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Rheumatology > Autoimmune conditions > Flashcards

Autoimmune conditions Flashcards

1
Q

What is Systemic lupus erythematosus

A
  • inflammatory autoimmune connective tissue disease
  • It presents with varying and non-specific symptom
  • more common in women and Asians
  • Presents in young and middle ages
  • Often takes a relapsing-remitting cause
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2
Q

Prognosis of Systemic lupus erythematosus

A
  • Chronic inflammation of vital organs such as the heart and kidneys can lead to problems
  • Cardiovascular disease and infection are leading causes of death.
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3
Q

What is the presentation of Systemic lupus erythematosus

A 
Fatigue
Weight loss
Arthralgia (joint pain) and non-erosive arthritis
Myalgia (muscle pain)
Fever
Photosensitive malar rash
Lymphadenopathy and splenomegaly
Shortness of breath
Pleuritic chest pain
Mouth ulcers
Hair loss
Raynaud’s phenomenon
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4
Q

What rash is seen in Systemic lupus erythematosus

A

Photosensitive malar rash. This is a “butterfly” shaped rash across the nose and cheek bones that gets worse with sunlight.

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5
Q

Which antibodies are mostly associated with Systemic lupus erythematosus

A
  • Anti-nuclear antibodies

- Anti-ds-DNA

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6
Q

What is the pathology behind Systemic lupus erythematosus

A
  • Anti-nuclear antibodies targets proteins in the nucleus in the cells
  • This generates an inflammatory response leading to many different types of symptoms
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7
Q

What autoantibodies should be looked for if suspected Systemic lupus erythematosus

A
  • anti-nuclear antibodies (ANA) (85%)
  • Anti-double stranded DNA (anti-dsDNA): VERY specific (70%)
  • Anti-Smith (highly specific to SLE but not very sensitive)
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8
Q

Which antibodies are mostly associated with limited cutaneous systemic sclerosis

A

Anti-centromere antibodies

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9
Q

Which antibodies are mostly associated with Sjogren’s syndrome

A

Anti-Ro and Anti-La

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10
Q

Which antibodies are mostly associated with systemic sclerosis

A

Anti-Scl-70

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11
Q

Which antibodies are mostly associated with dermatomyositis

A

Anti-Jo-1

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12
Q

How do you diagnose SLE

A
  • SLICC Criteria or the ACR Criteria
  • Confirming the presence of antinuclear antibodies and establishing a certain number of clinical features suggestive of SLE.
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13
Q

What are the complications of SLE

A
  • Cardiovascular disease: HTN and CAD
  • Infection (due to immunosupression)
  • Anaemia of chronic disease: normocytic anaemia (can be pancytopaenic)
  • Pericarditis
  • Pleuritis
  • Lupus nephritis
  • Pulmonary fibrosis
  • Neuropsychiatric SLE
  • Recurrent miscarriage (& other pregnancy complications)
  • VTE (anti-phospholipid syndrome)
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14
Q

What is lupus nephritis

A
  • occurs due to inflammation in the kidney
  • can progress to end-stage renal failure
  • assessed via urine protein:creatinine ratio and renal biopsy
  • renal biopsy is often repeated to assess response to treatment.
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15
Q

What is Neuropsychiatric SLE

A
  • caused by inflammation in the central nervous system
  • optic neuritis (inflammation of the optic nerve),
  • transverse myelitis (inflammation of the spinal cord)
  • psychosis.
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16
Q

What is the first line treatment of systemic lupus erythematous

A
  • NSAIDs
  • Steroids (prednisolone)
  • Hydroxychloroquine (first line for mild SLE)
  • Suncream and sun avoidance for the photosensitive the malar rash
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17
Q

In resistant or more severe lupus, what is the second line treatment

A
  • Immunosupression e.g. methotrexate, azothioprine, tacrolimus, ciclosporin
  • Biological therapies e.g. rituximab or bulimumab
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18
Q

What is Discoid lupus erythematous

A
  • Non-cancerous chronic skin condition
  • Rarely the lesions can progress to squamous cell carcinoma
  • increased risk of developing systemic lupus erythematosus
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19
Q

What do discoid lupus erythematous lesions look like

A 
Inflamed
Dry
Erythematous
Patchy
Crusty and scaling
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20
Q

What is the management of discoid lupus erythematous

A
  • Skin biopsy confirms diagnosis
  • Sun protection
  • Topical steroids
  • Intralesional steroid injections
  • Hydroxychloroquine
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21
Q

What is Sjogren’s syndrome

A
  • autoimmune condition
  • affects the exocrine glands
  • Leads to dry mucous membranes, such as dry mouth, dry eyes and dry vagina
  • Can be primary or secondary (SLE)
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22
Q

What is the Schirmer Test

A
  • folded piece of filter paper under the lower eyelid with a strip hanging out over the eyelid
  • left in for 5 minutes and the distance along the strip hanging out that becomes moist is measured
  • tears should travel 15mm in a healthy young adult
  • A result of less than 10mm is significant.
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23
Q

What is the management of Sjogren’s syndrome

A

Artificial tears
Artificial saliva
Vaginal lubricants
Hydroxychloroquine is used to halt the progression of the disease.

