salivary gland disease Flashcards
describe a ranula (what, presentation)
- sublingual gland mucus extravasation cyst in FOM
- 2-3cm fluctuant swelling, translucent
- may limit mouth opening and mastication
- can be plunging if it falls through discontinuous mylohyoid into neck
how does a ranula form/grow?
- damage to sublingual gland duct
- continuous flow of saliva without neural stimulation
list the 3 types of sialadentitis (inflammatory)
- acute sialadenitis (infections)
- chronic sialadenitis (obstruction)
- end stage sialadenitis
describe the two types of acute sialadenitis (cause, presentation)
1 mumps (highly contagious, paramyxovirus)
- painful uni/bilateral parotid swelling, headache, malaise, fever, orchitis
2 acute suppurative/bacterial parotitis - Staph aureus, often parotid
- painful gland swelling +/- suppuration from gland orifice
- more frequent in those with severe xerostomia
describe chronic sialadenitis (cause, site, symptoms)
- often duct obstruction (esp calculi)
- submandibular gland >
- unilateral swelling, asymptomatic or intermittently symptomatic +/- mealtime syndrome
- xerostomia predisposes to stones (stones do not cause xerostomia)
what is mealtime syndrome?
intermittent salivary gland region swelling and pain during mealtimes (increased pressure)
why is chronic sialadenitis more common in the submandibular gland? (2)
long pathway/duct
supersaturated saliva
how do salivary calculi form?
calcium, magnesium and phosphate ions deposit around a central nidus (cell debris, thick mucus), layer by layer
how may a sialolith be located? (3)
palpated
plain film radiographs with sialography
ultrasound imaging
how does chronic sialadenitis appear histologically? (3)
- loss of acini, ductal dilation, fibrosis
- chronic inflammatory cell infiltrate
- stone = dark, calcified, laminated appearance, often start in acini area
describe end stage sialadenitis (3)
- prolonged sialadenitis; “Kuttner tumour”
- hard fibrotic gland, no secretion or mealtime syndrome
- intermittent low-grade pain
describe HIV-associated salivary gland disease (demographic, aetiology, symptoms, histology)
- 5-10% of HIV-infected pts, children
- unknown aetiology, but linked to opportunistic infections
- uni/bilateral gland swelling +/- pain, often parotid
- T cell infiltration destroying acini, fibrosis –> multiple lymphoepithelial cysts
- low risk of lymphoma 1%
HIV-associated salivary gland disease management (2)
anti-retroviral therapy
+/- surgery or radiotherapy to decrease swelling
describe sialadenosis (what, symptoms, site, histology)
- non-inflammatory enlargement of salivary glands +/- pain, +/- decreased salivary flow
- often with associated systemic condition (endocrine, nutritional, neurological)
- usually parotid, bilateral
- 2-3x hypertrophy of gland acini with minimal inflammation
sialadenosis management (2)
- treatment of underlying systemic condition
- +/- surgery if significant cosmetic concerns
list salivary gland diseases/types (8)
- sialadenitis = infection or obstructions, allergy
- mucoceles, ranulas
- salivary tumours – benign or malignant
- systemic conditions:
– sialadenosis
– sarcoidosis
– lymphoma
– HIV-associated
– autoimmune (Sjogren’s, IgG4)
describe IgG4 sclerosing disease (what, presentation, histology)
- chronic autoimmune inflammation with dense enlarging fibrosis, mimics neoplastic conditions
- any site, esp pancreas, lungs, salivary and lacrimal glands
- uni/bilateral, parotid enlargement
- diagnostic histology = storiform pattern, many IgG4-secreting plasma cells, dense fibrosis
IgG4 sclerosing disease histology (2)
- storiform pattern
- many IgG4-secreting plasma cells, dense fibrosis
IgG4 sclerosing disease management (2)
- immediate corticosteroid therapy
- investigate other organs
describe allergic/eosinophilic sialadenitis (what, demographic, treatment)
- recurrent parotid swelling, mucus plugs, ductal abnormalities associated with eosinophilic-rich chronic inflammation
- Japan
- many pts atopic, with eosinophil presences suggests allergy component
- often fails to respond to treatment
allergic/eosinophilic sialadenitis demographic (2)
- Japanese
- atopic pts
list some imaging modalities for salivary gland diseases (7)
- ultrasound
- endoscopy
- scintigraphy and PET (nuclear medicine)
- plain radiograph (lower 90º, posterior oblique)
- sialography
- CT
- MRI
pros and cons of ultrasound imaging
+:
- simple, quick, non-invasive, cheap
- good compliance, no contrast or radiation
- high resolution, different orientations
- colour doppler for vascularity
-:
- operator-dependent, training
- messy with jelly
- superficial soft tissues only
salivary indications for ultrasound imaging (3)
- salivary gland lumps
- salivary gland obstructions
- diagnosis and follow up of Sjogren’s
how does a sialolith appear on ultrasound? (3)
- bright white, gently rounded
- acoustic shadow
- dilated duct associated
describe sialography
- radiopaque contrast solution (iodine-based) injected into duct, radiological techniques used to view and demonstrate ductal anatomy
- viewed live as injected or use plain film
indications for sialography (3)
- identify filling defects (stones, plugs), strictures, duct architecture, dilations
- suggests function of gland (clearance within 1 min normal)
- planning interventional treatments for sialolith extraction/stricture dilation
contraindications for sialography (3)
- iodine allergy
- acute infection of gland
- calculus at ostium of duct
what is punctate sialectasis? (2)
- lobules of radiopaque contrast where there is salivary gland breakdown/damage
- typical of Sjogren’s or recent sialadenitis
advantages of sialography (3)
- superior resolution and detail
- best sensitivity to differentiate stones and stenoses
- fluoroscopic sialography allows live visualisation
which investigation has 100% sensitivity for salivary calculi?
