T3 L6: Multisystem autoimmune disease

Question 1

What is Spondyloarthropathy (SpA)?

Answer 1

A family of inflammatory rheumatic diseases that have some key features in common, including: Inflammation, pain and stiffness in the spine and pelvic joints

Includes PEAR: Psoriatic arthritis, enteropathic arthritis, ankylosing spondylitis, reactive arthritis (Reiter’s syndrome)

Question 2

What is ankylosing spondylitis (AS)?

Answer 2

Chronic back pain caused by inflammation of the spine

Question 3

What are some characteristics of axial spondyloarthropathy?

Answer 3

• <40 years
• improvement with exercise
• No improvement with rest
• Pain at night

Question 4

What treatments are given for spondyloarthropathy?

Answer 4

• Physiotherapy and hydrotherapy
• NSAID’s
• Glucocorticoid injections into the joints
• DMARD’s
• Surgery

Question 5

What is vasculitis?

Answer 5

Immune-mediated inflammation of blood vessels

It can affect any organ or tissue

Question 6

What are some symptoms of vasculitis?

Answer 6

• Stroke
• MI
• Hypertension
• Bloody stool
• Abdominal pain
• Painful joints
• Palpable purpura
• Fever
• Headache
• Weight loss
• Bloody cough
• Nose bleeds

Question 7

What is giant cell arteritis?

Answer 7

Inflammation of external carotid artery branches

It’s a rheumatological emergency

Question 8

What are the symptoms of giant cell arteritis?

Answer 8

• Age >50
• Headaches
• Jaw or scalp tenderness
• New visual symptoms
• Raised CRP/ESR

Question 9

How is giant cell arteritis diagnosed?

Answer 9

Ultrasound or temporal artery biopsy

Question 10

What is the treatment for giant cell arteritis?

Answer 10

Hight dose of steroids over 12-18 months

Question 11

What is large cell vasculitis?

Answer 11

Inflammation of aorta and it’s proximal branches. There are reduced pulses

Often has systemic symptoms like fever, weight loss, fatigue

Question 12

What are some investigations for vasculitis?

Answer 12

• Raised CRP and ESR
• Autoantibodies
• Infections screening Eg. HIV, Hepatitis, TB
• Urine dip
• Abdominal/chest X-ray
• Tissue biopsy

Question 13

How is vasculitis managed?

Answer 13

• Initially steroids and related side effects are managed Eg. diabetes, osteoporosis
• Immunosuppressants
• Biological therapies
• DMARD’s

Question 14

What is Sjogren’s syndrome?

Answer 14

An autoimmune condition

causing degeneration of the salivary and lachrymal glands, causing dryness of the mouth and eyes

Question 15

What is systemic sclerosis?

Answer 15

a group of rare diseases that involve the hardening and tightening of the skin

Question 16

What are some examples of systemic sclerosis diseases?

Answer 16

Limited: calcinosis, Raynaud’s phenomenon, Oesophageal dysmotility, sclerodactyly, Telangiectasia

Diffuse: interstitial lung disease, myocardial disease, pulmonary hypertension, renal crisis, GI disease

Question 17

What is Calcinosis?

Answer 17

When calcium gathers in your skin, muscles, tendons, connective tissue, and subcutaneous tissue

Question 18

What is Oesophageal dysmotility?

Answer 18

When contractions in the Oesophagus become irregular, unsynchronized or absent

Question 19

What is sclerodactyly?

Answer 19

Skin tightening

Question 20

What is Telangiectasia?

Answer 20

Small, widened blood vessels on the skin

Question 21

What is idiopathic inflammatory myositis (IIM)

Answer 21

Proximal, symmetrical, painless muscle weakness. 20% overlap with other autoimmune diseases

Eg. Polymyositis and Dermatomysitis (muscle inflammation and skin rashes)

Question 22

What are some presentations of dermatomyositis?

Answer 22

• Peri-orbital heliotrope rash
• Gottron’s papules
• Nailfold
• Joint inflammation
• Muscle weakness
• Lung fibrosis
• Associated with malignancy

Question 23

What is Peri-orbital heliotrope rash?

Answer 23

A discoloration of the eyelids associated with periorbital oedema

Question 24

What are Gottron’s papules?

Answer 24

Inflamed bumps most commonly in the bony projections of the hand