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24
Q

What systemic affects can Sjogren’s Occasionally have?

A 
Pneumonia and bronciectasis
Non-Hodgkins lymphoma
Peripheral neuropathy
Vasculitis
Renal impairment
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25
Q

What eye complications can Sjogren’s cause

A

conjunctivitis and corneal ulcers

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26
Q

What oral complications can Sjogren’s cause

A

dental cavities and candida infections

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27
Q

What vaginal compliactions can Sjogren’s cause

A

candidiasis and sexual dysfunction

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28
Q

What does scleroderma mean

A
  • hardening of the skin
  • shiny, tight skin without the normal folds in the skin.
  • most notable on the hands and face
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29
Q

What is Systemic sclerosis/scerloderma

A
  • autoimmune inflammatory and fibrotic connective tissue disease
  • Cause is unclear
  • affects the skin in all areas but it also affects the internal organs.
30
Q

What are two main patterns of disease in systemic sclerosis:

A

Limited cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis

31
Q

What is limited cutaeneuos systemic sclerosis/scleroderma

A
  • more limited version of systemic sclerosis

- It used to be called CREST syndrome.

32
Q

What does CREST stand for

A 
C – Calcinosis
R – Raynaud’s phenomenon
E – oEsophageal dysmotility
S – Sclerodactyly
T – Telangiectasia
33
Q

What id diffuse cutaneous systemic sclerosis/scleroderma

A

Diffuse cutaneous systemic sclerosis includes the features of CREST syndrome plus many internal organs causing:

  • Cardiovascular problems
  • Lung problems
  • Kidney problems
34
Q

What cardiovascular problems does diffuse cutaneous systemic sclerosis/scleroderma cause

A
  • HTN

- CAD

35
Q

What lung problems does diffuse cutaneous systemic sclerosis/scleroderma cause

A
  • pulmonary hypertension

- pulmonary fibrosis.

36
Q

What renal problems does diffuse cutaneous systemic sclerosis/scleroderma cause

A
  • glomerulonephritis

- scleroderma renal crisis.

37
Q

What is Sclerodactyly

A
  • As the skin tightens around joints in the hand restricting the ROM and function
  • As the skin hardens and tightens further the fat pads on the fingers are lost.
  • The skin can break and ulcerate.
38
Q

What are Telangiectasia

A

dilated small blood vessels in the skin. They are tiny veins that have dilated. They have a fine, thready appearance.

39
Q

What is calcinosis

A
  • calcium deposits build up under the skin

- most commonly found on the fingertips.

40
Q

What is Raynauds phenomenon

A
  • fingertips go completely white and then blue in response to even mild cold
  • caused by vasoconstriction of the vessels supplying the fingers.
  • commonly occurs without any associated systemic disease, however it is a classical feature of systemic sclerosis.
41
Q

What causes Oesophageal dysmotility

A
  • connective tissue dysfunction in the oesophagus

- commonly associated with swallowing difficulties, acid reflux and oesophagitis.

42
Q

What auto-antibodies should you check for if suspecting Systemic sclerosis/scerloderma

A
  • ANA
  • Anti-centromere antibodies
  • Anti-Scl-70 antibodies
43
Q

What is the management of systemic sclerosis

A
  • MDT
  • Steroids and immunosuppressants are usually started with diffuse disease and complications such as pulmonary fibrosis
  • Treatment of symptoms e.g. nifidipine for Raynauds
44
Q

What non-medical management is advised for systemic sclerosis

A
  • Avoid smoking
  • Gentle skin stretching to maintain the range of motion
  • Regular emollients
  • Avoiding cold triggers for Raynaud’s
  • Physiotherapy to maintain healthy joints
  • Occupational therapy for adaptations to daily living to cope with limitations
45
Q

What is polymyositis

A

An autoimmune condition of chronic inflammation of muscles.

46
Q

What is dermatomyositis

A

Autoimmune connective tissue disorder where there is chronic inflammation of the skin and muscles.

