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Musculoskeletal > Autio-immune Connective Tissue Diseases > Flashcards

Autio-immune Connective Tissue Diseases Flashcards

1
Q

What is connective tissue?

A

Tissue that connects, supports, binds, or separates other tissues or organs

Typically having relatively few cells embedded in an amorphous matrix, with collagen and other fibres

Adipose tissue, cartilage, bone, blood, muscle
Loose connective tissue: fascia under skin
Fibrous connective tissue: tendons, ligaments

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2
Q

What are the main auto-immune connective tissue diseases?

A 
Systemic Lupus Erythematosus (SLE)
Systemic Sclerosis
Sjogren's Syndrome
Dermatomyositis + Polymyositis
Anti-phospholipid Syndrome
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3
Q

What is SLE?

A

Systemic Lupus Erythematosus

A systemic inflammatory disease

Characterised by the presence of auto-antibodies in the serum

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4
Q

Who gets SLE?

A

90% of patients are women

Present between age 14-64

More prevalent in afro-carribbeans

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5
Q

What causes SLE?

A

Hereditary

Genetics: certain HLA types

Links with oestrogen, hence more common in females

Drugs

UV light can trigger flares

Exposure to EBV virus

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6
Q

What drugs cause SLE?

Once they have caused it, is it permanent?

A

Hydralazine: smooth muscle relaxant
Isoniazid: antibiotic

No, once the drug is withdrawn often the SLE goes away

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7
Q

What is the pathogenesis of SLE?

A

Apoptotic cells and cell fragments are not cleared up efficiently by phagocytes

These fragments containing auto-antigens are taken up by APCs and presented to the T cells

These stimulate B cells to produce antibodies against these antigens - autoantibodies

Autoantigen-autoantibody complexes form and are deposited in areas around the body

This activates the complex system, influx of neutrophils, an inflammatory response occurs

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8
Q

What are the clinical features of SLE?

A

So many!!

Non-specific malaise
Fatigue
Myalgia
Fever

Lymphadenopathy
Weight loss
Alopecia
Skin rashes
Haematological disorders
Recurrent abortion
Neuro-psychiatric features
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9
Q

What skin rashes occur in SLE?

A

Butterfly (malar) rash

Photosensitive rash

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10
Q

What haematological disorders occur in SLE?

A 
Anaemia
Thrombocytopenia: platelet deficiency
Neutropenia
Lymphopenia
Venous + arterial thrombosis
Vasculitis
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11
Q

Why do SLE patients get recurrent abortion?

A

Clots form in the placenta

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12
Q

What neuro-psychiatric features occur in SLE?

A

Many…

Aseptic meningitis
Cerebrovascular disease
Psychosis
Depression

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13
Q

The American College of Rheumatology set out a guideline of what symptoms you need to see before being able to diagnose SLE.
How many symptoms on the list must the patient have?

A

4

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14
Q

Investigation of SLE?

A

Blood:

  • anaemia
  • neutro/lymphopenia
  • thrombocytopenia
  • raised ESR
  • serum autoantibodies

Biopsy of skin or kidney
- see deposition of autoantobody-autoantigen complexes + complement

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15
Q

Is CRP usually raised in SLE?

A

Not usually, unless they have a concurrent infection

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16
Q

Which serum autoantibodies are involved in SLE?

A

Anti-dsDNA (specific for lupus)
Anti-nuclear
Anti-La and Anti-Ro

Many more!

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17
Q

On a blood test, which serum autoantibody is only seen in SLE?

A

Anti-dsDNA

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18
Q

Management of SLE?

A

Advise on avoiding risk factors

Drug treatment:

  • NSAIDs
  • Steroids
  • Immunosuppressives
  • Biologics

Plasmapheresis

Stem cell transplants

Monitor the patient for any major organ involevement that may arise

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19
Q

What risk factors would you advise an SLE patient avoid?