CT scan
MI methods of removing sialoliths (4)
- extracorporeal shock wave lithotripsy (ECSWL)
- stone retrieval with baskets
- intracorporeal shock wave lithotripsy
- conservative surgery
explain briefly how extracorporeal shock wave lithotripsy works
- ultrasound to identify location
- piezoelectric shockwave generator targets stone
- multiple visits to hit 10,000 times
- aims to break down stone
indications and contraindications of stone retrieval with baskets
- indications = stone ≤25% of lumen, mobile, accessible
- contraindications = larger stone, small duct, unsuitable position, stricture distal to stone
briefly explain how balloon duct dilation helps with strictures
expandable balloon catheter introduced and inflated within salivary ductal system with guide wire
indications for balloon duct dilation (2)
- partial stenosis/multiple strictures of major gland duct
- open pathway for sialoliths to be removed
contraindications of balloon duct dilation (2)
- complete ductal stenosis
- asymptomatic or non-functioning gland
ranula treatment (2)
- marsupialisation/decompression
- excision of whole gland by intra/extraoral approach under GA
risks with submandibular gland removal (5)
- marginal mandibular nerve (angle of mouth), hypoglossal nerve, lingual nerve damage
- “stump” syndrome
- scarring
- haematoma (FOM)
- damage to duct/salivary flow
causes of false xerostomia (3)
- mouth breathing
- psychological
- night dryness
causes of true xerostomia (9)
- medications
- psychogenic (eg anxiety)
- dehydration
- systemic conditions (diabetes, cardiac and renal failure, hypovolaemic shock)
- gland/nerve damage
- Sjogren’s, SOX syndrome
- irradiation, cytotoxic drugs
- neurological damage
- hypoplasia/aplasia of glands, vitamin A deficiency
give some (types of) drugs which may cause xerostomia (up to 8)
- atropine-like muscarinic drugs = atropine, hyoscine, anti-Parkinsonian, tricyclic antidepressants, antihistamines, antipsychotics, old antihypertensives
- sympathomimetics = amphetamines, decongestants, monoamine oxidase inhibitors
- opiate-based analgesics
- benzodiazepines
- appetite suppressants
- H2 antagonists, PPIs
- anti-HIV drugs (protease inhibitors)
- alcohol, coffee
which drugs/substances do not cause xerostomia even though you would think they might? (2)
- beta blockers
- smoking (subjective feeling due to mucosal thickening)
what are the salivary flow rates for xerostomia? (2)
- whole unstimulated flow <0.2ml/min in 10 mins
- parotid flow <0.4ml/min per gland with citric acid used twice in 10 mins
what are the normal salivary flow rates? (2)
10min unstimulated flow 0.2-0.3ml/min
10min stimulated parotid flow 1-2ml/min
give some s/s of xerostomia (9 + indirect effects)
- low salivary flow rate
- mirror sticking to mucosa
- sticky, stringy, frothy saliva with no pooling in FOM
- absence of saliva
- difficulty swallowing and speaking
- general oral discomfort
- decreased denture tolerance/retention
- debris sticking to teeth
- erythematous gingivae and loss of papillae
- indirect effects = bad taste, caries, candidiasis, sialadentitis, lobulation or fissuring or depapillation of tongue dorsum, mucosal atrophy
what questions may you ask in the history for a dry mouth? (8)
- when? constant or intermittent
- any associated features - anxiety, drugs, taste
- smoking
- drug history
- any cancer treatment
- mouth breathing, snoring
- other mucosae
- autoimmune disease
what special investigations may you do for xerostomia? (7)
- salivary flow rate (dribble into universal container
- saliva sample for candida/pathogens
- parotid saliva sample to culture if cloudy
- urine glucose and osmolarity (diabetes)
- refer for specialist neurological investigations and ultrasound
