Sjogren's Disease Flashcards

1
Q

What is the definition of a dry mouth?

A

Decreases flow of saliva caused by failure of the salivary glands to function as normal

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2
Q

Name some functions of saliva

A
  1. Bolus formation
  2. Remineralisation of tooth tissue
  3. Lubrication
  4. Buffering capacity
  5. Taste aid
  6. Digestion: lysozyme, calprotectin
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2
Q

Are salivary glands under somatic or autonomic control?

A
  • Autonomic control
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2
Q

What does the parasympathetic and sympathetic function control?

A
  • Parasympathetic = secretory control
  • Sympathetic = protein secretion
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3
Q

When medications are taken to increase salivary secretions, do they target the sympathetic or parasympathetic system?

A

Parasympathetic

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3
Q

What is the normal resting and feeding flow rate for saliva?

A

RESTING: 0.35ml/min
FEEDING: 2ml/min

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4
Q

What are some subjective (false) and objective (true) causes of xerostomia?

A

Subjective:
- Mouth breathing
- Psychological
- Night dryness

Objective:
- Medications
- Anxiety
- Dehydration
- Conditions causing dehydration: diabetes mellitus, cardiac and renal failure, diuretic drugs, hypovolemic shock
- Gland / nerve damage
- Sjorgens syndrome
- Irradiations and cytotoxic drugs
- Neurological damage

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5
Q

If drugs cause xerostomia, what may they act on?

A
  • Directly on the glands
  • Sympathetic nerves and ganglia
  • Parasympathetic nerves and ganglia
  • Via dehydration
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6
Q

Name some drugs that cause xerostomia

A
  • Anti-Parkinson drugs
  • Tricyclic depressants
  • Antihistamines
  • Decongestants
  • Benzodiazepine
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7
Q

Name some clinical features of xerostomia

A
  • Mirror or fingers sticking to mucosa
  • Sticky, stringy or frothy saliva with no pooling
  • Difficulty swallowing and speaking
  • General oral discomfort
  • Reduced denture retention
  • Indirect effects: bad taste, increased caries, candidiasis, toothwear, lobulation of dorsum of tongue
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8
Q

What are some other history questions that should be considered with xerostomia?

A
  • Prolonged unremitting dryness
  • Abnormal taste
  • Does the patient smoke?
  • Drug history or history of radiotherapy
  • Does the patient mouth breathing?
  • Are their eyes affected?
  • History of current autoimmune disease
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9
Q

What is the name of the clinical dryness scale and what is a severe score?

A

Challacombe scale
>7 is severe

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10
Q

What are some special investigations needed for Sjorgrens disease?

A

1) Salivary flow rate (10 mins)
2) Saliva sample for candida
3) Urine glucose and osmolarity
4) Serology for auto-antibodies
5) Ultrasound
6) Biopsy (of lower lip to assess salivary glands)

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11
Q

What is the definition of Sjorgrens syndrome and how is most commonly affected?

A
  • Autoimmune disease which may affect all exocrine glands
  • A syndrome of dry eyes and dry mouth with or without connective tissue disorder
  • Can present as primary or secondary Sjorgrens
  • Commoner in female
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12
Q

What are some features of Sjogrens syndrome?

A
  • Failure of lacrimal secretion
  • Drying of anterior conjunctiva
  • Dry gritty eyes
  • Burning soreness
  • Cannot cry

Oral symptoms: mirror or fingers stick to mucosa, sticky&stringy saliva, discomfort swallowing and speaking, generalised oral discomfort, reduced denture retention, indirect: increased caries, candidiasis, toothwear, lobulated dorsum of tongue

Gland Swelling: parotid gland swelling in 10% of patients

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13
Q

What other conditions can sjorgrens present from?

A
  • Rheumatoid arthritis
  • SLE
  • Polyarteritis nodosa
  • Polymyositis
  • Autoimmune endocrine disorders: diabetes, thyroiditis
  • Scleroderma
  • Autoimmune hepatitis, primary biliary cirrhosis
14
Q

What are some extra-glandular features of Sjorgrens syndrome?

A
  • Excessive fatigue
  • Peripheral neuropathy
  • Lymphopenia, thrombocytopenia
  • Raynauds
  • CNS involvement: memory loss or confusion
  • Renal involvement
  • Pulmonary fibrosis
15
Q

What are the 6 criteria for Sjorgrens disease?

