Multisystem autoimmune disease Flashcards

1
Q

Types of Autoimmune Multisystem Diseases

A

Connective tissue disease

Systemic vasculitis

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2
Q

Examples of connective tissue disease

A
SLE
Scleroderma
Sjogren's
Autoimmune Myositis
Mixed connective disease
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3
Q

Examples of systemic vasculitides

A

Giant cell arteritis
Granulomatosis with polyangiitis/Wegener’s Syndrome
Microscopic polyangiitis
Eosinophilic granulomatosis with polyangiitis/Churg - Strauss syndrome

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4
Q

Diagnosis of autoimmune multisystem disease

A

(hot, red, swelling, painful)
Oral mucosa and ocular changes
Anti-nuclear Antibody test (ANA)
PET and Doppler scan, CXR for lung changes

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5
Q

What is SLE?

A

A chronic AI disease affecting any organ system.
+ve ANA test
+ve Anti double strand DNA antibody
Dysregulated immune pathways
Related to environmental insult in genetically susceptible patient

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6
Q

Outcomes for SLE?

A

Constitutional symptoms - skin and joint involvement
Recurrent flare ups lead to progressive disease
Early diagnosis and management lowers this risk

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7
Q

Treatment for SLE?

Mild, Moderate, Severe

A

Mild: Hydroxychloroquine
Moderate: DMARD and immunosuppression
- methotrexate and azathioprine when showing serositis/arthritis
- mycophenolate for renal involvement
Severe (neurological symptoms): Cyclophosphamide or rituximab

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8
Q

Presentation of SLE?

A
  • Malar or discoid rash + Oral ulcers
  • Polyarthritis, Serositis (pleural/pericardial inflammation +/- effusion)
  • Seizures/psychosis
  • Photosensitivity
  • Raynaud’s
  • Low WBC, thrombocytopenia, lymphocytopenia, haemolytic anaemia, Proteinuria
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9
Q

Investigations for suspected lupus?

A

FBC: neutropenia, thrombocytopenia, normochromaic/hypochromic normocytic anaemia (anaemia of chronic disease or haemolytic)
ESR raised, normal CRP
Renal function, Us+Es, Dipstick, ANA, ANCA, Anti-ds-DNA antibody (vasculitis)

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10
Q

Signs of general vasculitis?

A

Hand lesions, Splinter haemorrhages, Nail bed infarcts

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11
Q

What is giant cell arteritis?

A

Vasculitis of large and medium vessels (cranial vessels arising from aortic arch)
Granulomatous inflammation association A.k.a. temporal arteritis

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12
Q

Presentation of Giant Cell Arteritis?

A

> 50 y/o
New localised headache
Temporal artery tenderness/reduced pulsation
Jaw claudication
Visual abnormalities
Abnormal temporal biopsy (granulomatous inflammation, multinucleate giant cells)
50 ESR

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13
Q

What do you see in Granulomatous with polyangiitis? (Wegener’s)

A

Necrotising granulomatous inflammation
Upper + Lower Respiratory tract small + medium vessels inflamed
Associated with ANCA
Often with necrotising glomerulonephritis

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14
Q

What is seen in microscopic polyangiitis?

A
  • NO granulomatous inflammation
  • Necrotising arteritis of small and medium arteries
  • Necrotising vasculitis of small vessels
    (Often with necrotising glomerulonephritis, KIDNEY)
  • Pulmonary small vessel inflammation (LUNGS)
  • Associated with ANCA
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15
Q

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

A
  • Necrotising granulomatous inflammation
  • Involves upper and lower respiratory tract (LUNGS)
  • High eosinophil levels (Associated with asthma )
  • ANCA (likely if glomerulonephritis is present)
  • Glomerulonephritis (KIDNEY)
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16
Q

What is ANCA and when is it seen?

A

Antineutrophil cytoplasmic antibodies

Seen in vasculitides of small vessels (Wegener’s, Churg-Strauss and microscopic polyangiitis)

17
Q

What is GCA associated with?

A

Polymyalgia rheumatica

18
Q

Presentation of polymyalgia rheumatica

A

Female, over 50 (most over 70)
Increased ESR and CRP
Bilateral shoulder, pelvic girdle and neck morning stiffness
Systemic: low grade fever, weight loss, fatigue, anaemia

19
Q

Vasculitides treatment?

A
  • Steroids
  • DMARDs
  • Biologics
    Same as lupus: hydroxychloroquine, methotrexate, AZT, cyclophosphamide
20
Q

Anaemia in autoimmune disease…

A

Either normochromic, normocytic anaemia due to haemolysis (therefore jaundice) OR hypochromic normocytic anaemia due to anaemia of chronic disease; inflammation stops body using stored iron, fewer RBC