Autoimmune Diseases Flashcards

1
Q

List connective tissue diseases

A
Systemic Lupus Erythematosus
Scleroderma
Sjogren’s syndrome
Auto-immune myositis
Mixed connective tissue disease
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2
Q

List systemic vasculitis diseases

A
  • Giant cell arteritis
  • Granulomatosis polyangitis
  • Microscopic polyangitis
  • Eosinophilic granulomatosis polyangitis
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3
Q

How can autoimmune diseases be diagnosed

A

History and examination, immunology tests, imaging, tissue samples

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4
Q

State the drugs that can cause similar symptoms to that of autoimmune disease

A

Cocaine (can cause microvasculitis), minocycline (drug induced systemic lupus), Propylthiouracil (used to treat hyperthyroidism)

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5
Q

Systemic lupus tends to effect what age group

A

The young, 50% diagnosed at around 16-50

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6
Q

Prevalence of systemic lupus

A

28/100,000

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7
Q

Incidence of systemic lupus

A

4/100,000

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8
Q

Female to male ration systemic lupus

A

9:1

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9
Q

Systemic lupus is more common in what race

A

Caribbean afro

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10
Q

Face symptoms of systemic lupus

A

Butterfly rash and photo-sensitivity

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11
Q

Nervous system features of systemic lupus

A

Seizures, hemiplegia, ataxia, peripheral neuropathy, cranial nerve lesions

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12
Q

Cardiac features of lupus

A

Pericarditis, endocarditis, aortic valve lesions

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13
Q

Blood features of lupus

A

Anaemia (nomochromic normocytic Coombs test +), leukopenia, thrombocytopenia

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14
Q

Joint features of lupis

A

Aseptic necrosis of the hip, arthritis in small joints

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15
Q

Chest features of lupus

A

Pleurisy, pleural effusion, fibrosis

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16
Q

Skin changes in lupus

A

Vasculitis, purpura, urticaria

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17
Q

General features of lupus

A

Fever, depression, alopecia, abdominal pain, myopathy, raynauds phenomenon

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18
Q

To be diagnosed with systemic lupus, a patient must have 4 of:

A
  1. Acute Cutaneous lupus - malar rash, photosensitivity rash or maculopapular rash
  2. Chronic Cutaneous lupus - Discoid rash
  3. Non-scarring alopecia
  4. Oral or nasal ulcers
  5. Synovitis occuring in two or more joints
  6. Serositis - lung pleurisy, pleural effusions or pleural rub, or pericardial pain, pericardial effusion or pericarditis of ECG
  7. Urinalysis showing proteinuria or red cell casts
  8. Neurological features - seizures, psychosis, myelitis, neuropathy, confusional state
  9. Haemolytic anaemia
  10. Leucopenia (WCC<4) or lymphocytes <1
  11. Thrombocytopenia (platelets <100)
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19
Q

Why is it important to do urinalysis in Lupus

A

Glomerulonephritis can develop

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20
Q

Scleroderma

A

Spectrum of conditions linked by thickened sclerotic skin lesions (subcutaneous fibrosis), internal organ fibrosis and microvascular abnormalities

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21
Q

Prevelance of scleroderma

A

24/100,000

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22
Q

incidence of scleroderma

A

10/1,000,000

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23
Q

Age of onset scleroderma

A

30-50 years of age ,tends to be females

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24
Q

Local disease scleroderma

A

Morphea rash

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25
Q

Limited scleroderma disease

A

Involves the face, hands and feet. It is associated with anticentromere antibodies in 70-80%. Pulmonary hypertension often present sub-clinically.

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26
Q

Diffuse scleroderma

A

This can involve the whole body. Tends to involve SCL-70 antibodies (40%) and anti-RNA polymerase in 20%

27
Q

Action taken when scleroderma is diffuse

A

Control BP, perform annual ECG and spirometry

28
Q

Management of scleroderma

A

Immunosuppressive regimens IV cyclophosphamide, monitor BP and renal function, use regular ACE-i or A2RBS

29
Q

Immunology of systemic lupus

A

95% ANA +ve, ENA + in 20-30%, anti-DSDNA highly specific but only present in 60% of patients

