Connective tissue disorders and xerostomia symposium Flashcards
(92 cards)
1
Q
Types of connective tissue disorders (5)
A
Rheumatoid arthritis
Systemic and discoid lupus erythematosus
Systemic sclerosis
Sjogren’s syndrome
2
Q
Rheumatoid arthritis epidemiology (6)
A
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
3
Q
What is rheumatoid arthritis? (4)
A
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
4
Q
Clinical features of rheumatoid arthritis (11)
A
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
5
Q
Rheumatoid arthritis - joints most commonly affected (10)
A
Metacarpophalangeal/proximal interphalangeal 90% Metatarsophalangeal 90% Wrist 80% Ankle 80% Knee 80% Shoulder 60% Elbow 50% Hip 50% Cervical spine 40% TMJ 30%
6
Q
Extra-articular manifestations of rheumatoid arthritis (8)
A
Weight loss Malaise Fever Lymphadenopathy Rheumatoid nodules Felty’s syndrome Amyloidosis Sjögren’s syndrome
7
Q
Toothbrush for rheumatoid pts (2)
A
Electric with small head
-don’t need to move it as much so easier to use
8
Q
Diagnosis of rheumatoid arthritis (7)
A
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
9
Q
Management of rheumatoid arthritis - general measures (5)
A
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)
10
Q
Management of rheumatoid arthritis: pharmacotherapy (4)
A
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}
11
Q
NSAIDs side effects (3)
A
Stomatitis Erythema multiforme -target lesions Gastrointestinal bleeding – Depapillated tongue – Burning tongue – Candidosis
12
Q
DMARDs and their oral effects (5)
A
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
13
Q
Biologic drugs and oral relevance (4)
A
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
14
Q
Orofacial aspects of rheumatoid arthritis (6)
A
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
15
Q
What is lupus erythematosus and what are the forms? (3)
A
Immunologically mediated condition
2 forms
-DLE
-SLE
16
Q
Aetiology of lupus erythematosus (4)
A
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?
17
Q
Discoid lupus erythematosus (5)
- where does it affect
- epidemiology
- diagnosis
A
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
18
Q
Discoid lupus erythematosus - skin features (4)
A
Scaly, erythematous patches
Atrophic, hypopigmented areas
Occur on exposed surfaces
May be premalignant
19
Q
DLE - diagnosis (3)
A
Clinical appearance
Biopsy
Circulating autoantibodies – ANA, dsDNA
may be positive
20
Q
DLE - management (1)
A
Treat as for lichen planus
21
Q
Systemic lupus erythematosus - epidemiology (2)
A
Age of onset ~ 30 yrs
F:M 8:1
22
Q
Systemic features of SLE (6)
A
Malar rash Polyarthritis Photosensitivity Oral lesions Renal/cardiac/haematological/neurological Up to 40% with oral lesions
23
Q
SLE - oral features (4)
A
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
24
Q
Diagnosis of SLE (5)
A
Clinical Immunological: - hypergammaglobulinaemia - hypocomplimentaemia - ANA (DNA + ENA) 90% - RF 30%
25
Pharmacological management of SLE (5)
```
NSAIDs
Hydroxychloroquine
Corticosteroids
Cytotoxic drugs
– Cyclophosphamide
– Azathioprine
– (methotrexate)
– (mycophenolate mofetil)
– (ciclosporin)
(Dapsone) (Thalidomide) (Tacrolimus)
```
26
Survival rate of SLE (1)
85% 10 years
27
Prognosis of SLE (3)
Depends on extent of disorder
Death due to renal involvement
Males > females
28
Lupus-like drug reactions (5)
```
Carbamazepine
Hydralazine
Methyldopa
Penicillamine
Procainamide
```
29
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
```
30
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
31
CREST syndrome (5)
```
C – Calcinosis
R – Raynaud’s
phenomenon
E – oEsophageal
dysfunction
S - Sclerodactyly
T - Telangiectasia
```
32
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
```
33
Diagnosis of systemic sclerosis (5)
```
Difficult
Essentially clinical
Skin biopsy
Scl-70 autoantibodies
Periodontal widening on radiograph
(30%)
```
34
Management of systemic sclerosis (4)
Difficult
Nifedipine
D-penicillamine
Iloprost infusions
35
Microstomia (5)
```
Exercise programme
– Mouth stretching and oral augmentation
Iontophoresis & ultrasound
Sectional dentures
Implants
Surgical commissurotomy
- High incidence of wound dehiscence
```
36
Sjogrens syndrome epidemiology (4)
Incidence 0.5-2%
Females mainly
15% RA patients have secondary SS
30% SLE patients have secondary SS
37
Sjogrens syndrome - what is it (3)
