Multi-system Autoimmune Diseases

Question 1

What is the prevalence and incidence for systemic lupus erythematosus (SLE) in the UK?

Answer 1

• 28/100,000
• 4/100,000

Question 2

What are the risk factors for systemic lupus erythematosus (SLE)?

Answer 2

• F:M = 9:1
• 15-50 yrs
• afro-caribbbeans > asians > caucasians

Question 3

What is the classification criteria for systemic lupus erythematosus (SLE)?

Answer 3

Any 4 of:

• malar rash (butterfly rash)
• discoid rash (raised, scar, permanent marks, alopecia)
• photosensitivity
• oral ulcers
• arthritis (> 1 joint)
• serositis (pleurisy/pericarditis)
• renal (proteinurea, cellular casts)
• neurological (seizures, psychosis)
• haematological (low WCC, platelets, lymphocytes, haemolytic anaemia)
• immunological (anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement)
• anti-nuclear antibody (ANA)

Question 4

What is the prevalence and incidence for scleroderma in the UK?

Answer 4

• 24/100,000
• 10/1,000,000

Question 5

What are the risk factors for scleroderma?

Answer 5

• F:M = 3:1
• 30-50 yrs

Question 6

What are the different forms of scleroderma?

Answer 6

• morphea
• hardened patches of skin
• limited
• Calcinosis
• Raynaud’s phenomenom
• Esophageal dismotility
• Sclerodactyly
• Telangiectasia
• diffuse
• larger areas of hardened skin

Question 7

What are the complications of scleroderma?

Answer 7

Limited:

• pulmonary hypertension

Diffuse:

• pulmonary fibrosis
• renal crisis
• small bowel bacterial overgrowth

Question 8

What is the prevalence and incidence of Sjogrens syndrome?

Answer 8

• 1/100
• 4/100,000

Question 9

What are the risk factors for Sjogrens sydrome?

Answer 9

• F:M = 9:1
• 40-50 yrs

Question 10

What are the symptoms of Sjogrens syndrome?

Answer 10

• dry eyes + mouth
• parotid gland enlargement
• fatigue
• fever
• myalgia
• arthalgia

Question 11

What are the complications of Sjogrens syndrome?

Answer 11

• lymphoma
• neuropathy
• purpura
• interstitial lung disease
• renal tubular acidosis

Question 12

What is the incidence of autoimmune myositis?

Answer 12

• 6/1,000,000 (rare)

Question 13

What are the symptoms of autoimmune myositis?

Answer 13

• muscle weakness (symmetrical, diffuse, proximal)
• polymyositis
• dermatomyositis
• Gottrons papules
• heliotrope rash

Question 14

What are the complications of autoimmune myositis?

Answer 14

• cancer
• interstitial lung disease

Question 15

What are the different types of ANCA associated vasculitis and their prevalence and incidence?

Answer 15

• granulmatosis with polyangiitis (Wegener’s)
• microscopic polyangiitis
• eosiniphilic granulomatosis with polyangiitis
• 150/1,000,000
• 15/1,000,000

Question 16

What are the signs of granulomatosis with polyangiitis (GPA) (Wegener’s)?

Answer 16

• necrotising granulomatous inflammation
• upper + lower respiratory tract
• small to medium vessels
• necrotising glomerulonephritis

Question 17

What are the signs of microscopic polyangiitis (MPA)?

Answer 17

• necrotising vasculitis (few/no immune deposits), small vessels
• necrotising arteritis, small to medium arteries
• necrotising glomerulonephritis
• pulmonary capillaritis
• granulatomatous inflammation absent

Question 18

What are the signs of eosinophilic granulomatosis with polyangiitis (EGPA)?

Answer 18

• necrotising granulomatous inflammation (eosinophil rich), respiratory tract
• necrotising vasculitis, small to medium vessels
• associated with asthma and eosinophilia

* more frequent when glomerulonephritis present

Question 19

What is the management for organ threat, in multisystem autoimmune diseases?

Answer 19

Mild:

• hydroxychloroquine

Moderate:

• azathioprine
• methotrexate
• mycophenolate

Severe:

• cyclophosphamide
• rituximab