thrombocytopenia

15

What is anti-phospholipid syndrome?

venous and arterial thrombosis

16

What are the features of anti-phospholiid syndrome?

recurrent miscarriage; DVT; young stroke; livido reticularis; assoc. with other autoimmune condiitons esp. SLE; thrombocytopenia; prolnged platelet time

17

What are the intrinsic factors that increase an SLE pts susceptibilty to inection?

low complement; defective phagocytosis; impaired cell mediated immunity

18

What are the extrinsic factors that contribute to an SLE pts susceptibilty to infection?

steroids and other immunosuppressive drugs

19

What are the benefits and bad of ANA?

positive in 95% of pts, found in lots of other conditions

20

What other antinuclear antibodies can be positive in SLE?

anti-dsDNA
anti-Sm
anti-Ro
anti-RNP

21

What are the benefits of anti-dsDNA?

occurs in about 60% of SLE and is highly specific for SLE

22

What is Anti-Ro usually associated with?

anti-La

23

What is anti-Ro often associated with?

congential heart block, neonatal LE, cutaenous manifestation

24

What is the most specific autoantibody for SLE?

anti-Sm

25

What are the antibodies present in antiphospholipid syndrom?

anti-cardiolipin
lupus anticoagulant
anti-beta 2 glycoprotein

26

What antibody level postivitely correlates with activity?

anti-dsDNA

27

What negatively correlate with activity?

C3/C4

28

What are the drug treatmnents for SLE?

NSAIDs and simple analgesia
anti-malarials- hydroxychlorquine
steroids
immunosuppressives eg azathioprine
biologics eg rituximab

29

What drugs are used in mild SLE?

HCQ; topical steroids; NSAIDS

30

What drugs are used in moderate disease?

oral steroids; azathioprine; methotrexate

31

What drugs are used in severe SLE?

IV steroids; cyclophosphamide; tituximab

32

What is gender distribution in anti-phospholipid syndrome?

M:F 1:3.5

33

What neurological and cardiac symptoms are seen with anti-phospholpi syndrome?

Libman-Sacks endocarditis; migrane, transverse

34

What happens in Sjogren's syndrome?

lymphocyte infiltration of exocrine glands causing xerostomia and keratoconjunctivitis

35

What immunology might be seen with Sjogrens?

anti-Ro; anti-La

36

What test can be done to determine mouth and eye dryness?

schirmer test- paper on eye and in mouth

37

What are the more serious manifestations of Sjogrens?

ILD; neuropathy; lymphoma x40 risk; renal tubular acidosis; neonatal complete heart block (anti-Ro)

38

Who gets Sjogrens?

40-60yo; M:F 1:9

39

What are the treatment options for Sjogrens?

eye drops; saliva replacement; HCQ; steroid and immunosuppression; CVS risk factors

40

What is systemic sclerosis caused by?

vasculopathy of small vessels; inflammation and then fibrosis- excess deposition of collagen in skin and internal organs

41

What are the symptoms of systemic sclerosis?

Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactlyl
Telangiectasia
AND pulmonary HT

42

What antibodies are systemic sclerosis associated with?

anti-centromere
anti-Scl-70

43

What is diffuse systemic sclerosis?

skin changes within 1 year of Raynauds; truncal and acral skin involvemtn; early signif organ involvemtne

44

What are the GI organ manifestations of systemic sclerosis?

small bowel dysmotility
bacterial overgrowth
rectal dysmotility
pancreatic insufficiency
dysphagia

45

What are the respiratory manifestations of systemic sclerosis?

ILD; pulmonary HT; chest wall restriction

46

What are the CVS manifestations of systemic sclerosis?

Raynauds; atherosclerosis; hypertensive cardiomyopathy

47

Who is systemic sclerosis seen in?

25-55yo
M:F 1:4

48

What are the treatments for systemic sclerosis?

CCBs; prostacyclin analogue- Raynauds; ACEI; steroids; immunosupprestion

49

What is undifferentiated connective tissue disorder?

connecitve tissueish but dont fit a partiuclar syndrome (yet)

50

What antibody is seen with mixed connective tissue disease?

anti-RNP