Connective tissue diseases Flashcards
Examples of connective tissue diseases
Systemic lupus erythematous (SLE)
Scleroderma
Sjögren’s syndrome
Rheumatoid arthritis (RA)
Undifferentiated connective tissue disease (UCTD)
Large vessel disease
Inflammation of the aorta and its major branches.
- Giant cell arteritis/temporal arteritis
Medium vessel disease
- Polyarteritis nodosa
- Kawasaki disease
Small vessel disease
- Wegener’s granulomatosis
Management of vasculitic diseases
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).
Features of lupus
Can have features of any of the following conditions + inflammatory organ disease
- scleroderma
- RA
- mixed connective tissue disease
- Raynaud’s phenomenon
- sjogren’s syndrome
Antibodies commonly found in scleroderma
Anti-centromere antibody
Anti-Scl-70 antibody
Anti-neutrophil cytoplasmic antibody
Antibodies commonly found in connective tissue diseases
Anti-nuclear antibody
Anti-ds DNA
Anti-Ro
Anti-La
Discoid lupus
Tissue changes without blood autoantibodies.
It does not affect internal organs.
Features of systemic lupus erythematous
Pleural effusions
Heart problems
Lupus nephritis
Arthritis
Raynaud’s phenomenon
Photosensitive ‘butterfly’ rash (across face)
Dental aspects of SLE
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.
Antiphospholipid syndrome
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
Antibodies in anti-phospholipid syndrome
Anti-phospholipid (aPL)
Anti-cardiolipin (aCL)
Sjogren’s disease
Inflammatory disease associated with circulating autoantibodies - ANA, Ro and La.
Mainly associated with dry eyes and mouth.
Primary sjogrens
Not associated with any other disease
Secondary sjogrens
Associated with another connective tissue disease - RA, SLE, etc.
Dental implications of Sjögren’s syndrome
oral infection
caries
functional loss
poor denture retention
sialosis - swelling of salivary glands
salivary lymphoma
Scleroderma
Excessive collagen deposition. Elastic tissue replaced by fibrous connective tissue.
Can be local or generalised.
Localised scleroderma antibodies
anticentromere antibodies
(localised scleroderma affects the skin only)
Generalised scleroderma antibodies
anti-Scl-70 antibodies
Features of scleroderma
Gradual onset
Affects predominantly women
- Raynauds
- renal failure
- malabsorption (GI involvement)
- thickening of skin
- dysphagia
Dental aspects of scleroderma
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.
Features of giant cell arteritis
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.
Treatment of giant cell arteritis
Medical emergency - needs urgent treatment
Prednisolone.
Polymyalgia rheumatica
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.
Kawasaki disease
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.
Wegener’s granulomatosis
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!
Fibromyalgia
Non-specific collection of musculoskeletal symptoms
- joint pain
- muscle pain
- functional disorders
Managed through pain management, not immunosuppressants.
Which vasculitides may present to dentist?
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)