Autoimmune exocrinopathy
Primary & Secondary (with another systemic autoimmune condition such as RA/SLE)
Focal lymphocytic infiltration of salivary & lacrimal glands
38
Aetiology of Sjogrens syndrome (2)
```
Genetic predisposition
-HLA-B8
-HLA-DR3
Viral agents
-Herpes viruses (EBV, CMV, HHV-6)
-Hepatitis C virus (HCV)
-Retroviruses (HRV-5, HTLV-1)
```
39
Sjogrens syndrome - pathogenesis (4)
```
Lymphocytic infiltration
of exocrine glands
Hypertrophy of ductal
epithelium; formation of
epimyoepithelial islands
Acinar atrophy and
fibrosis
Probable hyperactivity of
B-cells
```
40
Subjective symptoms of xerostomia in pts with Sjogren's syndrome (lots)
```
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
```
41
Sjogren's syndrome - oral signs (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
42
Summary of Classification of signs and symptoms of Sjogren's Syndrome: European Diagnostic Criteria (6)
```
I Ocular symptoms
II Oral symptoms
III Ocular signs
IV Histopathology
V Salivary gland involvement
VI Autoantibodies (anti-RO [positive in 70%] and anti-LA [positive in 30%])
```
43
European Diagnostic Criteria: rules for classification of primary SS (2)
Presence of any 4 of the 6 items as long
as either item IV or VI positive
Presence of any 3 of III, IV, V, or VI
44
European Diagnostic Criteria: rules for classification of secondary SS (1)
Well-defined CT disease & presence of item I or II plus any 2 from III, IV and V
45
Sjogren's syndrome - non Hodgkin's lymphoma (3)
- type
- risk
```
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
```
46
Management of Sjogren's Syndrome (5)
```
Palliative
- increase lubrication
- maintain oral/dental
health
- review candida status
Therapeutic
- pilocarpine
- immunomodulating
agents
```
47
What is mixed connective tissue disease? (5)
```
Clinical signs of a number of A-I diseases
Oral lichenoid lesions
Trigeminal neuropathy
Presence of
- ANA (speckled)
- RNP autoantibody
```
48
Xerostomia versus hyposalivation (3)
Xerostomia is a symptom of oral dryness
May exist with or without hyposalivation
Hyposalivation is an actual decrease in
saliva flow rate
49
Salivary glands - anatomy
```
3 major
• Parotid
-account for 60% of total salivary tissue
• Submandibular (30%)
• Sublingual (5%)
•> 600 minor salivary glands (5%)
• Within lips/cheeks & palate
```
50
What are acini? (3)
```
Saliva producing cells
Serous - watery secretion
Mucous - viscous saliva
Parotid glands mainly serous cells
Other glands mainly mucous
```
51
Salivary duct system (4)
```
Initial fluid secreted
into ductal system
Intercalated/striated &
secretory ducts
Protein & ion content
modified within duct
Ion exchange in
striated ducts
```
52
Amount of water in saliva (1)
99.4% water
53
Organic solids in saliva (a lot)
```
Protein
Gamma globulin
Amylase
Lysozyme
Lactoferrin
Glucose
Lipids
Amino acids
```
54
Inorganic solids in saliva (a lot)
```
Sodium
Potassium
Calcium
Magnesium
Chloride
Phosphate
Iodide
Fluoride
```
55
Formation of saliva (4)
Salivary gland secretion mainly under autonomic nervous control
Various hormones may modify salivary composition
Increased salivary flow mainly a result of parasympathetic activity
Vasodilation in blood vessels within glands
56
2 components of saliva (5)
Fluid component includes ions produced by
parasympathetic stimulation
Protein component arising from secretory vesicles in
acini - released in response to sympathetic stimulation
Effects of parasympathetic stimulation stronger & longer
lasting
Parasympathetic stimulation --> copious saliva of low
protein concentration
Sympathetic stimulation --> little saliva but with high
protein concentration
57
Saliva flow rate (lots)
500mls saliva in 24 hour period
Unstimulated/resting flow rate 0.3ml/minute
Flow rate during sleep --> 0.1 ml/minute
During eating or chewing --> 4.0-5.0 ml/minute
Unstimulated conditions --> 60-65% saliva from
submandibular glands, 20-25% from parotids &
2-5% from sublingual glands
During eating --> parotid contributes 50% saliva
Smell/taste --> increased salivary flow
Anxiety --> increased salivary flow
58
Saliva flow rate: unstimulated whole saliva (2)
Measured by spitting into gradated container for
15 minutes
<1.5 mls in 15 minutes suggests decreased function
59
Saliva flow rate: stimulated flow rate (3)
Carlsson-Crittenden cups placed over parotid
orifice
Saliva stimulated by placing 1ml 10% citric acid
