Connective Tissue Disorders and Xerostomia Symposium Notes Flashcards
Aims To give an overview of connective tissue diseases including Sjogren’s syndrome and describe the involvement of the salivary glands To review the anatomy and physiology of salivary glands To describe the causes, investigation and management of xerostomia and its impact on the orofacial tissues Objectives By the end of the two lectures students should: Be able to distinguish between the different connective tissue disorders Have a basic knowledge of Sjogren’s syndrome an
What are the main five connective tissue disorders to be aware of in Dentistry? (5)
- Rheumatoid arthritis
- Lupus erythematous
- Systemic sclerosis
- Sjogren’s syndrome
- Mixed connective tissue disorders
Describe Rheumatoid Arthritis (3, 1+1, 5)
- Autoimmune
- HLA-DR4 (70%)
- Multisystem inflammatory disease primarily affecting the synovium & adjacent tissues
- Rheumatoid arthritis-associated autoantibodies
- IgM class antibodies to the Fc protein off IgG are not disease specific
- Affects 1-2% off UK population – different degrees off severity
- Females > males (3:1)
- Peak incidence 30-40 years
- Often familial
- 50% off individuals unable to work 10 years post onset
What are the clinical signs and symptoms 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
- 15% cases have Sjogren’s syndrome
- TMJ damage in juvenile R
What joints are most commonly affected in rheumatoid arthritis? (10)
- Metacarpophalangeal/proximal interphalangeal 90%
- Metatarsophalangeal 90%
- Wrist 80%
- Ankle 80%
- Knee 80%
- Shoulder 60%
- Elbow 50%
- Hip 50%
- Cervical spine 40%
- TMJ 30%
What are the extra-articular manifestations of rheumatoid arthritis? (8)
- Weight loss
- Malaise
- Fever
- Lymphadenopathy
- Rheumatoid nodules
- Felty’s syndrome
- Amyloidosis
- Sjogren’s syndrome
What is Amyloidosis?
Amyloidosis is a group of rare but serious diseases caused by deposits of abnormal protein, called amyloid, in tissues and organs throughout the body. Amyloid proteins are abnormally folded, which causes them to build up in tissues and organs, leading to organ failure. A particular type of amyloid protein also occurs in the brain of people with Alzheimer’s disease
What is considered in the diagnosis of Rheumatoid Arthritis?
- Clinical
- Radiographic changes
- Anaemia
- Raised ESR, CRP
- Rheumatoid factor positive (80%)
- ANA positive (30%)
What is an ANA test?
A positive ANA test means autoantibodies are present. By itself, a positive ANA test does not indicate the presence of an autoimmune disease or the need for therapy
What are the general (conservative) measures in management of Rheumatoid Arthritis? (4)
- Education - Empowers
- Exercise - Maintenance of general fitness & maintain muscle bulk
- Physiotherapy
- Dietary advice - Weight reduction, 3 omega fatty acids
Describe the pharmacotherapy in the management of rheumatoid arthritis (3, 1+2)
- Non-steroidal anti-inflammatory drugs
- Disease modifying anti-rheumatic drugs
- Corticosteroids
- Biological agents
- TNF biological response modifiers
- etanercept & infliximab
9) Give five examples of DMARDs (Disease modifying anti-rheumatic drugs) and their uses (table)
Methotrexate»_space; Ulceration;
Gold»_space; Lichenoid reactions;
Penicillamine»_space; Loss of taste,
Lichenoid reactions, Severe ulceration;
Hydroxychloroquine »_space; Lichenoid reactions; Ciclosporin»_space; Gingival hyperplasia;
What are the orofacial aspects of rheumatoid arthritis? (1+1, 1+1, 1+1, 1+2, 1, 1+4)
- Access
> Individual with RA less likely to visit dentist (Pokrajc-Zirojevic et all 2002) - Atlanto-axial joint dislocation
> Physical comfort – pillows, short appointments - Impaired manual dexterity
> Electric toothbrush more effective than manual (Risheim et all 1992) - TMJ
