Sjogren syndrome,Halitosis &Treatment of OSF

Dr. Tahmasub Faraz TayyabBDS, FCPS (OMFS)

Sjögren's syndrome

Pronounced as “shohgrinz syndrome”. It is an autoimmune disease in which immune cells

attack and destroy the exocrine glands. It is the second most common autoimmune disease

after SLE (Systemic lupus erythematous). Although Sjögren's occurs in all age groups in men

and women, it is most common in women. Nine out of ten Sjögren's patient are women and the

average age of onset is after the menopause.

Causes of Sjögren's syndrome

The cause of Sjögren's syndrome is not exactly known. Maybe by a combination of genetic and environmental

factors. Only the presence of the gene doesn’t cause Sjögren's

syndrome. An external trigger is said to activate the immune system.

The immune system responds even when there are no foreign substances to fight off.

This inflammatory response causes the body’s white blood cells to attack and destroy certain moisture producing glands.

Clinical Types This disease is caused by an immune-mediated inflammation

of salivary,lacrimal and sweat glands as Sicca Syndrome or with internal organ involvement.

Clinical Types PRIMARY SS - Alone.

SECONDARY SS - associated underlying connective tissue diseases (RA / SLE / Scleroderma )

SICCA SYNDROME – Xerophthalmia + Xerostomia – Internal Organ / Bone Inv

Signs and symptoms

The hall mark symptom of this disease is generalized dryness. Dryness of :

Mouth (Xerostomia) Eye (Keratoconjuctivitis sicca)

Signs and symptoms Teeth – multilpe carries and early loss

Signs and symptoms

Chronic oral candidiasis is frequent. Parotid Gland Enlargement

Signs and symptomsSKIN MANIFESTATIONS (50%)Xeroderma, pruritus and scaling

Annular erythema, Papular Erythema including Sweet’s-like lesions.Raynaud’s syndrome

Hyperglobulinemic Purpura Vitiligo

Sweating abnormalities Cutaneous Amyloidosis Alopecia—diffuse and generalized

Signs and symptoms

Other ManifestationsJoint symptoms: Arthralgia and arthritisMyalgia and myositisENT : Sinusitis / Hearing LossGI : GERD , Esophageal spasm and dysmotility , Celiac diseaseResp : Interstitial pneumonitis, pulmonary fibrosis and pulmonary hypertension xerotracheaNephro : Interstitial nephritis, Renal Tubular AcidosisNeuro : migraine, neuropathies, cerebral vasculitis Risk of lymphoma is 44 times greater than the general population.

Diagnosis

SS patients of both primary and secondary Sjögren’s syndrome have marked hypergammaglobulinemia (IgG>IgA>IgM),ANA(>50%) elevated total protein and sedimentation rate.

Anti-Ro and Anti-La Antibodies occur in approximately 60% of patients with Sjögren's syndrome

Histolgy of skin shows an absence of sebaceous glands and decrease in the sweat glands

DiagnosisBiopsy of labial salivary glands

lymphocytic and plasma cells infiltrate,Two excretory ducts and 3 mucous salivary gland acini are seen

DiagnosisSCHIRMER’S TEST

This test consists of placing a small strip of filter paper inside the lower eyelid (conjunctiva sac). The eyes are closed for 5 minutes. The paper is then removed and the amount of moisture is measured. <5 mm in 5 minutes is positive for SS

Rose Bengal Dye Test

Rose Bengal (4,5,6,7-tetrachloro-2',4',5',7'-tetraiodofluorescein) is a stain. Its sodium salt is commonly used in eye drops to stain damaged conjunctiva and corneal cells and thereby identify damage to the eye.

Revised classification criteria for Sjogren’s Syndrome 1. Ocular symptoms : at least one of -

Dry eyes for more than 3 months Sensation of sand or gravel in the eyes Need for tear substitutes more than 3 times a day 2. Oral Symptoms : at least one of – Dry mouth for more than 3 months Recurrently or Persistently swollen salivary glands Need liquids to swallow dry food 3. Ocular Signs –at least one the following two tests positive Schirmer’s test Rose Bengal score

Revised classification criteria for Sjogren’s Syndrome 4. Histopathology: in minor salivary glands, focal lymphocytic sialoadenitis (focus score ≥1).

