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Musculoskeletal + Rheumatology > Multi-System Autoimmune Disease > Flashcards

Multi-System Autoimmune Disease Flashcards

1
Q

What is Systemic Erythematous Lupus (SLE)?

A

Chronic autoimmune disease due to type 3 hypersensitivity reaction, characterised by reddening of the skin and generalised symptoms affecting multiple organs, due to widespread vasculitis

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2
Q

What can trigger SLE?

A

Drug induced

  • Isoniazid
  • Phenytoin
  • Hydralazine
  • Procainamide

UV Radiation

Smoking

Oestrogen

Infection

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3
Q

What sex is more likely to be affected by SLE?

A

F>M

9:1

Oestrogen may be a trigger?

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4
Q

What is the most common age of onset of SLE?

A

15-50 years, teens into middle age

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5
Q

What ethnicity is more likely to be affected by SLE?

A

Afro-Caribbean’s > Asians > Caucasian

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6
Q

What is the criteria for SLE?

A

DOPAMINE RASH

Any 4 of the following

Discoid rash

  • Raised, scarring, permanent marks, alopecia, appearing after sun-exposure

Oral ulcers

Photosensitivity

  • Other rashes appearing after sun-exposure

Arthritis

  • 2 joints at least, usually small joints, symmetrical, similar to RA

Malar rash

  • ‘Butterfly rash’ over the cheeks, appearing after sun-exposure

Immunological

  • Anti-phospholipid antibody

Neurological

  • Unexplained seizures or psychosis

Renal

  • Significant proteinuria
  • Glomerulonephritis

Antinuclear Antibodies (ANA)

Serositis

  • Pleurisy
  • Pericarditis

Haematological

  • Low WCC
  • Low platelets
  • Haemolytic anaemia
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7
Q

What investigations are used in SLE diagnosis?

A

Antinuclear antibodies

  • Most sensitive but not specific, 99% are +
  • Suggests connective tissue disease but not which one

Anti-dsDNA and anti-smith antibodies

  • Highly specific for lupus

Anti-Ro and Anti-La antibodies

  • Not specific for lupus

Anti phospholipid antibodies

  • Suggests antiphospholipid syndrome present in 25% of patients

Anti-histone antibodies

  • In drug induced lupus

Low C3 and C4 complement during active disease, useful in monitoring flares

>ESR, raised CRP suggests underlying infection

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8
Q

What antibody test is used for screening of SLE/most sensitive?

A

Antinuclear Antibodies

Sensitive but not specific

If ANA is negative, there is very low chance of SLE, so is a useful rule out test

Lead onto more specific tests

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9
Q

What antibody test is diagnostic of SLE/most specific?

A

Anti-dsDNA

A specific type of ANA

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10
Q

How SLE managed?

A

Hydroxychloroquine, first line for mild

Other immunosuppresants/DMARDs

Biologics for severe disease

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11
Q

Give complications of SLE

A

Cardiovascular disease, leading cause of death

Infection

Anaemia of chronic disease

Pericarditis

Pleuritis

Pulmonary fibrosis

Lupus nephritis

VTE

Recurret miscarriage

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12
Q

What condition is antiphospholipid syndrome associated with?

A

SLE

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13
Q

How does antiphospholipid syndrome present?

A

Recurrent venous/arterial thrombosis

  • CVA
  • Budd-Chiari syndrome
  • DVT

Recurrent spontaneous miscarriage

Livedo reticularis

Purpura

Splinter haemorrhages

Valvular heart disease

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14
Q

What investigations are used in antiphospholipid syndrome diagnosis?

A

>APTT

FBC

  • Thrombocytopenia
  • Haemolytic anaemia

Anticardiolipin antibodies

Lupus Anticoagulant

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15
Q

How is antiphospholipid syndrome managed?

A

Daily low dose aspirin

Lifelong warfarin after initial VTE event

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16
Q

What is autoimmune dermatomyositis?

A

Inflammatory disease of skeletal muscle of unknown aetiology causing symmetrical, proximal muscle weakness and characteristic skin lesions

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17
Q

What conditions is autoimmune myositis associated with?

A

RA

Malignancy

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18
Q

How does myositis present?

A

Muscle weakness

  • Symmetrical
  • Diffuse
  • Proximal

Associated with malignancy, as can be a paraneoplastic syndrome

Dysphagia

Dyspnoea

Raynauds

Photosensitivity, specifically rash on eyelids

Dermatomyositis

  • Gottron’s papules, erythematous scaly lesions affecting the dorsum of the hands
  • Heliotrope rash
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19
Q

What investigations are used in myositis diagnosis?

A

>CK

Antibodies

  • ANA
  • Anti-Jo-1 Antibody

Muscle biopsy

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20
Q

How is myositis managed?

