Connective Tissue Disease

Question 1

What are connective tissue diseases characterised by?

Answer 1

presence of spontaneous over-activity of the immune systerm

Question 2

Who does SLE affect?

Answer 2

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

Question 3

How do hormones affect SLE incidence?

Answer 3

increased in those with higher oestrogen exposure- contraceptives and HRT

Question 4

What environmental factors can increase SLE?

Answer 4

virsues; UV light; silica dust

Question 5

What is the pathogenesis of SLE?

Answer 5

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

Question 6

What causes renal disease in SLE?

Answer 6

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

Question 7

What are the general symptoms seen with SLE?

Answer 7

fever; malaise; poor appetite; wt loss; fatiuge

Question 8

What are the mucocuntanoues features?

Answer 8

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

Question 9

What are the MSK features of SLE?

Answer 9

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

Question 10

What are the serositis features of SLE?

Answer 10

pericarditis; pleurisy; pleural effusion; pericardial effusion

Question 11

What are the features of renal disease clinically?

Answer 11

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

Question 12

What test should always be done in pts with SLE?

Answer 12

urinalysis

Question 13

What are the neurological features seen in SLE?

Answer 13

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

Question 14

What are the haemotological features of SLE?

Answer 14

lymphadenopathy; leucopenia; lymphopenia; haemolytic anaemia; thrombocytopenia

Question 15

What is anti-phospholipid syndrome?

Answer 15

venous and arterial thrombosis

Question 16

What are the features of anti-phospholiid syndrome?

Answer 16

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

Question 17

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

Answer 17

low complement; defective phagocytosis; impaired cell mediated immunity

Question 18

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

Answer 18

steroids and other immunosuppressive drugs

Question 19

What are the benefits and bad of ANA?

Answer 19

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

Question 20

What other antinuclear antibodies can be positive in SLE?

Answer 20

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

Question 21

What are the benefits of anti-dsDNA?

Answer 21

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

Question 22

What is Anti-Ro usually associated with?

Answer 22

anti-La

Question 23

What is anti-Ro often associated with?

Answer 23

congential heart block, neonatal LE, cutaenous manifestation

Question 24

What is the most specific autoantibody for SLE?

Answer 24

anti-Sm

Question 25

What are the antibodies present in antiphospholipid syndrom?

Answer 25

anti-cardiolipin lupus anticoagulant anti-beta 2 glycoprotein

Question 26

What antibody level postivitely correlates with activity?

Answer 26

anti-dsDNA

Question 27

What negatively correlate with activity?

Answer 27

C3/C4

Question 28

What are the drug treatmnents for SLE?

Answer 28

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

Question 29

What drugs are used in mild SLE?

Answer 29

HCQ; topical steroids; NSAIDS

Question 30

What drugs are used in moderate disease?

Answer 30

oral steroids; azathioprine; methotrexate

Question 31

What drugs are used in severe SLE?

Answer 31

IV steroids; cyclophosphamide; tituximab

Question 32

What is gender distribution in anti-phospholipid syndrome?

Answer 32

M:F 1:3.5

Question 33

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

Answer 33

Libman-Sacks endocarditis; migrane, transverse

Question 34

What happens in Sjogren's syndrome?

Answer 34

lymphocyte infiltration of exocrine glands causing xerostomia and keratoconjunctivitis

Question 35

What immunology might be seen with Sjogrens?

Answer 35

anti-Ro; anti-La

Question 36

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

Answer 36

schirmer test- paper on eye and in mouth

Question 37

What are the more serious manifestations of Sjogrens?

Answer 37

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

Question 38

Who gets Sjogrens?

Answer 38

40-60yo; M:F 1:9

Question 39

What are the treatment options for Sjogrens?

Answer 39

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

Question 40

What is systemic sclerosis caused by?

Answer 40

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

Question 41

What are the symptoms of systemic sclerosis?

Answer 41

``` Calcinosis Raynauds Esophageal dysmotility Sclerodactlyl Telangiectasia AND pulmonary HT ```

Question 42

What antibodies are systemic sclerosis associated with?

Answer 42

anti-centromere | anti-Scl-70

Question 43

What is diffuse systemic sclerosis?

Answer 43

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

Question 44

What are the GI organ manifestations of systemic sclerosis?

Answer 44

``` small bowel dysmotility bacterial overgrowth rectal dysmotility pancreatic insufficiency dysphagia ```

Question 45

What are the respiratory manifestations of systemic sclerosis?

Answer 45

ILD; pulmonary HT; chest wall restriction

Question 46

What are the CVS manifestations of systemic sclerosis?

Answer 46

Raynauds; atherosclerosis; hypertensive cardiomyopathy

Question 47

Who is systemic sclerosis seen in?

Answer 47

25-55yo | M:F 1:4

Question 48

What are the treatments for systemic sclerosis?

Answer 48

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

Question 49

What is undifferentiated connective tissue disorder?

Answer 49

connecitve tissueish but dont fit a partiuclar syndrome (yet)

Question 50

What antibody is seen with mixed connective tissue disease?

Answer 50

anti-RNP