5. Salivary gland involvement: a positive result for at least one of the following diagnostic tests:

1  Unstimulated whole salivary flow (≤1.5 ml in 15 min) 2  Parotid sialography showing punctate, cavitary, or destructive pattern, without evidence of obstruction in the major ducts 3  Salivary scintigraphy showing delayed uptake, reduced concentration6. Laboratory Abnormality (one must be present)

Anti-SS-A(Ro) or Anti SS-B(La)(more specific) ANA IgM rheumatoid factor

Criteria

For primary SS In patients without any potentially associated disease, primary SS may

be defined as follows: a. The presence of any four of the six items is indicative of primary SS,

as long as either item 4 (Histopathology) or 6 (Serology) is positive. b. The presence of any three of the four objective criteria items (that is,

items 3, 4, 5, 6) For secondary SS

In patients with a potentially associated disease, the presence of item 1 or item 2 plus any two from among items 3, 4, and 5 may be considered as indicative of secondary SS

Treatment for dry skin

There is no surgery available for treating dry skin. Some moisture providing Substitutes are: Heavy moisturizers. Use of sunscreen with at least SPF 15.

Treatment for dry eyes

Artificial tears.To provide moisture to the

eyes.Cyclosporine eye drops

reduce inflammation of tear

glands.

Moisture chamber spectacles.

Surgery for eyes

Plugging of tear ducts It reduces the mount of tears

drained from the eye. Collagen or silicone plugs are

inserted into the ducts for a temporary closure.

Collagen plug eventually dissolves but silicone plug stays until they fall out or are removed.

Halitosis

Halitosis Halitosis is a general term used to define an unpleasant

or offensive odour emanating from the breath regardless of whether the odour originates from oral or non-oral sources

Originates from two Latin words Halitus → breath Osis → disease

odur originates from oral or non-oral sources Halitosis is a crippling social problem with a common

complaint of up to one-third of the general population

Terminology

Classification

• Genuine halitosis Physiologic halitosis

Pathologic halitosis

(i) Intra-oral (80-90%)

(ii) Extra-oral (10-20%)

• Pseudo halitosis

• Halitophobia.

Genuine halitosis

Physiological halitosis

Morning breath odour, tobacco smoking & certain foods & medications.

Pathological halitosis

intra oral or extra oral origin

90% of patients → oral cavity

Bacteria, volatile sulphur compounds.

Intra oral origin poor oral hygiene, dental caries, periodontal diseases in particular

NUG, NUP, periodontitis, pericoronitis, dry socket, other oral infections, tongue coating & oral carcinoma.

The role of tongue coatings in the aetiology of oral malodour has been

extensively documented.

Tongue coatings include desquamated epithelial cells, food debris,

bacteria and salivary proteins and provide an ideal environment for the

generation of VSCs and other compounds that contribute to malodour

Extra oral origin

10-20%

gastro intestinal diseases

infections or malignancy in respiratory tract

Chronic sinusitis and tonsillitis

stomach, intestine, liver or kidney affected by systemic diseases

Examples of systemic pathological conditions that cause halitosis

Systemic condition Characteristic odour Diabetes mellitus Acetone , sweet fruity.

Renal failure Urine or ammonia

Liver failure Fresh cadaver

Tuberculosis/ lung abscess Foul, putrefactive

Internal hemorrhage/ blood disorders Decomposed blood

Fever , dehydration Odour due to xerostomia and poor oral

hygiene

Pseudo halitosis

Apparently healthy individuals

Halitophobia

exaggerated fear of having halitosis

also referred as delusional halitosis

considered variant of monosymptomatic hypochondrial psychosis.

Etiology

Halitosis generally arises as a result of the bacterial

decomposition of food particles, cells, blood and some chemical

compounds of the saliva.

Moss, 1998

Etiology (Contd.)

Volatile sulphur compounds → hydrogensulphide [H2S, rotten

egg smell], dimethyl sulphide [(CH3)2S, rotten cabbage smell,

and methyl mercaptan [CH3SH, fecal smell].