- serology for inflammatory markers or auto-Abs
- biopsy (focal lymphocytic sialadenitits)
investigations for suspected Sjogren’s syndrome (4)
- parotid flow rate
- minor gland biopsy
- blood test = FBC, film; ESR, Ig levels; autoantibody screen (ANA, SSA, SSB)
- lacrimal flow (conjunctival staining and tear breakup time)
what investigations may be done for dry eyes? (3)
- Schirmer test (filter paper, 5mins)
- ocular staining with Lissamine green
- tear breakup time
describe Sjogren’s syndrome (what, demographic, types)
- chronic, autoimmune, rheumatic, progressive gland atrophy
- middle aged females with 2 peaks (mid-30s, mid-50s)
- may affect all exocrine glands
- primary = dry eyes and mouth, non-glandular features
- secondary = pre-existing connective tissue disease, usually less severe features
ocular features of Sjogren’s syndrome (6)
- failure of lacrimal secretion
- drying of anterior conjunctiva
- dry, gritty eyes
- eyelids adherent in morning
- burning soreness
- keratoconjunctivitis sicca
glandular features of Sjogren’s syndrome (7)
- parotid swelling (more in primary SS)
- lacrimal gland swelling
- increased risk of lymphoma
- dry vagina
- dry skin
- dry oesophagus
- dry lungs +/- infections
extraglandular s/s of Sjogren’s syndrome (9)
- Raynaud’s phenomenon
- excessive fatigue in waves
- myalgia, arthralgia
- peripheral neuropathy
- lymphopaenia, thrombocytopaenia
- hypergammaglobulinaemia and cryoglobulinaemia (sign of active SS)
- CNS symptoms - brain fog to cognitive difficulties
- renal = esp if pre-existing lupus
- hepatobiliary chronic inflammation (primary biliary cirrhosis)
what two features are uncommon in secondary Sjogren’s syndrome compared to primary?
- gland swelling
- lymphoma
secondary Sjogren’s associated connective tissue disorders (3)
- rheumatoid arthritis (50%)
- systemic lupus erythematosus
- systemic sclerosis
what is the D/C for Sjogren’s syndrome?
- autoantibodies (Ro, La, ANA, RF +/or positive minor gland biopsy)
plus 2 of: - ocular symptoms >3 months
- oral symptoms >3 months
- ocular signs (Schirmer test, Rose Bengal stain)
- salivary gland involvement with decreased flow
what would the Schirmer test show with Sjogren’s?
<5mm wetting in 5 mins
describe the pathogenesis of Sjogren’s syndrome (3)
- failure of immune control and loss of tolerance (autoimmune)
- systemic polyclonal B cell hyperreactivity
- activated B cells infiltrate glands and collect around ducts, destroy acinar cells
what may you expect the blood markers to look like for Sjogren’s syndrome? (2)
- increased ESR and gammaglobulin
- autoantibodies = ANF/ANA, Ro/SSA, La/SSB, RF, antithyroid, gastric parietal cells
histology of Sjogren’s syndrome (3)
- periductal CD4+ lymphocytic infiltrates
- lymphoepithelial lesions (epimyoepithelial islands in background of lymphocytes)
- atrophy of salivary acini
where is a biopsy usually taken for Sjogren’s syndrome and why?
lower lip (easily accessible, similar features in major and minor glands)
which type of Sjogren’s syndrome increases the risk of MALT lymphoma?
primary SS
describe MALT-type lymphoma (AKA, presentation, prognosis)
- extranodal marginal zone B cell lymphoma
- persistent swelling of parotid (all swollen glands should be suspected)
- low grade with good prognosis
MALT-type lymphoma investigations (2)
- biopsy
- PCR and immunohistochemistry to detect clonality
MALT-type lymphoma risk factors (5)
- parotid enlargement, splenomegaly, lymphadenopathy
- purpura, cryoglobulinaemic vasculitis
- hypergammaglobulinaemia
- neutropenia
- low CD4+ count
MALT-type lymphoma treatment options (2)
controversial:
- wait and watch
- low dose radiotherapy
how does Sjogren’s syndrome appear on ultrasound and why?
