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MSK > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

What are connective tissue diseases characterised by?

A

presence of spontaneous over-activity of the immune systerm

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2
Q

Who does SLE affect?

A

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

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3
Q

How do hormones affect SLE incidence?

A

increased in those with higher oestrogen exposure- contraceptives and HRT

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4
Q

What environmental factors can increase SLE?

A

virsues; UV light; silica dust

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5
Q

What is the pathogenesis of SLE?

A

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

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6
Q

What causes renal disease in SLE?

A

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

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7
Q

What are the general symptoms seen with SLE?

A

fever; malaise; poor appetite; wt loss; fatiuge

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8
Q

What are the mucocuntanoues features?

A

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

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9
Q

What are the MSK features of SLE?

A

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

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10
Q

What are the serositis features of SLE?

A

pericarditis; pleurisy; pleural effusion; pericardial effusion

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11
Q

What are the features of renal disease clinically?

A

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

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12
Q

What test should always be done in pts with SLE?

A

urinalysis

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13
Q

What are the neurological features seen in SLE?

A

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

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14
Q

What are the haemotological features of SLE?

A

lymphadenopathy; leucopenia; lymphopenia; haemolytic anaemia; thrombocytopenia

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15
Q

What is anti-phospholipid syndrome?

A

venous and arterial thrombosis

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16
Q

What are the features of anti-phospholiid syndrome?

A

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

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17
Q

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

A

low complement; defective phagocytosis; impaired cell mediated immunity

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18
Q

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

A

steroids and other immunosuppressive drugs

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19
Q

What are the benefits and bad of ANA?

A

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

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20
Q

What other antinuclear antibodies can be positive in SLE?

A

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

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21
Q

What are the benefits of anti-dsDNA?

A

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

22
Q

What is Anti-Ro usually associated with?

A

anti-La

23
Q

What is anti-Ro often associated with?

A

congential heart block, neonatal LE, cutaenous manifestation

24
Q

What is the most specific autoantibody for SLE?

A

anti-Sm

25
Q

What are the antibodies present in antiphospholipid syndrom?

A

anti-cardiolipin
lupus anticoagulant
anti-beta 2 glycoprotein

26
Q

What antibody level postivitely correlates with activity?

A

anti-dsDNA

27
Q

What negatively correlate with activity?

A

C3/C4

28
Q

What are the drug treatmnents for SLE?

A 
NSAIDs and simple analgesia
anti-malarials- hydroxychlorquine 
steroids
immunosuppressives eg azathioprine
biologics eg rituximab
29
Q

What drugs are used in mild SLE?

A

HCQ; topical steroids; NSAIDS

30
Q

What drugs are used in moderate disease?

A

oral steroids; azathioprine; methotrexate

31
Q

What drugs are used in severe SLE?

A

IV steroids; cyclophosphamide; tituximab

32
Q

What is gender distribution in anti-phospholipid syndrome?

A

M:F 1:3.5

33
Q

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

A

Libman-Sacks endocarditis; migrane, transverse

34
Q

What happens in Sjogren’s syndrome?

A

lymphocyte infiltration of exocrine glands causing xerostomia and keratoconjunctivitis

35
Q

What immunology might be seen with Sjogrens?

A

anti-Ro; anti-La

36
Q

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

A

schirmer test- paper on eye and in mouth

37
Q

What are the more serious manifestations of Sjogrens?

A

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

38
Q

Who gets Sjogrens?

A

40-60yo; M:F 1:9

39
Q

What are the treatment options for Sjogrens?

A

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

40
Q

What is systemic sclerosis caused by?

A

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

41
Q

What are the symptoms of systemic sclerosis?

A 
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactlyl
Telangiectasia
AND pulmonary HT
42
Q

What antibodies are systemic sclerosis associated with?

A

anti-centromere

anti-Scl-70

43
Q

What is diffuse systemic sclerosis?

A

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

44
Q

What are the GI organ manifestations of systemic sclerosis?

A 
small bowel dysmotility
bacterial overgrowth
rectal dysmotility
pancreatic insufficiency
dysphagia
45
Q

What are the respiratory manifestations of systemic sclerosis?

A

ILD; pulmonary HT; chest wall restriction

46
Q

What are the CVS manifestations of systemic sclerosis?

A

Raynauds; atherosclerosis; hypertensive cardiomyopathy

47
Q

Who is systemic sclerosis seen in?

A

25-55yo

M:F 1:4

48
Q

What are the treatments for systemic sclerosis?

A

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

49
Q

What is undifferentiated connective tissue disorder?

A

connecitve tissueish but dont fit a partiuclar syndrome (yet)

50
Q

What antibody is seen with mixed connective tissue disease?

A

anti-RNP

MSK (39 decks)

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