A

High dose glucocorticoids

Azathioprine or Methotrexate

Immunoglobulin

Physiotherapy

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21
Q

Give complications of myositis

A

Malignancy

Interstitial lung disease

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22
Q

What is Sjogren’s Syndrome?

A

Chronic autoimmune condition in which the immune system attacks various exocrine glands, causing fibrosis

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23
Q

What causes Sjogren’s Syndrome?

A

Primary

Secondary to other connective tissue disease

  • Scleroderma
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24
Q

What sex is more likely to be affected by Sjogren’s?

A

F>M

9:1

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25
Q

What is the age of onset of Sjogren’s?

A

Middle age

40-50

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26
Q

How does Sjogren’s present?

A

Dry eyes

Dry mouth

  • Unable to eat certian foods such as bread

Oral pain

Parotid gland enlargement

Vaginal dryness

Systemic upset

  • Fatigue
  • Fever
  • Myalgia
  • Arthralgia
  • Arthritis
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27
Q

What investigations are used in Sjogren’s diagnosis?

A

>ESR

Antibodies

  • Anti-Ro and Anti-la, most specific
  • ANA
  • RF

Schrimer’s Test

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28
Q

What is Schrimer’s Test?

A

Uses filter paper under the lower eyelid to measure tear production

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29
Q

How is Sjogren’s managed?

A

Artificial tears/eye drops

Mouthwash

Diet changes

30
Q

Give complications of Sjogrens

A

Lymphoid malignancy/lymphoma

Renal tubular acidosis

31
Q

What is polymyalgia rheumatica?

A

Chronic inflammatory condition of the elderly characterised by joint stiffness and pain in surrounding tissues along with weakness of proximal muscles

32
Q

What age group does polymyalgia rheumatica affect?

A

Elderly

33
Q

What sex is more likely to be affected by polymyalgia rheumatica?

A

F>M

2:1

34
Q

What causes polymalgia rheumatica?

A

Genetic

  • HLA-DR4

Associated conditions

  • Giant Cell Arteritis
35
Q

How does polymyalgia rheumatica present?

A

Sudden onset of shoulder and pelvic girdle stiffness

  • Starts on one side but progresses to other side within weeks
  • At least 45 minutes in morning

Pain

  • Worsens with movement
  • Disturbs sleep

Anaemia

Malaise

Weight loss

Fever

Depression

No true weakness of limb girdles

36
Q

What investigations are used in polymyalgia rheumatica diagnosis?

A

Usually a clinical diagnosis, investigatons to rule out differentials

>ESR and >CRP

FBC

  • Normocytic normochromic anaemia

Creatine Kinase normal, unlike conditions that affect muscle

37
Q

How is polymyalgia rheumatica managed?

A

Corticosteroids

  • Initially started on 15mg/day prednisolone
  • Begin to reduce after 3-4 weeks
38
Q

What is giant cell arteritis?

A

Giant cell granulomatous inflammation of the arteries of the face and scalp, causing narrowing which can lead to infarction of the brain and optic nerve

39
Q

What is the classification criteria for giant cell arteritis?

A

3 of the following

Age at onset > 50 years

New headache

  • Diffuse
  • Persistent
  • Severe

Temporal artery tenderness/reduced pulsation

ESR > 50 (>CRP)

Abnormal temporal biopsy

Jaw claudication, particularly when chewing

Fever

40
Q

What investigations are used in giant cell arteritis diagnosis?

A

>CRP

Greatly raised ESR

FBC

  • Normochromic normocytic anaemia

Superficial temporal biopsy

  • Skip lesions
41
Q

How is giant cell arteritis managed?

A

High dose 60mg prednisolone, continue even if biopsy returns as normal due to skip lesions

Bisphosphonates, for risk of osteoporosis

Urgent opthalmology review

IV methylprednisilone if blindness has occurred

Note, if co-existing infection steroid dose should be maintained or decreased

42
Q

Give complications of giant cell arteritis

A

Blindness, due to ischaemic optic neuritis

Stroke

Aortic aneurysm

Heavily associated with polymyalgia rheumatica

43
Q

What is Scleroderma/systemic sclerosis?

A

Chronic autoimmune condition characterised by over-production of collagen resulting in hardened sclerotic skin and other connective tissues

44
Q

What sex is more likely to be affected by Scleroderma?

A

F>M

3:1

45
Q

Describe limited cutanenous scleroderma

A

Raynaud’s may be first sign

Scleroderma affects face and distal limbs predominately

Associated with anti-centromere antibodies

Subtype of limited systemic sclerosis is CREST syndrome

Associated with pulmonary hypertension

46
Q

Describe diffuse cutaneous scleroderma

A

Scleroderma affects trunk and proximal limbs predominately

Associated with scl-70 antibodies

Most common cause of death is now respiratory involvement (interstitial lung disease and pulmonary arterial hypertension)

Other complications include renal disease and hypertension

Poor prognosis

47
Q

What investigations are used in scleroderma diagnosis?

