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msk > connective tissue diseases > Flashcards

connective tissue diseases Flashcards

1
Q

what are connective tissue diseases

A

autoimmune conditions associated with spontaneous overactivity of the immune system
-auto antibodies

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2
Q

what is systemic lupus erythematosus

A

systemic autoimmune condition that can affect almost anywhere in the body
immune system attacks cells and tissue resulting in inflammation and tissue damage

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3
Q

is SLE more common in males or females

A

females

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4
Q

pathogenesis of SLE 1

A
  • loss of immune regulation
  • increased and defective apoptosis
  • necrotic cells release nuclear materials which act as auto-antigens
  • auto-immunity results from exposure to nuclear and cellular auto-antigens
  • B and T cells are stimulated
  • autoantibodies are produced
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5
Q

pathogenesis of SLE 2

A
  • complexes of antigens and auto-antibodies form and circulate
  • deposition of immune complexes in basement membrane
  • activation of complement which attracts leucocytes which release cytokines
  • cytokine release perpetuates inflammation which causes necrosis and scarring
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6
Q

is ANA positive or negative in SLE

A

positive

can be negative but very very rare

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7
Q

what must EULAR/ACR score be to confirm SLE

A

> 10

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8
Q

cutaneous features of SLE

A

subacute cutaneous or discoid lupus
acute cutaneous lupus
non-scarring alopecia
oral ulceration

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9
Q

types of arthritis associated with SLE

A

synovitis of at least 2 joints with >30 mins early morning stiffness
Jaccouds arthropathy

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10
Q

neurological manifestations of SLE

A
  • delirium
  • psychosis
  • seizure
  • headache
  • cranial nerve disorder
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11
Q

serositis manifestations of SLE

A
  • pleural or pericardial effusion

- acute pericarditis

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12
Q

haemotological manifestations of SLE

A
  • leukopenia
  • thrombocytopenia
  • haemolytic anaemia
  • lymphadenopathy
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13
Q

renal involvement of lupus

A
  • proteinuria >0.5g in 24 hrs
  • biopsy proven nephritis
  • red cell casts
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14
Q

what is a specific antibody for lupus

A

dsDNA

-present in 60% of lupus patients

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15
Q

what are some anti-phospholipid antibodies

A
  • lupus anticoagulant
  • anti-cardiolipin antibodies
  • anti-beta2glycoprotein antibodies
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16
Q

what is APLS associated with

A

venous and arterial thrombosis and recurrent miscarriage

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17
Q

what happens to complement levels when lupus is treated

A

they will normalise again from being low

18
Q

whats anti ro antibody associated with

A

neonatal lupus

congenital heart block

19
Q

what is management for all patients with lupus

A
  • sun protection measures
  • hydroxychlororoquine
  • steroids but in small doses and for short periods of time
  • monitor disease activity using SLEDAI score
20
Q

what are the symptoms of Sjrogen’s

A
  • dry eyes - gritty feeling
  • dry mouth
  • dry throat
  • vaginal dryness
  • bilateral parotid gland enlargement
  • joint pain
  • fatigue
  • unexplained increase in dental caries
21
Q

what antibodies for Sjogrens

A

anti ro
anti la
also may have raised IgG

22
Q

does Sjogrens patients have increased risk of lymphoma

A

YES

23
Q

what is systemic sclerosis

A

multisystem autoimmune disease characterised by vasculopathy, autoimmunity and fibrosis

24
Q

what are symptoms of systemic sclerosis

A
  • raynaud’s
  • skin thickening
  • difficulty swallowing
  • GORD
  • telangiectasia
  • calcinosis
  • +/- SOB
25
Q

red flags for systemic sclerosis

A
  • onset of Raynaud’s in mid adulthood
  • Raynaud’s and SOB
  • Raynaud’s and Telangiectasia
  • Digital ulcers/ischaemia
  • skin tightness/loss of dexterity of hands
26
Q

what defines diffuse cutaneous SSc

A

skin involvement on extremities above and below elbows and knees (plus face and trunk)

27
Q

what defines limited cutaneous SSc

A

skin involvement on extemities and only below elbows and knees (plus face)

28
Q

facial changes for systemic sclerosis

A
  • small mouth with puckering of lips
  • beaked nose
  • tightened/tight skin
  • telangiectasia
29
Q

GI complications of systemic sclerosis

A
  • dysphagia
  • GORD
  • gastric antral vascular ectasia
  • small intestine bacterial overgrowth
  • malabsorption
  • fluctuating bowel habit (constipation/diarrhoea)
  • faecal incontinence
30
Q

cardio/respiratory manifestations of systemic sclerosis

A
  • ILD
  • pulmonary arterial hypertension
  • myocardial disease
31
Q

what are renal complications of systemic sclerosis

A
  • scleroderma renal crisis

- non specific progressive renal dysfunction

32
Q

msk changes in SSc

A
  • sclerodactyly
  • digital ischaemia
  • myositis
33
Q

what are the three phases of Raynaud’s

A

1) blanching
2) acrocyanosis
3) reactive hyperaemia

34
Q

treatments of Raynaud’s/Vasculopathy

A
  • calcium channel blockers (Nifedipine)
  • others (fluoxetine, ARBs, Nitrates)
  • PDE-5 inhibitor (sildenafil)
  • Prostacyclin infusion (Iloprost)
  • endothelin receptor antagonist (Bosentan)
35
Q

what can increase risk of pulmonary hypertension

A
  • telangiectasia
  • anticentromere antibody
  • increased duration of disease
36
Q

treatment for systemic sclerosis pulmonary hypertension

A
  • PDE 5 inhibitor (Sildenafil/Tadalafil/Vardenafil)
  • ERA (Bosentan/Ambrisentan/Macitentan)
  • Eproprostenol infusions
  • oxygen
37
Q

treatment for ILD with systemic sclerosis

A
  • mycophenolate mofetil
  • rarely cyclophosphamide
  • Rituximab as second line
  • Nintedanib (antifibrotic)
  • lung transplant
38
Q

which antibody suggests renal crisis

A

Anti RNA polymerase III antibody

39
Q

what can put people at risk of renal crisis

A

high dose steroids

40
Q

features of renal crisis

A

uncontrolled hypertension with proteinuria and rapidly worsening renal function

41
Q

treatment for renal crisis

A

ACE inhibitors

42
Q

treatment for skin fibrosis with systemic sclerosis

A

Methotrexate

Mycophenolate

msk (45 decks)

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