Connective tissue disorders and xerostomia symposium Flashcards
Types of connective tissue disorders (5)
Rheumatoid arthritis
Systemic and discoid lupus erythematosus
Systemic sclerosis
Sjogren’s syndrome
Rheumatoid arthritis epidemiology (6)
Affects 1-2% of UK population F > M (3:1) Peak incidence 30-40 Often familial Significant risk of mortality 50% of individuals unable to work 10 years post onset -anti-CCP is highly specific
What is rheumatoid arthritis? (4)
Autoimmune
HLA-DR4 (70%)
Multisystem inflammatory disease of synovium & adjacent
tissues
Rheumatoid arthritis-associated autoantibodies
- IgM class antibodies (Rheumatoid Factor) to the Fc protein of IgG are not
disease specific
-anti-CCP is highly specific
Clinical features of rheumatoid arthritis (11)
Insidious onset Pain and stiffness of small joints Fatigue and malaise Anaemia Weight loss Muscle weakness and wasting Neurological effects – carpal tunnel syndrome Lymphadenopathy Lung problems - pleural effusion, pleural nodules 15% cases have Sjögren’s syndrome TMJ damage in juvenile RA
Rheumatoid arthritis - joints most commonly affected (10)
Metacarpophalangeal/proximal interphalangeal 90% Metatarsophalangeal 90% Wrist 80% Ankle 80% Knee 80% Shoulder 60% Elbow 50% Hip 50% Cervical spine 40% TMJ 30%
Extra-articular manifestations of rheumatoid arthritis (8)
Weight loss Malaise Fever Lymphadenopathy Rheumatoid nodules Felty’s syndrome Amyloidosis Sjögren’s syndrome
Toothbrush for rheumatoid pts (2)
Electric with small head
-don’t need to move it as much so easier to use
Diagnosis of rheumatoid arthritis (7)
Clinical
Radiographic changes
Anaemia
Raised ESR, CRP
-C-reactive protein is accurate indicator for inflammation (goes up within hours, falls quickly too)
-erythrocyte sedimentation rate goes up slowly but lingers
Anti-CCP positive (80%+)
{Rheumatoid factor positive (80%)}
{ANA positive (30%)} - anti-nucleic antibodies
Management of rheumatoid arthritis - general measures (5)
Education: Empower – self management programmes Exercise: Maintenance of general fitness & maintain muscle bulk Physio/OT: Individual needs identified Surgery: For progressive deformity etc -synovectomy, tenosynovectomy, reconstructive surgery Dietary advice: Weight reduction (3 omega fatty acids)
Management of rheumatoid arthritis: pharmacotherapy (4)
DMARDs (Disease Modifying AntiRheumatic Drugs)
Corticosteroids – IA/IM/PO
Biological agents
– Anti-TNFα “biological agents”
– E.g. etanercept & infliximab
{Symptomatic relief – NSAIDs, COX-2 inhibitors}
NSAIDs side effects (3)
Stomatitis Erythema multiforme -target lesions Gastrointestinal bleeding – Depapillated tongue – Burning tongue – Candidosis
DMARDs and their oral effects (5)
Methotrexate (makes you folate deficient) -oral ulceration Gold (rarely used) -lichenoid reactions Penicillamine (rarely used) -loss of taste perception -lichenoid reactions -severe oral ulcerations Hydroxychloroquine -lichenoid reactions Cyclosporin -gingival hyperplasia
Biologic drugs and oral relevance (4)
Adalimumab (“Humira”)
-TB, oral candidosis, erythema multiforme
Etanercept (“Enbrel”)
-oral candidosis, sarcoid nodules to face, erythema multiforme
Infliximab (“Remicade”)
-histoplasmosis infection, OLP, mandibular osteomyelitis, parotid swelling, ulceration, erythema mutiforme
Rituximab (can be used for severe stubborn pemphigus)
-candidosis, ulceration
Orofacial aspects of rheumatoid arthritis (6)
Access
– Individual with RA less likely to visit dentist
Atlanto-axial joint dislocation
– Physical support – pillows, short appointments
Impaired manual dexterity
– Electric toothbrush more effective than manual
TMJ
– commonly affected but one of last joints
involved
– may lead to open bite
Secondary Sjögrens syndrome
Felty’s syndrome
– RA & splenomegaly and lymphadenopathy
– increased risk of infection (chronic sinusitis)
– oral ulceration
– angular cheilitis
What is lupus erythematosus and what are the forms? (3)
Immunologically mediated condition
2 forms
-DLE
-SLE
Aetiology of lupus erythematosus (4)
Genetic predisposition (more in black people, more in females)
Environmental trigger
T cell dysregulation of B cell activity
Possible defect in clearance of apoptotic cells?