A

Antibodies

  • ANA
  • Scl-70 antibodies for diffuse
  • Anti-centromete antibodies for central/limited
48
Q

How is scleroderma managed?

A

Preventative

  • Handwarmers and battery powered gloves

Raynaud’s

  • CCBs
  • Vasodilators

Acid reflux

  • Omeprazole

Arthralgia

  • Hydroxychloroquine/DMARDS
49
Q

What is CREST syndrome?

A

Calcinosis

Raynaud’s

Oesophageal dysmotility/acid reflux

Sclerodactyly

Telangiectasia/spider naevi

Associated with anti-centromere antibodies

50
Q

What type of scleroderma is CREST syndrome associated with?

A

Limited scleroderma

51
Q

What is Wegner’s Granulomatosis?

A

Systemic vascular disease characterised by necrotizing granulomas in the respiratory tract and focal necrotizing glomerulonephritis

52
Q

How does Wegner’s present?

A

Cough

Pleurisy

SOB

Haemoptysis

General malaise

Haematuria

Arthralgia

Purulent/bloody nasal discharge

Purpura

Oral ulceration

Pleural effusion

Conjunctivitis

53
Q

What investigations are used in Wegners diagnosis?

A

cANCA antibodies

FBC

  • Leucocytosis
  • Thrombocytosis

Blood Film

  • Schistocytes
  • Burr cells

Urine

  • Proteinuria
  • Haematuria
  • Red cell casts

Imaging

Biopsy: Granulomatous changes

54
Q

How is Wegners managed?

A

Immunosuppressants

Corticosteroids

55
Q

What is Takayasu’s Arteritis?

A

Chronic, progressive, inflammatory, occlusive disease of the aorta and its branches

56
Q

What group of people are more likely to be affected by Takayasus?

A

Young females

57
Q

How does Takayasus present?

A

Systemic illness

Tenderness over palpable arteries

Bruits

Loss of pulses

Claudication

58
Q

How is Takayasus managed?

A

Steroids and immunosuppressants

Angioplasty/surgical repair for stenosed vessels

59
Q

What is Raynaud’s Syndrome?

A

Phenomenon characterised by spasm of arteries causing episodes of reduced blood flow to, typically, the fingers, resulting in colour change, pain and parathesia

60
Q

What causes Raynaud’s syndrome?

A

Primary/Idiopathic

Secondary to connective tissue disease

  • Scleroderma
  • Mixed connective tissue disease
  • Lupus
  • Sjogren’s

Secondary to other

  • Myeloma/paraproteinemia
  • Leukaemia
  • Cervical rib

Drugs

  • COCP
61
Q

What triggers episodes of Raynaud’s syndrome?

A

Cold

Stress

62
Q

How does Raynaud’s present?

A

Typically young women with bilateral symptoms if primary Raynauds

Pain

Colour change, typically red to white to blue

Parathesia

63
Q

How is Raynaud’s managed?

A

CCBs, such as nifedipine

IV prostacyclin (epoprostenol) infusions, effects may last several weeks/months

64
Q

What is fibromyalgia?

A

Syndrome of unknown cause characterised by widespread pain throughout the body with tender points at specific anatomical sites

65
Q

What sex is fibromyalgia most common in?

A

F>M

66
Q

Give features of fibromyalgia

A

Chronic pain at multiple site, sometimes ‘pain all over’

Lethargy

Cognitive impairment, known as ‘fibro fog’

Sleep disturbance

Headaches

Dizziness

67
Q

How is fibromyalgia managed?

A

Aerobic exercise

CBT

Medication

  • Pregabalin
  • Duloxetine
  • Amitriptyline
68
Q

What ANCA associated vasculitis is associated with pANCA?

A

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

69
Q

What ANCA associated vasculitis is associated with cANCA?

A

Granulomatosis with polyangiitis

70
Q

Give features of ANCA associated vasculitis

A

Renal impairment, haematuria

Dyspnoea, haemoptysiis

Fatigue

Weight loss

Fever

Vasculitic rash. present only in a minority of patients

ENT symptoms

Sinusitis

71
Q

What is Behcet syndrome?

A

Autoimmune inflammation of arteries and veins characterised by the triad of oral ulcers, genital ulcer and anterior uveitis

72
Q

How does Behcet syndrome present?

A

Triad of oral ulcers, genital ulcers and anterior uveitis

DVT and phlebitis

Arthritis

Erythema nodosum

Abdominal pain and diarrhoea

Acute pericarditis

Musculoskeletal + Rheumatology (16 decks)

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