Connective Tissue Diseases Flashcards

1
Q

what are autoantibodies

A

antibodies generated by the immune system against the bodys own proteins

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2
Q

what is another name for conditions with auto-antibody production

A

seropositive conditions

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3
Q

condition associated with anti-CCP antibody

A

rheumatoid arthritis

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4
Q

conditions associated with anti-nuclear antibody (5)

A

SLE
sjogrens
systemic sclerosis
MCTD
autoimmune liver disease

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5
Q

condition associated with anti-double stranded DNA antibody

A

SLE

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6
Q

condition associated with anti-Sm antibody

A

SLE

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7
Q

conditions associated with anti-Ro antibody

A

SLE, sjogrens

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8
Q

condition associated with anti-La antibody

A

sjogrens

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9
Q

condition associated with anti-centromere antibody

A

systemic sclerosis (limited)

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10
Q

condition associated with anti-Scl-70 antibody

A

systemic sclerosis (diffuse)

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11
Q

condition associated with anti-neutrophil cytoplasmic antibody

A

small vessel vasculitis

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12
Q

who is SLE more common in

A

Women
Asian, African, Caribbean and Hispanic ethnicity
Young to middle-aged adults

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13
Q

pathophysiology of SLE

A

anti-nuclear antibodies generate a chronic inflammatory response

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14
Q

name some non-specific symptoms of SLE

A

malar rash - worsened by sunlight
joint pain
muscle pain
non-scarring alopecia
raynauds
jaccouds arthropathy

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15
Q

describe the arthritis seen in SLE

A

synovitis or tenderness of at least 2 joints with >30 mins of early morning stiffness

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16
Q

what is jaccouds arthropathy

A

non-erosive reversible joint disoder

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17
Q

name a renal complication of SLE

A

nephritis

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18
Q

what is the first line inevstigation for SLE

A

antinuclear antibody titre

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19
Q

name some antibodies that may be tested for if ANA positive in suspected SLE

A

anti-dsDNA
anti-Ro
anti-smith

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20
Q

blood results in a patient with SLE

A

leukopenia, thrombocytopenia, haemolytic anaemia
low complement (C3+4)

21
Q

proteinuria in suspected SLE

A

indicated lupus nephritis

22
Q

investigation for lupus nephritis following positive urine dipstick

A

biopsy

23
Q

general management of SLE

A

sun protection
minimise steroid use
monitoring !!!

24
Q

management of mild-moderate SLE

A

hydroxychloroquine

25
Q

management of acute flairups of SLE

A

immunosuppressants, short stints of oral steroids

26
Q

management of severe organ damage associated with SLE

A

IV steroids and cyclophosphamide

27
Q

how do we monitor SLE

A

anti-dsDNA and complement levels, urinalysis for blood or protein

28
Q

complication of SLE

A

avascular necrosis, usually of the femoral head

29
Q

what is sjogrens syndrome

A

autoimmune condition that affects the exocrine glands

30
Q

who is more likely to present with sjogrens syndrome

A

middle aged women

31
Q

symptoms of sjogrens syndrome

A

dry eyes, mouth, throat
vaginal dryness in pre-menopausal women
joint pain
fatigue

32
Q

name 2 clinical signs of sjogrens syndrome

A

bilateral parotid gland enlargement
unexplained increase in dental carries

33
Q

investigations for sjogrens syndrome

A

bloods: anti-ro and anti-la antibodies

34
Q

management of sjogrens syndrome

A

symptom control: tear and salivary replacement, analgesia and exercise for pain and fatigue

35
Q

complications of sjogrens

A

increased risk of lymphoma
peripheral neuropathy
ILD

36
Q

what are the 2 main types of systemic sclerosis

A

limited and diffuse

37
Q

who usually presents with systemic sclerosis

A

women aged 30-50

38
Q

skin involvement in limited systemic sclerosis

A

face, hands, forearms and feet

39
Q

raynauds preceeding systemic sclerosis

A

limited

40
Q

clinical presentation of systemic sclerosis

A

CREST
calcinosis, Raynaud’s, oesophageal dysfunction, sclerodactyly, telangiectasia

41
Q

what is sclerodactyly

A

thickening of the skin of the fingers and hands which can cause loss of dexterity of hands

42
Q

name some complications of systemic sclerosis

A

pulmonary arterial hypertension
pulmonary fibrosis

43
Q

non-medical management of systemic sclerosis

A

avoid smoking
skin stretching, regular emollients
physiotherapy for joints

44
Q

what is used to treat symptoms of raynauds

A

nifedipine

45
Q

clinical presentation of mixed connective tissue disease (6)

A
  • Raynaud’s
  • Arthralgia/arthritis
  • Myositis
  • Sclerodactyly
  • Pulmonary hypertension
  • ILD
46
Q

3 main investigations for mixed connective tissue disease

A

bloods, antibodies, imaging

47
Q

bloods in mixed connective tissue disease

A

FBC: anaemia, leukopenia
hypergammaglobulinemia
raised inflammatory markers

48
Q

who usually presents with anti-phospholipid syndrome

A

women

49
Q

bloods in anti-phospholipid syndrome

A

thrombocytopenia
prolonged aPTT