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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
What is the age of onset of Sjogren's?
Middle age 40-50
26
How does Sjogren's present?
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
27
What investigations are used in Sjogren's diagnosis?
\>ESR Antibodies * **Anti-Ro and Anti-la**, most specific * ANA * RF Schrimer's Test
28
What is Schrimer's Test?
Uses filter paper under the lower eyelid to measure tear production
29
How is Sjogren's managed?
Artificial tears/eye drops Mouthwash Diet changes
30
Give complications of Sjogrens
**Lymphoid malignancy/lymphoma** Renal tubular acidosis
31
What is polymyalgia rheumatica?
Chronic inflammatory condition of the elderly characterised by joint stiffness and pain in surrounding tissues along with weakness of proximal muscles
32
What age group does polymyalgia rheumatica affect?
Elderly
33
What sex is more likely to be affected by polymyalgia rheumatica?
F\>M 2:1
34
What causes polymalgia rheumatica?
Genetic * HLA-DR4 Associated conditions * Giant Cell Arteritis
35
How does polymyalgia rheumatica present?
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
What investigations are used in polymyalgia rheumatica diagnosis?
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
How is polymyalgia rheumatica managed?
Corticosteroids * Initially started on 15mg/day prednisolone * Begin to reduce after 3-4 weeks
38
What is giant cell arteritis?
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
What is the classification criteria for giant cell arteritis?
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
What investigations are used in giant cell arteritis diagnosis?
\>CRP Greatly raised ESR FBC * Normochromic normocytic anaemia Superficial temporal biopsy * Skip lesions
41
How is giant cell arteritis managed?
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
Give complications of giant cell arteritis
Blindness, due to ischaemic optic neuritis Stroke Aortic aneurysm Heavily associated with polymyalgia rheumatica
43
What is Scleroderma/systemic sclerosis?
Chronic autoimmune condition characterised by over-production of collagen resulting in hardened sclerotic skin and other connective tissues
44
What sex is more likely to be affected by Scleroderma?
F\>M 3:1
45
Describe limited cutanenous scleroderma
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
Describe diffuse cutaneous scleroderma
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
What investigations are used in scleroderma diagnosis?
Antibodies * ANA * **Scl-70 antibodies** for diffuse * **Anti-centromete antibodies** for central/limited
48
How is scleroderma managed?
Preventative * Handwarmers and battery powered gloves Raynaud's * CCBs * Vasodilators Acid reflux * Omeprazole Arthralgia * Hydroxychloroquine/DMARDS
49
What is CREST syndrome?
Calcinosis Raynaud's Oesophageal dysmotility/acid reflux Sclerodactyly Telangiectasia/spider naevi Associated with **anti-centromere antibodies**
50
What type of scleroderma is CREST syndrome associated with?
Limited scleroderma
51
What is Wegner's Granulomatosis?
Systemic vascular disease characterised by necrotizing granulomas in the respiratory tract and focal necrotizing glomerulonephritis
52
How does Wegner's present?
Cough Pleurisy SOB Haemoptysis General malaise Haematuria Arthralgia Purulent/bloody nasal discharge Purpura Oral ulceration Pleural effusion Conjunctivitis
53
What investigations are used in Wegners diagnosis?
**cANCA antibodies** FBC * Leucocytosis * Thrombocytosis Blood Film * Schistocytes * Burr cells Urine * Proteinuria * Haematuria * Red cell casts Imaging Biopsy: Granulomatous changes
54
How is Wegners managed?
Immunosuppressants Corticosteroids
55
What is Takayasu's Arteritis?
Chronic, progressive, inflammatory, occlusive disease of the aorta and its branches
56
What group of people are more likely to be affected by Takayasus?
Young females
57
How does Takayasus present?
Systemic illness Tenderness over palpable arteries Bruits Loss of pulses Claudication
58
How is Takayasus managed?
Steroids and immunosuppressants Angioplasty/surgical repair for stenosed vessels
59
What is Raynaud's Syndrome?
Phenomenon characterised by spasm of arteries causing episodes of reduced blood flow to, typically, the fingers, resulting in colour change, pain and parathesia
60
What causes Raynaud's syndrome?
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
What triggers episodes of Raynaud's syndrome?
Cold Stress
62
How does Raynaud's present?
Typically young women with bilateral symptoms if primary Raynauds Pain Colour change, typically red to white to blue Parathesia
63
How is Raynaud's managed?
CCBs, such as nifedipine IV prostacyclin (epoprostenol) infusions, effects may last several weeks/months
64
What is fibromyalgia?
Syndrome of unknown cause characterised by widespread pain throughout the body with tender points at specific anatomical sites
65
What sex is fibromyalgia most common in?
F\>M
66
Give features of fibromyalgia
Chronic pain at multiple site, sometimes 'pain all over' Lethargy Cognitive impairment, known as 'fibro fog' Sleep disturbance Headaches Dizziness
67
How is fibromyalgia managed?
Aerobic exercise CBT Medication * Pregabalin * Duloxetine * Amitriptyline
68
What ANCA associated vasculitis is associated with pANCA?
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
69
What ANCA associated vasculitis is associated with cANCA?
Granulomatosis with polyangiitis
70
Give features of ANCA associated vasculitis
Renal impairment, haematuria Dyspnoea, haemoptysiis Fatigue Weight loss Fever Vasculitic rash. present only in a minority of patients ENT symptoms Sinusitis
71
What is Behcet syndrome?
Autoimmune inflammation of arteries and veins characterised by the triad of oral ulcers, genital ulcer and anterior uveitis
72
How does Behcet syndrome present?
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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