A

o Autoantibody – anti Ro, anti LA, ANA, FR
o Positive minor gland biopsy
o Ocular symptoms >3 months, gravel/gritty eyes, eye drops
o Oral symptoms >3 months +- gland swelling, frequent water needed
o Ocular signs – Schirmer 1 <5mm/5 mins, rose Bengal
o Salivary gland involvement – reduced flow, scintigraphy

16
Q

What is the aetiology of Sjorgrens?

A

Autoimmune disease.
Glands get infiltrated with activated B cells.
Lymphocytes collect around the ducts and destroy acinar cells.
Raised ESR and gamma-globulin.
- Influence from virus, hormonal and genetic factors leading to activation of auto-antibodies

17
Q

What are some tests for Sjorgrens disease?

A

Parotid flow rates.
Minor gland biopsy
Imaging glands
Blood tests
Immunoglobulin levels
Autoantibody screen
Lacrimal flow

18
Q

What changes do i see on biopsy in sjogren’s syndrome?

A

1) Periductal lymphocytic infiltrates
2) Expansion of lymphocytic infiltrates
3) Atrophy of salivary acini
4) Ductal hyperplasia forming epimyo-epithelial islands
5) Formation of lymphoepithelial lesion (lymphocytes infiltrating into ducts)

19
Q

How is MALT lymphoma linked to Sjorgrens?

A
  • B cell proliferation predisposes development of lymphoma
  • Greater risk in primary Sjogrens
  • Suspect in all cases with swollen glands
    Risks factors: parotid enlargement/splenomegaly, low CD4+ lymphocyte count, hypergammaglobulinemia
20
Q

What is SOX syndrome?

A
  • Sialadenitis
  • Osteoarthritis
  • Xerostomia
21
Q

What are the main aims of treatment for Sjorgrens?

A
  • Relief of symptoms dryness, glossodynia (burning pain of tongue)
  • Salivary stimulation or replacement therapy to keep mouth moist
  • Restoration of function (speech, swallowing)
  • Preservation of anatomical form & structures (filiform papillae - taste)
  • Dental tissues requiring repair / cosmetic improvement
  • Prevention of further dental disease
  • Screen for MALT lymphoma
  • Management of extra glandular disease
  • Manage fatigue
  • Manage inflammatory arthritis
  • Manage other problems (Raynaud’s,vasculitis etc)
22
Q

How do we manage xerostomia?

A
  • Ensure hydration (1-1.5l)
  • Preserve what flow remains
  • Avoid drying drugs
  • Diet analysis and fluoride to reduce caries risk
  • Regular sips of water
  • OHI
  • High fluoride toothpaste, fluoride varnish & chlorhexidine
  • Avoid strong flavoured toothpaste
  • Monitor for candida infection
  • Monitor for lymphoma
  • Stimulation: good hydration, sugar free gum, SST tablets, sugar free sweets, pilocarpine drug
  • Management of raynauds : Amlodipine or isoprolol
  • Use of Sjogrens: TNF alpha, retuximab
23
Q

What is the general advice for dry mouth?*

A
  • Encourage regular dental review (6 monthly)
  • Avoid strong soaps (use aqueous creams and emollients)
  • Avoid dry environments
  • Avoid cigarette smoking and excess alcohol
  • Smoking worsens oral disease
  • Alcohol worsens oral disease & very dehydrating
  • Receive information about self-help groups (e.g. BSSA)
24
Q

What are the differentials of Sjogrens?

A
  • Dehydration (D&V, Diabetes mellitus , Fluid restriction (CHF / CKD)
  • Iatrogenic (Drugs, Radiotherapy (<IMRT – laryngeal Ca*), Parotidectomy
  • Salivary gland disease (Salivary gland agenesis, Infections (HIV), Inflammatory (Sjögren’s / sarcoidosis / IgG4 disease)
  • Other (Anxiety)
  • Local (mouth breathing)
  • Subjective
25
Q

What is the name for excessive saliva?
What are some clinical symptoms?
What is the treatment?

A

PTYALISM
- psychiatric
- poor neuromuscular control
- muscle wasting disease
- local irritation

Treatment:
* Anticholinergic drugs local (hyoscine patches) or systemic
* Speech therapy
* Botox into submandibular glands before direct salivary ducts or excise submandibular glands
* Section of chorda tympani (some taste lost)