A

Excessive sunlight

Reduce cardiovascular risk:

  • stop smoking
  • healthy diet
  • exercise

These things will reduce the risk of a flare-up

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20
Q

What is plasmapheresis?

A

Like dialysis

Use a machine to wash the autoantibodies out of the blood

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21
Q

Most patients with SLE can live a full life.

True or false?

A

True

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22
Q

What is anti-phospholipid syndrome?

A

A condition featuring antibodies against phospholipids

Occurs on its own or as in association with another autoimmune condition

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23
Q

What does anti-phospholipid syndrome cause?

A

CLOT

C - coagulation defect
L - livedo reticularis (mottled skin rash
O - obstetric problems (recurrent miscarriage)
T - thrombocytopenia

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24
Q

Investigation of anti-phospholipid syndrome?

A

Blood tests:

you will see the anti-phospholipid antibodies

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25
Q

Management of anti-phospholipid syndrome?

A

Warfarin to prevent clotting

Or aspirin as prophylaxis

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26
Q

What is livedo reticularis?

A

A mottled skin rash, caused by capillary dilation and blood stasis in skin venules

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27
Q

What is Sjogren’s syndrome?

A

An autoimmune disorder where epithelial exocrine glands are destroyed, especially the lacrimal and salivary gland

28
Q

Who gets Sjogren’s syndrome?

A

Often middle aged women

40s-50s

29
Q

What causes Sjogren’s syndrome?

A

Primary: just happens

Secondary: as a result of other autoimmune conditions

  • SLE
  • RA
  • Scleroderma
  • Primary Biliary Cirrhosis
30
Q

Clinical features of Sjogren’s syndrome?

A

Sicca complex:

  • dry eyes
  • dry mouth
  • enlarged salivary glands

Systemic features:

  • inflammatory arthritis
  • neuropathies
  • vasculitis
31
Q

What is the ‘sicca complex’?

A

Dry eyes and dry mouth

32
Q

What does xerostomia mean?

A

Dry mouth

33
Q

What does keratoconjunctivitis sicca mean?

A

Dry eyes

34
Q

Investigation of Sjogren’s syndrome?

A

Blood:

  • serum autoantibodies
  • raised ESR

Labial (lip) gland biopsy:
destruction + lymphocyte infiltration

Schirmer test: positive

35
Q

Which autoantibody is commonly seen in patients with Sjogren’s syndrome?

A

Antinuclear

36
Q

What is the Schirmer’s test?

A

Put a strip of filter paper on the inside of the eyelid

If it doesn’t get wet up to 10mm in 5 mins then the test is positive and there is defective tear production

37
Q

Management of Sjogren’s syndrome?

A

Treat symptoms:

  • artificial tears
  • saliva replacement

Immunosuppression for systemic problems

38
Q

What is systemic sclerosis?

A

A multi-system autoimmune disease, with particular involvement of the skin

39
Q

What is scleroderma?

A

Another name for systemic sclerosis

40
Q

What two types of systemic sclerosis are there?

A

Limited (CREST)

Diffuse

41
Q

What causes systemic sclerosis?

A

Genetic predisposition
Triggered by immune activation, infection, environmental toxin

Causes the immune system to produce autoantibodies

Connective tissue cells begin to overproduce collagen, causing fibrosis and thickening of the tissue

42
Q

What are the clinical features of CREST systemic sclerosis?

A 
C - calcinosis
R - Raynaud's phenomenon
E - esophageal reflux + strictures
S - sclerodactyl
T - telangiectasia

PLUS: pulmonary artery hypertension

43
Q

What is calcinosis?

A

Lumps of calcium deposited under the skin

44
Q

What is sclerodactyl?

A

Tightness, thickening of skin on fingers and toes

45
Q

What is telangiectasia?

A

Dilatation of capillaries

They appear as red/purple spidery clusters on skin or organs

46
Q

What are the clinical features of diffuse systemic sclerosis?