Non - sulphur containing substances → diamines [cadaverine

(cadaver smell) and putrescine (rotting meat smell), acetone and

acetaldehyde

Etiology (Contd.)

Common causes of halitosis

1) Local Causes

A

Oral disease

sFood

impaction

ANUG

Acute gingivitis

Adult and aggressive periodontiti

s

Pericoronitis

Dry socket

Xerostomia

Oral ulceration

Oral malignancy

B

RESPIRATORY DISEASES

SINUSITIS

TONSILLITIS

MALIGNANCY

BROCHIECTASIS

C VOLATILE FOOD STUFF

GARLIC ONIONS SPICES

2) SYSTEMIC CAUSES

Acute febrile illness

Leukaemias

Respiratory tract infection (usually upper)

Helicobacter pylori infection

Pharyngo-oesophageal diverticulum

Gastro-oesophageal reflux disease

Pyloric stenosis or duodenal obstruction

Hepatic failure (fetor hepaticus)

Renal failure (end stage)

Diabetic ketoacidosis

Trimethylaminuria

Hypermethioninaemia

Menstruation (menstrual breath)

Role of volatile sulphur compounds in the pathogenesis of halitosis

Major compounds implicated in halitosis

VSC’s - Methylmercaptan, Hydrogen sulfide, dimethyl sulfide &

Dimethyl disulfide. Polyamides - Putrescein, Cadaverine, Skatole, Indole. Short chain FA - Butyric, Propionic, Valeric & Isovaleric acid. Others - Acetone, Acetaldehyde, Ethanol diacyl.

It increases the permeability of oral mucosa and crevicular epithelium. It impairs oxygen utilization by host cells, and reacts with cellular proteins, and interferes with collagen maturation.

It also increases the collagen solubility. It decrease the DNA synthesis. It increases the secretion of collagenases, prostaglandins from

fibroblasts. VSC reduce the intracellular pH; inhibit cell growth, and

periodontal cell migration.

Diet +bacteri

a+ epithelial

cells

Peptides/ proteins

Amino acids

Putrefaction products

Oral malodor

Diagnosis

Self assessment testsWhole mouth malodor (Cupped breath)

The subjects are instructed to smell the odor emanating from their

entire mouth by cupping their hands over their mouth and

breathing through the nose. The presence or absence of malodor

can be evaluated by the patient himself/herself.

Wrist lick test

Subjects are asked to extend their tongue and lick their wrist in a

perpendicular fashion. The presence of odor is judged by

smelling the wrist after 5 seconds at a distance of about 3 cm.

Spoon test

Plastic spoon is used to scrape and scoop material from the back

region of the tongue. The odor is judged by smelling the spoon

after 5 seconds at a distance of about 5 cm organoleptically.

Saliva odor test

Involves having the subject expectorate approx. 1-2 ml of saliva

into a petridish. The dish is covered immediately, incubated at 370

C for five minutes and then presented for odor evaluation at a

distance of 4 cm from the examiner’s nose.

Dental floss test

Unwaxed floss is passed through interproximal contacts.

OBJECTIVE TESTS

Organoleptic measurement

Gas chromatography (GC)

Sulphide monitoring

Organoleptic measurement (sniff test)

Organoleptic measurement is a sensory test scored on the basis of

the examiner’s perception of a subject’s oral malodor.

Organoleptic measurement can be carried out simply by sniffing

the patient’s breath and scoring the level of oral malodor.

By inserting a translucent tube (2.5 cm diameter, 10 cm length)

into the patient’s mouth and having the person exhale slowly, the

breath, undiluted by room air, can be evaluated and assigned an

organoleptic score.

The tube is inserted through a privacy screen (50cm-70cm) that

separates the examiner and the patient. The use of a privacy screen

allows the patient to believe that they have undergone a specific

malodor examination rather than the direct-sniffing procedure.

Organoleptic Scores (0- 5) By Rosenberg , Mulloch Et Al 1991

0 - No appreciable odor

1 - Barely noticeable odor

2 - Slight but noticeable odor

3 - Moderate odor

4 - Strong odor

5 - Extremely foul odor

VOLATILE SULFIDE MONITOR

This electronic (Haiimeter, InterScan, Chatsworth, Calif) analyzes concentration of hydrogen sulfide and methyl-mercaptan , but without discriminating between them.