on tongue dorsum
<5ml in 5 minutes implies decreased function
60
Functions of saliva (8)
Lubricant effect
Physical cleanser
Caries control – high HCO3
- buffers acid
Saliva saturation with Ca++ & PO4 prevents
demineralisation
Pellicle formation – salivary proteins form barrier
Antimicrobial – Igs/lysozyme/proteins/lactoferrin
Taste – substances in solution for sense of taste
Digestion of carbohydrates begin (amylase)
61
Effects of long-standing xerostomia (lots)
Difficulties in oral function & wear of dentures
Increased Frequency of caries (particularly cervical caries)
Acute gingivitis
Dysarthria
Dysphagia
Taste disturbances
Increased susceptibility to oral candidosis
Burning tongue/depapillation of tongue
Dry, sore cracked lips
Salivary gland
enlargement
62
Factors associated with hyposalivation and/ or xerostomia (6)
```
Age ~ 30% aged > 65 yrs
Anxiety/depression states
Dehydration
Drugs
Radiotherapy/ chemotherapy
Diabetes
```
63
Drug-related xerostomia - common (lots)
```
Tricyclics
SSRIs
Antihistamines
Diuretics
Sympathomimetics
Anticholinergics
Antipsychotics
Antiparkinsonian
Sedatives
```
64
Drug-induced xerostomia - must consider other confounding factors: (6)
```
Age
Gender
Smoking
Psychological factors
Other diseases causing hyposalivation
Drug-related xerostomia reversible with
cessation of drug
```
65
Radiation-induced salivary dysfunction (5)
Therapeutic doses of radiation for head & neck
cancer --> permanent reduction in salivary gland
function
Degree of damage dependent on no & volume
of salivary glands exposed
26Gy = threshold below which recovery of 25%
stimulated saliva flow rate can occur
Acute inflammatory reaction --> eventual fibrosis
--> decreased blood flow --> loss of acinar cells
Scant/sticky saliva --> increased caries risk/oral
candidosis/ taste disturbance/ dysphagia etc
66
Chemotherapy-induced salivary dysfunction (2)
Chemotherapy may also adversely affect
salivary function but the extent & underlying mechanism requires further
clarification
Effect appears reversible over the following year
67
Factors associated with hyposalivation and/ or xerostomia (9)
```
Autoimmune/immune-based
-Sjogren’s syndrome 1. & 2.
-primary biliary cirrhosis
-autoimmune thyroiditis
-chronic graft versus host
disease
-sarcoidosis
Infections
-HIV
-hepatisis C
-CMV
-Epstein Barr virus
```
68
Rare factors associated with hyposalivation and/ or xerostomia (4)
Amyloidosis
Haemochromatosis
Wegener’s disease
Salivary gland agenesis
69
Xerostomia - history (13)
```
Does your mouth usually feel dry?
Does your mouth feel dry when eating?
Do you have difficulty swallowing food?
Do you require liquids to sip to swallow foods?
Do you take water to bed at night because your
mouth feels dry at night?
Have you noticed difficulty wearing your
dentures?
Any soreness of your mouth?
Dry sore eyes/skin/genital area?
Past medical history
Anxiety/depression
Drug history
Social history – smoking/alcohol
Family history – ? autoimmune diseases
```
70
Xerostomia - clinical examination (lots)
```
General appearance
-access problems
Extraoral features
-stigmata of connective tissue diseases
-salivary gland swellings
Intraoral findings
-lack of pooling of saliva in floor of mouth
-dental mirror sticks to mucosa
-food retention
-gingival health
-caries
-candidosis
-depapillation/lobulation of tongue
```
71
Clinical investigations for xerostomia (3)
Sialometry
Schirmer's test
Rose Bengal staining
72
Radiological investigations for xerostomia (6)
```
USS
Sialography
Salivary scintigraphy
PET scan
PET scan
MRI
CT
```
73
Lab-based investigations for xerostomia (lots)
```
Immunology
–ENA/ANA/Rh Factor
Haematology
–FBC/CRP/ESR
SACE
Liver function
Thyroid function
Cryoglobulins
Other antibodies
-anti-mitochondrial
-anti-smooth muscle
```
74
Scintigraphy (2)
```
Technique that
investigates glandular
function rather than
structure
Measures the active
uptake of a radiolabelled marker such
as technetium-99m
after IV infusion
```
75
Labial gland biopsy (2)
```
Examination of at least 5 lobules of minor glands ideally
Histopathological features supportive of Sjogren’s syndrome
-acinar loss
-duct dilation
-periductal fibrosis
-focal lymphocytic infiltrate
-focal aggregate of at least
50 lymphocytes
--> > 1 focal aggregate per
4mm2 has high
specificity
```
76
Treatment of xerostomia (9)
Treatment is directed at underlying cause