- honeycomb
- lymphoepithelial infiltrates
describe SOX syndrome (what, features)
- mutation in SOX genes
- Sialadenitis, Osteoarthritis, Xerostomia
- symptomatic dry eyes and mouth, decreased whole flow but NORMAL stimulated flow
- non-specific diffuse sialadenitis
- primary generalised nodal osteoarthritis
- NO RF or Ro/La auto-Abs
oral management of Sjogren’s syndrome (6)
- explain and reassure
- preserve remaining flow, avoid drying drugs (liaise with GP)
- salivary stimulation
- salivary replacement
- symptomatic treatment for soreness/dryness
- monitor for caries, TW, candida, lymphoma
what is involved in salivary stimulation for Sjogren’s disease? (3)
- hydration - 1-1.5L water/day (consider renal)
- sugar-free gum/sweets, saliva stimulating tablets (buffered, lemon)
- pilocarpine 5mg 1-3x/day, parasympathomimetic
contraindications to pilocarpine (3)
asthma
arrhythmias
glaucoma
what is involved in salivary replacement for Sjogren’s syndrome?
sprays, gels, MW (eg Biotene)
medical management for Sjogren’s syndrome (not oral) (5)
- NSAID and paracetamol pain relief (arthralgia)
- education to increase low intensity exercise (fatigue)
- amlodipine or isoprolol (severe Raynaud’s)
- hydroxychloroquine, methotrexate, prednisolone +/- steroid sparing (musculoskeletal pain)
- rituximab considered for primary SS where conventional therapy has failed
general advice for dry mouth or Sjogren’s syndrome pts (4)
- regular dental review (6 monthly)
- avoid strong soaps, use aqueous creams and emollients
- avoid dry environments, smoking, XS alcohol
- self help groups
dry mouth differential diagnoses/causes (6)
- dehydration (recent diarrhoea, vomiting; diabetes, fluid restrictions)
- iatrogenic (drugs, radiotherapy, parotidectomy)
- salivary gland diseases - agenesis (rare), infection, inflammatory
- anxiety
- local, mouth breathing
- subjective feeling
give the features measured by the clinical dryness scale (12)
- mirror sticks to buccal mucosa
- mirror sticks to both buccal mucosa
- mirror sticks to tongue
- frothy saliva
- no pooling of saliva in FOM
- tongue depapillation
- highly fissured tongue
- lobulated tongue
- altered gingival architecture
- glassy appearance of oral mucosa
- cervical caries >2 teeth
- debris on palate
what is mild dryness and its management as guided by the clinical dryness scale? (4)
- score 1-3 - may not need tx
- sugar-free chewing gum 15mins BD
- attention to hydration
- routine check ups and monitoring
what is moderate dryness and its management as guided by the clinical dryness scale? (5)
- score 4-6, investigate if unclear cause
- sugar-free gum or simple sialogogues
- saliva substitutes
- topical fluoride
- regular monitoring
what is severe dryness and its management as guided by the clinical dryness scale? (4)
- score 7-10 - must ascertain cause and exclude Sjogren’s (REFER)
- saliva substitutes
- topical fluoride
- monitored for changing s/s +/- specialist input
functions of saliva (6)
- lubrication (eating, speech)
- cleansing
- buffering (bicarb)
- limits pathogen growth (lysozyme, IgA)
- remineralisation
- digestion (amylase)
how does each part of the autonomic system affect salivary glands (normal function and if blocked)?
- parasympathetic = secretory; blocked –> gland atrophy
- sympathetic = protein secretion, little effect if blocked
what factors influence salivary centres? (5)
- taste
- smell
- chewing
- anxiety
- sleep/diurnal variation
what is ptyalism?
excess salivation
what may cause true ptyalism? (5)
- local irritation/reflex (ulcers, ANUG, dentures)
- lithium, anti-cholinergics (can also cause dry mouth)
- waterbrash, idiopathic paroxysmal sialorrhoea (rare)
- heavy metal poisoning, iodine
- rabies
what may cause false ptyalism? (4)
- psychiatric
- poor neuromuscular control (eg not swallowing) = Parkinson’s, stroke, Bell’s palsy
- muscle wasting diseases
- head-down posture in disability
treatment for ptyalism (4)
(if not self-limiting)
- anticholinergic drugs - local (hyoscine patches) or systemic
- speech therapy (esp if previous stroke)
- botox into submandibular glands (can also be diagnostic) or surgical excision
- sectioning of chorda tympani