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Musculoskeletal > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

1
Q

list key connective tissue disorders

A 
systemic lupus erythematous
Sjorgen's syndrome
autoimmune inflammatory muscle disease
systemic sclerosis (scleroderma)
overlap syndromes
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2
Q

what is rheumatoid arthritis?

A

chronic joint inflammation that can result in joint damage

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3
Q

site of inflammation of rheumatoid arthritis

A

synovium

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4
Q

rheumatoid arthritis is associated with?

A

autoantibodies

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5
Q

what is ankylosing spondylitis?

A

chronic spinal inflammation that can result in spinal fusion and deformity

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6
Q

site of inflammation of ankylosing spondylitis

A

includes the enthesis

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7
Q

is ankylosing spondylitis associated with autoantibodies?

A

no

‘seronegative’

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8
Q

list seronegative spondyloarthropathies

A

ankylosing spondylitis
reactive arthritis (Reiter’s syndrome)
Psoriatic arthritis
arthritis associated with GI inflammation (enteropathic synovitis)

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9
Q

what is systemic lupus erythematosus?

A

chronic tissue inflammation in the presence of antibodies directed against self antigens

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10
Q

site of inflammation of SLE?

A

multi site

particularly the joints, skin, kidney

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11
Q

is systemic lupus erythematosus associated with autoantibodies?

A

yes
anti nuclear antibodies
anti-double stranded DNA antibodies
anti-phospholipid antibodies

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12
Q

list connective tissue diseases

A 
SLE
Sjogren's syndrome
autoimmune inflammatory muscle disease
systemic sclerosis
overlap syndrome
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13
Q

what phenomenon is common in connective tissue disorders?

A

Raynaud’s phenomenon

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14
Q

what is Raynaud’s phenomenon?

A

intermittent vasospasm of digits on exposure to cold

colour change: blanching of digits, cyanosis, reactive hyperaemia

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15
Q

typical demographic for SLE

A

female

15-45

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16
Q

clinical manifestations of SLE include

A 
malar rash
photosensitive rash
mouth ulcers
hair loss
Raynaud's phenomenon
arthralgia/arthritis
serositis e.g. pericarditis, pleuritis
renal disease e.g. glomerulonephritis
cerebral disease e.g. psychosis
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17
Q

current paradigm for pathogenesis of systemic lupus erythematosus

A

apoptosis > translocation of nuclear antigens to membrane surface > impaired clearance of apoptotic cells > enhanced presentation of nuclear antigens to immune cell > B cell autoimmunity > tissue damage by antibody effector mechanisms e.g. complement activation and Fc receptor engagement

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18
Q

what autoantibodies are involved in systemic vasculitis?

A

antinuclear cytoplasmic antibodies

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19
Q

antinuclear antibody screening includes?

A 
Anti-Ro
Anti-La
Anti-centromere
Anti-Sm
Anti-RNP
Anti-ds-DNA antibodies 
Anti-Scl-70
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20
Q

cytoplasmic autoantibodies include?

A

Anti-tRNA synthetase antibodies

Anti-ribosomal P antibodies

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21
Q

which autoantibodies are specific for SLE?

A

anti dsDNA

anti Sm

22
Q

antiphospholipid antibodies are associated with the risk of what in SLE?

A

arterial and venous thrombosis

23
Q

serum level of anti-dsDNA antibody correlates with?

A

disease activity

24
Q

treatment for SLE aims for?

A

remission or low disease activity and prevention of flares

25
Q

drug treatments for SLE patients

A

hydroxychloroquine recommended
maintenance treatment glucocorticoids minimised (can be helped by methotrexate, azathioprine)
in persistently active/severe disease use cyclophosphamide and B cell targeted therapies e.g. rituximab/belimumab

26
Q

patients with SLE should be assessed for?

A

antiphospholipid antibody status
infectious and cardiovascular diseases risk profile
pregnancy planning

27
Q

disease activity in SLE

A 
low complement C3/C4
high anti-dsDNA antibodies
low platelet count
low albumin
low Hb, lymphocyte
high reticulocyte
28
Q

what is sjogren’s syndrome?