A

Proximal scleroderma: hardening of the skin

Lung: pulmonary fibrosis

GI tract:

  • dilatation of oesophagus
  • obstruction of colon

Renal crisis - hypertension

Myositis: inflammation + degeneration of muscle

Heart:
- fibrosis causing arrhythmia

47
Q

Management of systemic sclerosis?

A

No proper treatment
Treat the symptoms:

  • Raynauds
  • Reflux
  • Prevent renal crisis
  • Treat skin oedema

Monitor organs for early detection, especially pulmonary artery hypertension

48
Q

What is the most important thing to do when managing a patient with systemic sclerosis?

A

Monitor regularly for pulmonary artery hypertension

If not it can be serious: SOB, R heart damage, hypoxia

49
Q

What is dermatomyositis?

A

Inflammation and necrosis of the skin and skeletal muscle

50
Q

What is polymyositis?

A

Inflammation and necrosis of many skeletal muscles, but not the skin

51
Q

What causes dermatomyositis and polymyositis?

A

Can just occur

Can be a para-neoplastic syndrome

52
Q

What should you do if you see someone with dermatomyositis but their serum blood tests showed nothing?

A

Suspect a malignancy

53
Q

Clinical features of dermatomyositis?

A

Skin rash:

  • macular rash over back and shoulders
  • heliotrope (lilac) rash on eyelids and knuckes

Muscle weakness
Myalgia + arthralgia

Fever
Raynaud’s
Interstitial lung involvement
Myocarditis + arrhythmia

54
Q

Clinical features of polymyositis?

A

Muscle weakness
Myalgia + arthralgia

Fever
Raynaud’s
Interstitial lung involvement
Myocarditis + arrhythmia

55
Q

Investigation of dermatomyositis and polymyositis?

A

Muscle/skin biopsy to look for signs of inflammation

  • inflammatory cell infiltration
  • necrosis

Serum muscle enzymes will be raised

Serum autoantibodies:

  • anti-JO
  • if none suspect malignancy

Electromyography: looks at electrical activity of skeletal muscles

56
Q

Management of dermatomyositis and polymyositis?

A

Steroids short term until symptoms have settled

Immunosuppressive drugs: methotrexate

57
Q

What is Raynaud’s syndrome?

A

Peripheral digital ischaemia due to paroxysmal (sudden) vasospasm caused by cold weather or emotion

Causes the digits to change colour and ache

Refers to both Raynaud’s disease and phenomenon

58
Q

What colour(s) do the digits turn in Raynaud’s and why?

A

First they go white due to ischaemia

Then they go blue due to deoxygenation

Then they go red due to blood rushing black after vasospasm has stopped, reactive hyperaemia

59
Q

What is the difference between Raynaud’s disease and Raynaud’s phenomenon?

A

Disease: primary, idiopathic cause

Phenomenon: secondary, a result of an underlying cause

60
Q

When does Raynaud’s disease (primary Raynaud’s) usually present?

A

In late teens, early 20s

61
Q

What are some causes of Raynaud’s phenomenon (secondary Raynaud’s)?

A

Connective tissue diseases:

  • SLE
  • Systemic Sclerosis

Drugs

Vascular damage:

  • atherosclerosis
  • frost bite
  • long term use of vibrating tools
62
Q

Investigation of Raynaud’s syndrome?

A

Often have to rely on history because in consulting room they will be warm

Look for an underlying cause, like SLE etc.

63
Q

Management of Raynaud’s phenomenon?

A

Lifestyle advice:

  • keep hands warm
  • stop smoking

Treat underlying cause if there is one

Drugs:

  • nifedipine
  • sildenafil (Viagra)
  • fluoxetine (SSRI)

Sympathectomy: chemical or surgical

64
Q

What is a Sympathectomy?

A

Surgery where a portion of the sympathetic nerve trunk in the thoracic region is destroyed

This prevents excessive vasoconstriction that occurs in Raynaud’s

65
Q

What type of drug is nifedipine?

A

Calcium channel blocker

Prevents vasospasm

Musculoskeletal (15 decks)

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