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MSK SOH > Connective tissue diseases > Flashcards

Connective tissue diseases Flashcards

1
Q

What are CTD?

A

Characterised by presence of auto-antibodies

Characterised by spontaneous over activity of the immune system

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2
Q

SLE - definiton

A

Systemic auto-immune disease that can affect any part of the body

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3
Q

SLE - epidemiology

M/F, age, ethnicity

A

More common in females
After puberty
Asians, Afro-carribeans

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4
Q

SLE - causes

A

Increased oestrogen levels
Silica dust exposure
UV light

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5
Q

SLE - pathogenesis

A

Defect in apoptosis causes increased cell death.
Debris of dead cells are not effectively cleared which allows the persistence of antigen and immune complex production.
Complement activation and inflammation.
Dead cells release nuclear material which act as potential auto-antigens
T and B cells are stimulated
Auto-antibodies are produced
Deposition of small immune complexes in the skin

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6
Q

SLE - clinical features

A

Involves a wide range of body systems
General: fever, fatigue, malaise, weight loss
MSK: arthralgia, myalgia, inflammatory arthritis
Muco-cutaneous: Butterfly malar rash which spares naso-labial folds, discoid lupus (circular), painless mouth ulcers, alopecia, raynauds
Resp: pleural effusion, PE, ILD
Renal: lupus nephritis
Neuro: headache, depression

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7
Q

SLE - which type of hypersensitivity reaction is involved?

A

Type III hypersensitivity (immune complex mediated)

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8
Q

SLE - immunology

A

Complement binding to immune complexes helps remove antigen from the body
Large complexes can be removed by macrophages (phagocytosis)
Small complexes are not removed as easily so they inset into the small blood vessels and cause symptoms

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9
Q

SLE - investigations

A

Bloods
- raised inflammatory markers
- FBC: thrombocytopenia, anaemia, leukopenia
Auto-antibodies
- +ve ANA (not specific - can be +ve in other conditions. Usually over 1:160 titre in SLE patients)
- If ANA is +ve, do more specific testing to determine cause
- Anti-dsDNA - levels change depending on flare ups
- Anti-Sm - highly specific
- Anti-Ro
- Anti-RNP
Decreased complement
- Decreased C3 and C4 levels (as complement is consumed by immune processes)

MUST SCREEN FOR ORGAN INVOLVEMENT
Urinalysis - screen for renal disease
Renal biopsy
Imaging - x-ray, CT, MRI

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10
Q

SLE - MILD management

A

Hydroxychloroquine
- for arthritis, skin manifestations
Topical steroids
NSAIDs

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11
Q

SLE - MODERATE management

A

Oral steroids
Immunosuppressants
- azathioprine, methotrexate, myclophenolate mofetil)
- to decrease production of auto-antibodies

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12
Q

SLE - SEVERE management

A

IV steroids
IV immunosuppressants
- cyclophosphamide
Rituximab

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13
Q

APS - definition

A

Venous or arterial thrombosis and/or adverse pregnancy outcomes

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14
Q

APS - Epidemiology

M/F, age

A

More common in females

Young patients

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15
Q

APS - clinical features

A

1 or more otherwise unexplained venous or arterial events
Foetal death after 10 weeks
3 pregnancy losses before 10 weeks
Premature birth (due to severe pre-eclampsia or placental insufficiency)

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16
Q

APS - investigations

A 
Bloods
- thrombocytopenia
Antibodies
- Lupus anticoagulant
- IgM or IgG beta 2 glycoproteins
- IgM or IgG anticardiolipin antibodies
17
Q

APS - management

A

Life long anti-coagulation
LMWH/Aspirin (in patients with recurrent pregnancy loss)
(Do NOT use warfarin, it is teratogenic)
Hydroxychloroquine

18
Q

Sjogrens syndrome - definition

A

Characterised by lymphatic infiltrates in exocrine organs. Diminished lacrimal and salivary gland function

19
Q

Sjogrens syndrome - epidemiology

M/F, age

A

More common in females

Age 50-60

20
Q

Sjogrens syndrome - clinical features

A 
Dry eyes 
Dry mouth 
Dry throat
Vaginal dryness
Joint pains
Fatigue
21
Q

Sjogrens syndrome - investigations

A 
Diagnosis based on ocular dryness 
- Schrimmers test (to assess tear production) 
Auto-antibodies 
- +ve Anti-Ro
- +ve Anti-La
22
Q

Sjogrens syndrome - management

A

Lubricating eye drops

Saliva replacement

23
Q

Systemic sclerosis - definition

A

Excessive collagen deposition

24
Q

Systemic sclerosis - DIFFUSE form clinical features

A

Early organ involvement
skin changes: proximal to forearms, involves torso
ILD

25
Q

Systemic sclerosis - DIFFUSE form auto-antibodies

A

Anti-Scl-70

26
Q

Systemic sclerosis - LIMITED form clinical features

A

Skin changes: distal to elbows, face, feet

27
Q

Systemic sclerosis - LIMITED form auto-antibodies

A

Anti-centromere

28
Q

Systemical sclerosis - general features

A 
Tightening of the skin
Raynauds 
Calcinosis
Sclerodactyly 
Telangectasia
29
Q

Systemic sclerosis - management

A

Raynauds: CCB

Renal involvement: ACE inhibitors

30
Q

Mixed CTD - definition

A

Features of SLE, polymyositis, systemic sclerosis

31
Q

Mixed CTD - clinical features

A 
Raynauds
Arthralgia
Myositis
Sclerodactyly 
Pulmonary hypertension
ILD
32
Q

Mixed CTD - auto-antibodies

A

Anti-RNP

MSK SOH (24 decks)

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