Prevention is key due to lack of efficacy of
saliva replacement therapy
Assess patients before radio & chemoTx
Manage xerostomia early
Prevent dental complications
Multidisciplinary approach often required
Stimulation of saliva production
Use of saliva substitutes
Oral healthcare to prevent & manage:
- Caries
- Gingivitis
- Candidosis
77
Management of SS - the multidisciplinary team (6)
```
Rheumatologist
Ophthalmologist
Oral Medicine specialist
GDP
GMP
Psychologist
```
78
Dental caries prevention strategy - xerostomia (7)
```
Patient education
Diet and nutrition counselling
Hygiene control
Fluoride
Microbial control
Rehydration therapy
Dental treatment considerations
```
79
Diet and nutrition counselling - xerostomia (5)
Avoid soft sticky and liquid diets which promote dental plaque development
Eliminate salty spicy foods - irritant
Non cariogenic foods –suggest sugar substitutes
Limit caffeine – dehydration
Nutrient deficiencies have been described
80
Hygiene control - xerostomia (5)
```
Soft electric tooth brush – handle modification
Accessories - interdental brush / floss
Use disclosing tablets
Children’s toothpaste – mint irritant
Oranurse toothpaste - bland
```
81
Microbial control - xerostomia (5)
```
High levels lactobacilli reported
Chlorhexidine rinse
Chlorhexidine varnish
Fluoride rinse has some antimicrobial activity
Alcohol containing mouthwashes avoided
```
82
Dental treatment considerations - xerostomia (6)
```
3-4 monthly visits
Remember fragility of oral tissues
Consider fissure sealants
Glass ionomer choice for provisional
restorations
Dentine of exposed roots – dentine bonding
resin adhesive systems
Amalgam more successful than bonded
materials
```
83
Dental treatment considerations - prosthetics (5)
Some patients use dentures successfully
Tongue adheres to and dislodges denture
Mucosal irritation and ulceration common
Dentures with reservoirs for artificial saliva
Implants
– Increased comfort and function of prostheses
84
Management of dry mouth (6)
```
Oral moisturisers
Gustatory and mechanical stimulation of
salivation
Special toothpastes and mouthwashes
Saliva substitutes
Lip creams and ointments
Systemic therapy
```
85
Oral moisturisers (4)
```
Frequent sips of water
Saline solutions
Water plus sodium bicarbonate
Overuse removes small amounts mucous
saliva from oral tissues and increases dry
mouth sensation
```
86
Gustatory and mechanical stimulants (3)
```
Acidic stimulation – uncomfortable &
increase enamel demineralisation
Sugarless chewing gum
Lozenges
–Salivix® pastilles
–SST® tablets
```
87
Other stimulatory methods (2)
Acupuncture
– mainly stimulated salivary function affected
– result of neuropeptide release (VIP and calcitonin generelated peptide)
– In recent systematic review inconclusive evidence to confirm efficacy
Electrostimulation
88
About saliva substitutes (lots)
```
Carboxymethyl cellulose, mucin, polyacrylic acid
Oils & glycerin
Majority of patients prefer water
Relief insignificant & short lived
Unpalatable
Impractical to handle
Expensive
Choice based on personal preference
Mucin may have better patient acceptance
```
89
Systemic therapy for Sjogrens syndrome (2)
When residual secretory capacity in salivary glands
exists may use cholinergic agents:
Pilocarpine
– Parasympathomimetic with mild β-adrenergic stimulating
properties & non-specific
– RCT
– Increased salivary output is transient dose related and consistent
– No tolerance
– Animal studies show caries reduction
– Lack of correlation between improved salivary flow and QoL scores may be related to coexistence of comorbidities
– Approved for treatment of radiation-induced
xerostomia
– 5mg orally 3 times daily with titration up to 10mg
– From present evidence advisable to prescribe
pilocarpine after completion of radiotherapy for 3 month trial if no contraindications
90
Adverse effects / contraindications of pilocarpine (4)
```
• Adverse effects - flushing sweating
urinary frequency
• Contraindicated in
-uncontrolled asthmatics
-narrow angle glaucoma
-acute iritis
```
91
Systemic therapy for Sjogrens syndrome: hydroxychloroquine (4)
– Flow rate increased in 82% patients
– Improved oral discomfort
– 40% decrease in number of oral infections
– Few adverse effects
92
Systemic therapy for Sjogren's syndrome: corticosteroid irrigation parotid gland (3)
– Increased flow rate
– Relief of symptoms
– Risk of infection and pain