47
Q

What can causes a raised Createnine kinase

A
  • Polymyositis/dermatomyositis
  • Rhabdomyolysis
  • Acute kidney injury
  • Myocardial infarction
  • Statins
  • Strenuous exercise
48
Q

What investigations should you complete if suspecting dermatomyositis/polymyositis

A

Createnine kinase

  • <300 U/L. In polymyositis
  • > 1000U/L in dermatomyositis
49
Q

Polymyositis or dermatomyositis can be a result of paraneoplastic syndrome, which cancers is it most associated with

A

Lung
Breast
Ovarian
Gastric

50
Q

What is the presentation of dermatomyositis/polymyositis

A

Muscle pain, fatigue and weakness
Occurs bilaterally and typically affects the proximal muscles
Mostly affects the shoulder and pelvic girdle
Develops over weeks
(Polymyositis occurs without any skin feature)

51
Q

Dermatomyositis Skin Features

A

Gottron lesions

  • Photosensitive erythematous rash on the back, shoulders and neck
  • Purple rash on the face and eyelids
  • Periorbital oedema
  • Subcutaneous calcinosis (calcium deposits in the subcutaneous tissue)
52
Q

What are gottron lesions

A

(scary erythematous patches) on the knuckles, elbows and knees

53
Q

What autoantibodies should you check for if ? dermatomyositis/polymyositis

A

Anti-Jo-1 antibodies: polymyositis (but often present in dermatomyositis)
Anti-Mi-2 antibodies: dermatomyositis.
Anti-nuclear antibodies: dermatomyositis.

54
Q

How is dermatomyositis/polymyositis diagnosed

A 
Clinical presentation
Elevated creatine kinase
Autoantibodies
Electromyography (EMG)
Muscle biopsy can be used to establish a definitive diagnosis
55
Q

What is the management of dermatomyositis/polymyositis

A
  • Guided by rheumatologist
  • Assess for underlying cancer
  • Physiotherapy and OT
  • Corticosteroids first line
56
Q

When steroids are insufficient management for dermatomyositis/polymyositis what else can be considered

A

Immunosuppressants (such as azathioprine)
IV immunoglobulins
Biological therapy (such as infliximab or etanercept)

57
Q

What is Antiphospholipid syndrome

A
  • disorder associated with antiphospholipid antibodies where the blood becomes prone to clotting
  • patient is in a hyper-coagulable state
  • Can be primary or secondary associated with SLE
58
Q

Antiphospholipid syndrome is associated with which autoantibodies

A

Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies

59
Q

What is Anti-phospholipid syndrome associated with

A
  • VTE: DVT/PE
  • Arterial thrombosis: Stroke, MI, Renal thrombosis
  • Pregnancy complications
  • Livedo reticularis
  • Libmann-Sacks endocarditis
  • Thrombocytopenia (low platelets
60
Q

What pregnancy complications can you get with anti-phospholipid syndrome

A

Recurrent miscarriage
Stillbirth
Pre-eclampsia

61
Q

What is Livedo reticularis

A

purple lace like rash that gives a mottled appearance to the skin.

62
Q

what is Libmann-Sacks endocarditis

A
  • non-bacterial endocarditis where there are growths (vegetations) on the valves of the heart
  • mitral valve is most commonly affected
  • associated with SLE and antiphospholipid syndrome
63
Q

How is anti-phospholipid syndrome diagnosed

A

history of thrombosis or pregnancy complication plus persistent antibodies

64
Q

What is the management of anti-phospholipid syndrome

A
  • Managed by rheumatology, haematology and obstetrics (if pregnant).
  • Long term warfarin
  • Pregnant women: low molecular weight heparin (e.g. enoxaparin) plus aspirin
65
Q

What is the INR range in patients with antiphospholipid syndrome

A

2-3

66
Q

What is Felty’s syndrome

A
  • RA
  • Splenomegaly
  • Neutropaenia
67
Q

What is ankylosing spondylitis

A
  • arthritis mainly affecting the back caused by inflammation
  • the body produces extra calcium around the bones of the spine.
  • This can make extra bits of bone grow and cause your back and neck to be more stiff.
68
Q

Symptoms of Ankylosing Spondylitis

A
  • Back pain and stiffness (neck, shoulder, hip)
  • Can wake in night
  • Morning stiffness
  • Eases with exercise
69
Q

Typical epidemiology of ankylosing Spondylitis

A
  • males (sex ratio 3:1)

- 20-30 years old.

70
Q

Signs of progressive disease on x ray in ankylosing spondylitis

A
  • sacroiliitis: subchondral erosions, sclerosis
  • squaring of lumbar vertebrae
  • ‘bamboo spine’ (late & uncommon)
  • syndesmophytes: due to ossification of outer fibers of annulus fibrosus
71
Q

What impact does ankylosing spondylitis have on the lung

A
  • Apical fibrosis

- restrictive spirometry: pulmonary fibrosis, kyphosis and ankylosis of the costovertebral joints

72
Q

Management of ankylosing spondylitis

A
  1. NSAIDs + paracetamol
  2. Encourage exercise and swimming
  3. DMARDs: sulfasalazine if peripheral joint involvement
    (Anti-TNF)

Rheumatology (5 decks)

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