A

chronic autoimmune disease affecting salivary glands and tear glands, autoimmune exocrinopathy

29
Q

exocrine gland pathology results in?

A 
dry eyes (xerophthalmia)
dry mouth (xerostomia)
parotid gland enlargement
30
Q

Commonest extra-glandular manifestations in Sjogren’s syndrome are?

A

non-erosive arthritis

Raynaud’s phenomenon

31
Q

salivary gland biopsy of patient with Sjogren’s syndrome will show?

A

lymphocytic infiltration predominantly CD4 helper T cells and to lesser extent B lymphocytes

32
Q

what is Schirmer’s test?

A

a test to assess tear production. Filter paper is placed under lower eyelid and extent of wetness measured after 5 minutes

33
Q

abnormal result of Schirmer’s test is?

A

<5mm after 5 minutes

34
Q

Sjogren’s syndrome typical demographic

A

females

35-50

35
Q

inflammatory muscle disease

A

Proximal muscle weakness due to autoimmune-mediated inflammation either with (dermatomyositis) or without (polymyositis) a rash

36
Q

skin changes in dermatomyositis

A

Lilac-coloured (heliotrope) rash on eyelids, malar region and naso-labial folds
Red or purple flat or raised lesions on knuckles (Gottron’s papules)
Subcutaneous calcinosis
Mechanic’s hands (fissuring and cracking of skin over finger pads)

37
Q

inflammatory muscle disease is associated with?

A

malignancy

pulmonary fibrosis

38
Q

inflammatory muscle disease will have what abnormal test results?

A

elevated CPK
abnormal electromyography
abnormal muscle biopsy (polymyositis = CD8 T cells; dermatomyositis = CD4 T cells in addition to B cells)

39
Q

what is systemic sclerosis?

A

group of diseases causing hardening and tightening of skin and connective tissues.

40
Q

manifestations of systemic sclerosis

A

dermal fibrosis

cutaneous calcinosis telangiectasia

41
Q

skin changes in systemic sclerosis may be ________ or ______

A

diffuse

limited

42
Q

diffuse systemic sclerosis

A

Fibrotic skin proximal to elbows or knees (excluding face and neck)
Anti-topoisomerase-1 (anti-Scl-70) antibodies
Pulmonary fibrosis, renal (thrombotic microangiopathy) involvement
Short history of Raynaud’s phenomenon

43
Q

limited systemic sclerosis

A

Fibrotic skin hands, forearms, feet, neck and face
Anti-centromere antibodies
Pulmonary hypertension
Long history of Raynaud’s phenomenon

44
Q

CREST describes a sub-type of limited systemic sclerosis, it stands for?

A 
Calcinosis
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactyly
Telangietasia
45
Q

what is overlap syndrome?

A

features of more than 1 connective tissue disorder are present e.g. SLE and inflammatory muscle disease

46
Q

what is undifferentiated connective tissue disease?

A

When incomplete features of a connective tissue disease are present

47
Q

what is mixed connective tissue disease?

A

In one instance a group of patients with features of seen in SLE, scleroderma, rheumatoid arthritis, and polymyositis were identified by the presence of an autoantibody:
Anti-U1-RNP antibody

48
Q

key autoantibodies in diffuse systemic sclerosis

A

anti-Scl-70 antibody

49
Q

key autoantibodies in limited systemic sclerosis

A

anti-centromere antibodies

50
Q

key autoantibodies in dermato-/polymyositis

A

anti-tRNA transferase antibodies

51
Q

key autoantibodies in Sjogren’s syndrome

A

no unique antibodies but typically see
antinuclear antibodies (anti-Ro and anti-La)
rheumatoid factor

52
Q

key autoantibodies in mixed connective tissue disorders

A

anti-U1-RNP antibodies

Musculoskeletal (7 decks)

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