Gas Chromatography (GC):

GC, performed with apparatus equipped with a flame photometric

detector, is specific for detecting sulphur in mouth air.

It measures directly the three VSC methyl mercaptan, hydrogen sulfide

and dimethyl sulfide.

GC is considered the gold standard for measuring oral malodor.

This device can analyze air, saliva, crevicular fluid for a volatile

component.

Electronic nose

Tanaka M et al used these electronic noses to clinically assess oral malodor and examined the association between oral malodor strength and oral health status.

Halitox System:

Quick and simple Detects VSCs and poly amines It detects both VSC and polyamines in the sample.

The absorbent point given with the kit is inserted into the pocket.

Left in place for 1 minute.

Submerge the absorbent point tip in the toxin reagent .

Wait for 5 minutes and see for yellow color in the specimen on

the scale of 0-5, which is directly proportional to the level of

toxins in the sample.

BANA test:

Used to determine the proteolytic activity of certain oral anaerobes that contribute to oral malodor.

PREVENTIVE MEASURES

Preventive measures rather than curative aspects are highly recommended.

Visit dentist regularly

Periodical tooth cleaning by dental professional.

Brushing of teeth twice daily with appropriate brushing techniques and for a

duration of 2-3 mins.

Use of a tongue scraper to get rid of the lurking odour causing bacteria in the

tongue surface.

Flossing after brushing to remove food particles stuck in between the tooth

surfaces.

Limit intake of strong odour species.

Limit sugar and caffeine intake.

Drink plenty of liquids.

Chew sugar free gum for a minute when mouth feels dry.

Eat fresh fibrous vegetables such as carrots.

MANAGEMENT:

Treatment needs (TN) for halitosis have been categorized into 5 classes in order to

provide guidelines for clinicians in treating halitosis patients:

Treatment of physiologic halitosis (TN-1),

Oral pathologic halitosis (TN-1 and TN-2), and

Pseudo-halitosis (TN-1 and TN-4) should be the responsibility of a dentist,

However, treatment of extra-oral pathologic halitosis (TN-3) or halitophobia (TN-5)

should be undertaken by a physician or medical specialist such as a psychiatrist or

psychologist.

(i) Mechanical reduction of intraoral nutrients and

micro-organisms

(ii)Chemical reduction of oral microbial load

(iii) Rendering malodorous gases nonvolatile

(iv) Masking the malodor.

1. Mechanical reduction of intraoral nutrients and micro-organisms- Tongue cleaning- Tooth brush- Inter-dental cleaning- Professional periodontal therapy- Chewing gum

2. Chemical reduction of oral microbial load- Chlorhexidine- Essential oils- Chlorine dioxide- Two-phase oil- water rinse- Triclosan- Aminefluoride/ Stannous fluoride- Hydrogen peroxide- Oxidising lozenges

3.Conversion of volatile sulfide compounds- Metal salt solutions- Toothpastes- Chewing gum

4. Masking the malodor-Rinses

-Mouth sprays

-Lozenges containing volatiles

-Chewing gum

Herbal treatment:

Herbs and essential oils can be made into very effective mouthwash remedies to

sweeten breath and help keep gums and teeth healthy  fennel  not only improves

digestion, but also can reduce bad breath and body odor that originates in the intestines.

Give raw carrots as a midday treat to help scour teeth of bacteria-laden plaque, a

common cause of bad breath. 

Cardamom tea contains cineole, a potent antiseptic that kills bad-breath bacteria and

sweetens breath.

Thymol, one of the constituents of thyme, is contained in antiseptic

mouthwashes. 

 Neem leaf powder can be used as an effective tooth powder to fight plaque

and gingivitis when mixed with astringent herb powders and/or baking soda.

A few drops of Tea tree oil , lemon or peppermint essential oils can be added

to warm water for an effective mouth rinse to freshen breath

During Follow Up

Use of a Confidant Research shows that the patients are generally unable to rate the intensity of

their own halitosis.

-Rosenberg et al 1995 Therefore, the patient cannot reliably assess the effectiveness of the prescribed

therapy. The recommended course of action is to ask them to use another person as a

confidant. A confidant could be a spouse, a family member or a close friend, who is

willing to smell the patient’s breath and provide straightforward feedback.