> Commonly affected but one of the last joints involved
> May lead to open bite - Secondary Sjogren’s syndrome
- Felty’s syndrome
> RA & splenomegaly and lymphadenopathy
> Increased risk of infection (chronic sinusitis)
> Oral ulceration
> Angular cheilitis
What is Lupus Erythematosus? How many forms are there? (2)
- Immunologically mediated condition
- 2 forms: DLE, SLE
What is the aetiology of Lupus Erythematosus? (4)
- Genetic predisposition
- Environmental trigger
- Increase in B-cell activity
- Abnormal function of T-cells
Describe Discoid Lupus Erythematosus (5)
- Affects skin & oral mucosa
- F>M
- peak incidence 40 years
- Oral lesions similar to LP in appearance
- Diagnosis based on clinical/biopsy/immunology
How does Discoid Lupus Erythematosus affect the skin? (4)
- Scaly, erythematous patches
- Atrophic, hypo-pigmented areas
- Occur on exposed surfaces
- May be premalignant
What is involved in the diagnosis of DLE? (3)
- Clinical appearance
- Biopsy
- Circulating immunological profile
How is DLE managed? (1)
- Treat as for lichen planus
Describe Systemic Lupus Erythematosus (9)
- Age of onset ~ 30yrs
- F:M 8:1
- Systemic features
- Malar rash
- Polyarthritis
- Photosensitivity
- Oral lesions
- Renal/cardiac/haematological/neurological
- Up to 40% with oral lesions
Describe the clinical manifestations of SLE orally (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
What is involved in the diagnosis of SLE? (1,1+4)
Clinical
Immunological
- Hypergammaglobulinaemia
- Hypocomplimentaemia
- ANA (DNA + ENA) 90%
- RF 30%
What is the pharmacological management of SLE? What is the % survival after 10years? (4, 1+5,1)
-85% survival 10 years
-NSAIDs
-Hydroxychloroquine
-Corticosteroids
-Cytotoxic drugs
- Cyclophosphamide
- Azathioprine
- (methotrexate)
- (mycophenolate mofetil)
- (ciclosporin)
-(Dapsone) (Thalidomide) (Tacrolimus)
What is the prognosis of SLE? (3)
- Depends on extent of disorder
- Death due to renal involvement
- Males > females
What drugs can cause Lupus-like drug reactions? (6)
- Carbamazapine
- Hydralazine
- Methyldopa
- Penicillamine
- Procainamide
- Thiouracils
What is Systemic Sclerosis? (4)
- Autoimmune disorder
- Affects mainly females 20-50yrs
- Dense collagen is deposited in the tissues off the body
- Clinical features include
- Raynaud’s phenomenon
What is CREST syndrome? (6)
- C – calcinosis
- R – Raynaud’s phenomenon
- E – oesophageal dysfunction
- S - sclerodactyly
- T – telangiectasia
What are the orofacial manifestations of systemic sclerosis? (4, 1+3, 7)
- Facial skin rigidity
- Sharp nose
- Thinning of lips
- Loss of facial wrinkles
- Microstomia
-Poor access for oral hygiene
-Incidence off dental caries
-Incidence periodontal disease(abnormal immunoregulation & obliterative microvasculopathy) - Hypomobile tongue
- Dysphagia and xerostomia
- Periodontal ligament widened on XR
- Pseudoankylosis off TMJ (Temporomandibular joint (TMJ) pseudoankylosis is characterized by limited mouth opening and mandibular movements, resulting from factors outside the joint)
- Speech
- Eating
- Deteriorating quality of life
What is involved in the diagnosis of Systemic Sclerosis? (5)
- Difficult
- Essentially clinical
- Skin biopsy
- Scl-70 autoantibodies
- Periodontal widening on radiograph (30%)
What is the management for Systemic Sclerosis? (4)
- Difficult
- Nifedipine
- D-penacillamine
- Iloprost infusions
What is Microstomia? (2)
- Microstomia is the term used to describe a congenital or acquired reduction in the size of the oral aperture
- Severe enough to compromise cosmesis, nutrition, and quality of life
What is the management for Microstomia? (1+1, 3, 1+1)
- Exercise programme
-Mouth stretching and oral augmentation (Pizzo et al 2003) - Iontophoresis & ultrasound
- Sectional dentures
- Implants
- Surgical commissurotomy
