Connective tissue diseases

Question 1

Examples of connective tissue diseases

Answer 1

Systemic lupus erythematous (SLE)
Scleroderma
Sjögren’s syndrome
Rheumatoid arthritis (RA)
Undifferentiated connective tissue disease (UCTD)

Question 2

Large vessel disease

Answer 2

Inflammation of the aorta and its major branches.
- Giant cell arteritis/temporal arteritis

Question 3

Medium vessel disease

Answer 3

• Polyarteritis nodosa
• Kawasaki disease

Question 4

Small vessel disease

Answer 4

• Wegener’s granulomatosis

Question 5

Management of vasculitic diseases

Answer 5

No cure - cause is not clear.
NSAIDs - joint/muscle symptoms.
Immune modulating treatment - hydroxychloroquine, methotrexate, azathioprine, biologic medication.
Systemic steroids - prednisolone (not used long-term due to side effects).

Question 6

Features of lupus

Answer 6

Can have features of any of the following conditions + inflammatory organ disease
- scleroderma
- RA
- mixed connective tissue disease
- Raynaud’s phenomenon
- sjogren’s syndrome

Question 7

Antibodies commonly found in scleroderma

Answer 7

Anti-centromere antibody
Anti-Scl-70 antibody
Anti-neutrophil cytoplasmic antibody

Question 8

Antibodies commonly found in connective tissue diseases

Answer 8

Anti-nuclear antibody
Anti-ds DNA
Anti-Ro
Anti-La

Question 9

Discoid lupus

Answer 9

Tissue changes without blood autoantibodies.
It does not affect internal organs.

Question 10

Features of systemic lupus erythematous

Answer 10

Pleural effusions
Heart problems
Lupus nephritis
Arthritis
Raynaud’s phenomenon
Photosensitive ‘butterfly’ rash (across face)

Question 11

Dental aspects of SLE

Answer 11

Chronic anaemia - oral ulceration, GA risk
Bleeding tendency - thrombocytopenia
Impaired drug metabolism - Renal disease
Drug interactions can trigger photosensitivity
Steroid and immunosuppressive therapy - possible increased risk of malignancy.
Lichenoid oral reactions.
Oral pigmentation - hydroxychloroquine use.

Question 12

Antiphospholipid syndrome

Answer 12

Autoimmune condition (mostly affects young women) which causes blood clotting due to presence of antiphospholipid autoantibodies.
More common in people with lupus (known as lupus anticoagulant).
Thrombophilic - tendency to form DVT and pulmonary emboli without any other risk factors.
Treatment = warfarin

Question 13

Antibodies in anti-phospholipid syndrome

Answer 13

Anti-phospholipid (aPL)
Anti-cardiolipin (aCL)

Question 14

Sjogren’s disease

Answer 14

Inflammatory disease associated with circulating autoantibodies - ANA, Ro and La.
Mainly associated with dry eyes and mouth.

Question 15

Primary sjogrens

Answer 15

Not associated with any other disease

Question 16

Secondary sjogrens

Answer 16

Associated with another connective tissue disease - RA, SLE, etc.

Question 17

Dental implications of Sjögren’s syndrome

Answer 17

oral infection
caries
functional loss
poor denture retention
sialosis - swelling of salivary glands
salivary lymphoma

Question 18

Scleroderma

Answer 18

Excessive collagen deposition. Elastic tissue replaced by fibrous connective tissue.
Can be local or generalised.

Question 19

Localised scleroderma antibodies

Answer 19

anticentromere antibodies
(localised scleroderma affects the skin only)

Question 20

Generalised scleroderma antibodies

Answer 20

anti-Scl-70 antibodies

Question 21

Features of scleroderma

Answer 21

Gradual onset
Affects predominantly women
- Raynauds
- renal failure
- malabsorption (GI involvement)
- thickening of skin
- dysphagia

Question 22

Dental aspects of scleroderma

Answer 22

Limited mouth opening, limited tongue movement, progressively poor oral access - plan treatment ahead!
May also have Sjögren’s syndrome.
Dysphagia & reflux - swallowing difficulties and dental erosion possible.
Cardiac and renal vasculitic disease - drug metabolism
Widening of PDL space - due to changes in connective tissue constituents.

Question 23

Features of giant cell arteritis

Answer 23

Headache/facial pain.
Pain when eating.
Pain and tenderness over temples.
Vision problems - permanent blindness can occur if not treated asap - due to narrowing of central retinal artery.

Question 24

Treatment of giant cell arteritis

Answer 24

Medical emergency - needs urgent treatment
Prednisolone.

Question 25

Polymyalgia rheumatica

Answer 25

Mainly affects joints and muscles around shoulder and hip regions. Disease of the elderly. Pain and morning stiffness of muscles - restricted movement. Non-specific systemic features - malaise, weight loss, profound fatigue. Responds well to steroids.

Question 26

Kawasaki disease

Answer 26

Mainly a disease of children. clinically: - fever and lymphadenopathy - crusting/cracked tongue and lips - strawberry tongue and erythematous mucosa - peeling rash on hands and feet May need antibiotics.

Question 27

Wegener's granulomatosis

Answer 27

Can lead to destruction of hard and soft tissues of the face and oral cavity. Spongy red tissue. Associated with ANCA. Affects many systems - renal and respiratory tract most affected. Red rash over gingiva and palate = urgent referral for assessment!

Question 28

Fibromyalgia

Answer 28

Non-specific collection of musculoskeletal symptoms - joint pain - muscle pain - functional disorders Managed through pain management, not immunosuppressants.

Question 29

Which vasculitides may present to dentist?

Answer 29

Giant cell arthritis (pain when eating, painful temples, facial pain) Wegener's granulomatosis (red rash over gingiva and palate) Kawasaki disease (crusting/cracked tongue and lips, strawberry tongue)