Discoid lupus erythematosus (5)
- where does it affect
- epidemiology
- diagnosis
Affects skin & oral mucosa F>M peak incidence 40 years Oral lesions similar to lichen planus in appearance -but lichen planus usually bilateral Diagnosis based on clinical/biopsy/immunology
Discoid lupus erythematosus - skin features (4)
Scaly, erythematous patches
Atrophic, hypopigmented areas
Occur on exposed surfaces
May be premalignant
DLE - diagnosis (3)
Clinical appearance
Biopsy
Circulating autoantibodies – ANA, dsDNA
may be positive
DLE - management (1)
Treat as for lichen planus
Systemic lupus erythematosus - epidemiology (2)
Age of onset ~ 30 yrs
F:M 8:1
Systemic features of SLE (6)
Malar rash Polyarthritis Photosensitivity Oral lesions Renal/cardiac/haematological/neurological Up to 40% with oral lesions
SLE - oral features (4)
Up to 40% have oral lesions Unilateral or bilateral white patches with central area of erythema or ulceration May involve the palate May be extensive
Diagnosis of SLE (5)
Clinical Immunological: - hypergammaglobulinaemia - hypocomplimentaemia - ANA (DNA + ENA) 90% - RF 30%
Pharmacological management of SLE (5)
NSAIDs Hydroxychloroquine Corticosteroids Cytotoxic drugs – Cyclophosphamide – Azathioprine – (methotrexate) – (mycophenolate mofetil) – (ciclosporin) (Dapsone) (Thalidomide) (Tacrolimus)
Survival rate of SLE (1)
85% 10 years
Prognosis of SLE (3)
Depends on extent of disorder
Death due to renal involvement
Males > females
Lupus-like drug reactions (5)
Carbamazepine Hydralazine Methyldopa Penicillamine Procainamide
What is systemic sclerosis? (4)
Autoimmune disorder Affects mainly females 20 - 50yrs Dense collagen is deposited in the tissues of the body Clinical features include Raynaud’s phenomenon
Types of systemic sclerosis (3)
Localised cutaneous
- limited to the skin on the face, hands and feet
- 10-year survival of 75%
- <10% develop pulmonary arterial hypertension after 10-20yrs
Diffuse cutaneous
- more extensive skin involvement
- may progress to the visceral organs
- 10-year survival of 55%
- death most often from pulmonary, heart & kidney involvement
CREST syndrome
- collection of symptoms seen in limited cutaneous
CREST syndrome (5)
C – Calcinosis R – Raynaud’s phenomenon E – oEsophageal dysfunction S - Sclerodactyly T - Telangiectasia
Orofacial manifestations of systemic sclerosis (12)
Facial skin rigidity Sharp nose Thinning of lips Loss of facial wrinkles Microstomia – Poor access for oral hygiene – ↑ incidence of dental caries – ↑ incidence periodontal disease (abnormal immunoregulation & obliterative microvasculopathy) Hypomobile tongue Dysphagia and xerostomia Periodontal ligament widened on XR Pseudoankylosis of TMJ Speech Eating Deteriorating quality of life
Diagnosis of systemic sclerosis (5)
Difficult Essentially clinical Skin biopsy Scl-70 autoantibodies Periodontal widening on radiograph (30%)
Management of systemic sclerosis (4)
Difficult
Nifedipine
D-penicillamine
Iloprost infusions
Microstomia (5)
Exercise programme – Mouth stretching and oral augmentation Iontophoresis & ultrasound Sectional dentures Implants Surgical commissurotomy - High incidence of wound dehiscence
Sjogrens syndrome epidemiology (4)
Incidence 0.5-2%
Females mainly
15% RA patients have secondary SS
30% SLE patients have secondary SS