-High incidence off wound dehiscence (surgical complication in which a wound ruptures along surgical suture)
What is Sjogren’s ’s syndrome? (7)
- Autoimmune exocrinopathy
- Primary & Secondary (with another systemic autoimmune condition such as RA/SLE)
- Focal lymphocytic infiltration of salivary & lacrimal glands
- Incidence 0.5-2%
- Females mainly
- 15% RA patients have secondary SS
- 30% SLE patients have secondary SS
What is the aetiology of Sjogren’s Syndrome (SS)? Genetic and Viral (1+2, 1+5)
Genetic predisposition
- HLA-B8
- HLA-DR3
Viral agents
- Herpes viruses
- (EBV, CMV, HHV-6)
- Hepatitis C virus (HCV)
- Retroviruses
- (HRV-5, HTLV-1)
What is the pathogenesis of SS? (4)
- Lymphocytic infiltration of exocrine glands
- Hypertrophy of ductal epithelium; formation of epimyoepithelial islands
- Acinar atrophy and fibrosis
- Probable hyperactivity of B-cells
Give examples of the subjective symptoms of xerostomia in patients with SS (9)
- Difficulty swallowing /chewing dry food
- Sensitivity to spicy food
- Altered salty bitter metallic taste
- Burning mucosa
- Lack or diminished taste
- Salivary gland swelling/pain
- Cough
- Voice disturbance
- Nocturnal discomfort
What are the Oral Signs of SS? (5)
- Initially often little change
- Oral mucosa - dry, atrophic, wrinkled, ulcerated, increased debris
- Tongue - dry, red, lobulated, loss of papilla
- Teeth - increased caries
- Salivary glands - firm on palpation – if swollen
Summarise the European Diagnostic Criteria for SS (1+6)
Summary of Classification of Signs & Symptoms
- I Ocular symptoms
- II Oral symptoms
- III Ocular signs
- IV Histopathology
- V Salivary gland involvement
- VI Autoantibodies
What are the rules for classification (using the European Diagnostic Criteria for SS) in primary and secondary SS? (1+2, 1+1)
Primary SS
- Presence of any 4 of the 6 items as long as either item IIV or VII positive
- Presence off any 3 of III, IV, V, or VI
Secondary SS
- Well-defined CT disease & presence of item I or II plus any 2 from III, IV and V
Describe SS lymphoma (2, 1+3)
- Type - predominantly B-cell (80% marginal zone of MALT type)
- Risk - 44x normal population
- Worse in patients with:
- Vasculitis
- Peripheral neuropathy
- Anaemia and lymphopenia and chronic glandular swelling
What is the palliative management of SS? (3)
- Increase lubrication
- Maintain oral/dental health
- Review candida status
What is the therapeutic management of SS? (2)
- Pilocarpine
- Immunomodulating agents
What are the indications of Mixed Connective Tissue Disease? (3, 1+2)
- Clinical signs of a number of A-I diseases
- Oral lichenoid lesions
- Trigeminal neuropathy
- Presence of:
-ANA (speckled)
-RNP autoantibody
What is the difference between xerostomia and hyposalivation? (3)
- Xerostomia is a symptom off oral dryness
- May exist with or without hyposalivation
- Hyposalivation is an actual decrease in saliva flow rate
How should salivary glands be assessed in terms of xerostomia/hyposalivation? (6)
- Salivary gland anatomy
- Saliva flow rate
- Saliva neural control
- Functions of saliva
- Caries control
- Effects of xerostomia
What are acini? What types of acini are there and what do they do? (1)
- Acini = saliva producing cells
- Serous – watery secretion
- Mucous – viscous saliva
What kind of gland is the parotid? (1)
- Mainly serous
How is saliva made and secreted? (4)
- Initial fluid secreted into ductal system
- Intercalated/striated & secretory ducts
- Protein & ion content modified within duct
- Ion exchange in striated ducts
47) What are the components of saliva? (1, 1+8, 1+8)
99.4% water
Organic solids
- Protein
- Gamma globulin
- Amylase
- Lysozyme
- Lactoferrin
- Glucose
- Lipids
- Amino acids
Inorganic solids
- Sodium
- Potassium
- Calcium
- Magnesium
- Chloride
- Phosphate
- Iodide
- Fluoride