30
Q

How can systemic lupus be diagnosed

A

> 4 criteria or biopsy-proven lupus nephritis with positive ANA or anti-DNA

31
Q

List the drugs that can cause drug-induced lupus

A

isoniazid, hydralaxine, quinidine, procainamide, phenytoin, anti-TNF agents

32
Q

General measures in the management of lupus

A

High factor sunblock, hydroxychloroquine, topical steroids for skin lesions

33
Q

Drugs used in the maintenance of lupus

A

NSAIDS,

hydroxychloroquine (for joint and skin lesions),

Azathioprine, methotrexate and mycophenolate as steroid-sparing agents

Belimumab used as an add-on therapy for autoantibody positive disease where disease activity is high

34
Q

Treatment of mild flares of lupus

A

Hydroxychloroquine or low dose steroids

35
Q

Treatment of moderate flares of lupus

A

DMARDs or mycophenolate

36
Q

Severe flare of lupus

A

haemolytic anaemia, nephritis, severe pericarditis, CNS disease

37
Q

How is a severe flare up of lupus treated

A

Urgent high dose steroids, mycophenolate, rituximabd, cyclophosphamide

38
Q

Prognosis of lupus

A

80% survival at 15 years

39
Q

Complications of diffuse scleroderma

A

Pulmonary fibrosis, renal crisis, small bowel bacterial overgrowth

40
Q

Sjogrens syndrome

A

A chronic inflammatory autoimmune disorder associated with connective tissue disease

41
Q

Prevelance of sjogrens syndrome

A

1:100

42
Q

Incidence of sjogrens syndrome

A

4 in 100,000

43
Q

Age of onset of sjogrens

A

40-50 years

44
Q

Female:male sjogrens

A

9:1

45
Q

Pathology of sjogrens

A

Lymphocytic infiltration and fibrosis of exocrine glands especially lacrimal and salivary

46
Q

Features of sjogrens syndrome

A

Decreased tear production resulting in dry eyes, keratoconjunctivitis sicca), decreased salvation, parotid swelling, vaginal dryness, dypareunia, dry cough, dysphagia

47
Q

Systemic features of sjogrens

A

polyarteritis, arthalgia, rynauds, lymphadenopathy, vasculitis, lung, liver and kidney involvement, peripheral neuropathy, myositis and fatigue

48
Q

Complications of sjogrens

A

Lymphomay, neuropathy, interstitial lung disease, renal tubular acidosis

49
Q

Diagnosis of sjogrens

A

Schirmers test, +ANA (74%), rheumatoid factor is +ve in 38% , biopsy shows focal lymphocytic aggregation

50
Q

Management of sjogrens

A

Treat sicca with hypromelose, frequent drinks, sugar free pastilles and gum

NSAIDs and hydroxychloroquine for arthralgia

51
Q

Prevelance of autoimmune myositis

A

6 per million

52
Q

Presentation of autoimmune myositis

A

Symmetrical, diffuse muscle weakness. There may be polymyositis, dermatomyositis

53
Q

Diagnosis of myositis

A

Muscle enzymes (ALT, AST, LDH, CK) all increased in plasma

EMG shows characteristic fibrillation potential

Muscle bopshy

54
Q

Management of myositis

A

Prednisolone, immunosuppressives and cytotoxics. Hydroxychloroquine for skin disease

55
Q

Large vasculitis

A

Giant cell arteritis, takayasus arteritis

56
Q

Medium vasculitis

A

Polyarteritis nodosa, kawasaki disease

57
Q

Small vasculitis

A

ANCA associated: microscopic polyangiitis, granulomatosis with polyangitis, eosinophilic granulomatosis with polyangitis

Immune complex: good pastures disease, cyroglobulinaemic vasculitis , IgA vasculitis

58
Q

Giant Cell Arteritis symptoms

A

Headache, temporal artery and scalp tenderness, tongue and jaw claudication, amaurosis fugax, sudden unilateral blindness

59
Q

Diagnosis of Giant Cell Arteritis

A

Increase ESR (above 50) and CRP, increased platelets, increased ALP

Temporal artery biopsy within 14 days of starting steroids

60
Q

Management of GCA

A

Prednisolone or IV methylprednisilone (if involving vision loss)

61
Q

Microscopic polyangitis symptoms

A

Rapidly progressive glomerulonephritis usually featuring pulmonary haemorrhage

62
Q

Diagnosis of microscopic polyangitis

A

pANCA +

63
Q

Treatment of microscopic polyangitis

A

steroids plus methotrexate