Treatment Of Oral Submucous Fibrosis

Treatment options

MEDICAL / CONSERVATIVE

SURGICAL

CONSERVATIVE TREATMENT OPTIONS

Steroids Anti inflammatory effect Decrease fibroblastic proliferation and deposition relieved symptoms at an early stage of the disease less useful in reversing the abnormal deposition of fibrotic tissue and

restoring elasticity of the oral mucosa. Significantly better results have been obtained by giving local injections of

Dexamethasone hyaluronidase and chymotrypsin

Dose 40 mg triamcinolone acetonide intralesional per side/10-20 mg per site In weekly divided doses for 5-6 weeks Patient is encouraged to do mouth opening exercises with wooden spatula

for 5 mins atleast 8 times per day

Hyaluronidase

Breaks down hyaluronic acid 1500 IU intra lesional Lowers the viscosity of the intercellular cement substances Decreases collagen formation. Hyaluronidase is much quicker in relief of painful ulceration and

burning sensation than dexamethasone, but the effect is short term

chymotripsinogen

Hydrolysis ester and peptide bonds Proteolytic and anti inflammatory agent 5000 IU twice weekly for 10 weeks

Anti helminthics

Levamisole Immuno modulating effect Scavenges free radicals Recommended dose is 150mg OD for 3 days twice a month×3 month Contraindications include

pregnancy lactating mothers Renal failure cases

Collagenase

Needs evaluation as a single modality treatment

vitamins

Vit A,B,C,D,E These are anti oxidants and protect cells from oxidative damage Vit A 50,000 units once daily oral tablets Oral administration of vitamin A, D, B complex,vitamin E produces -

13.6% improvement in symptoms of OSF Combined with minerals like iron, calcium, copper, zinc and

magnesium produce up to 41% improvement in symptoms Beneficial response in MULTIMICRONUTRIENT THERAPY

Zinc / iron

antagonises tha action of copper Iron supplements are also given to correct anemia because OSF

patients more prone to mucosal injury

lycopene

Synthesized by plants (tomatoes) Powerful antioxidant Has singlet oxygen quenching capability which is :

2 times beta-carotene 10 times alpha-tocopherol.

Daily dose – 16mg First line drug in OSF

pentoxifyllin

Dose upto 1200 mg daily in divided dosage Cause vasodilatation and hence increased mucosal vascularity of OSF Causes neutrophil degranulation and the release of peroxides Promotes natural killer cell activity Decrease production of tumor necrosis factor, and T and B cell

activation. Improvement in mouth opening, tongue protrusion, and relief from

perioral fibrotic bands. Improvement in subjective symptoms of intolerance to spices,

burning sensation of mouth, tinnitus, difficulty in swallowing and difficulty in speech

Nylidrin hydrochloride

peripheral vasodilator Relaxes and dilates the blood vessels,

ensuring greater blood supply to the ischemic tissues helps the nutritional and therapeutic measures reach the effected

tissues.

Buflomedil hydrochloride

Dose 450 mg When used in combination with vitamins+steroids, increased

symptomatic relief

Immune milk

Cow milk immunised from human intestinal bacteria Modulates cytokine function Anti inflammatory 45g immunised milk powder twice a day for 3 months showed :

Improved tolerance to spicy food – 80% Improved mouth opening 70% patients

Interferon gamma

Known anti-fibrotic cytokine Showed

Reduced burning dysaesthesia Increased suppleness of the buccal mucosa Improvement in the mouth opening

Treatment with interferon gamma showed a decreased amount of inflammatory cell infiltrate and an altered level of cytokines compared with the pre-treatment lesional tissue

Placental extracts

Placentrex is an aqueous extract of human placenta that contains Nucleotides Enzymes Vitamins Amino acids Steroids

action is “biogenic stimulation” based on Fitalov’s bio-logical stimulant concept. It has been suggested that it stimulates

the pituitary and adrenal cortex, regulates the metabolism of tissues.

Topical application as well as 2.0cc submucosal injections of aqueous placental extract have been used in past with variable degree of success rate

Ayurvedic treatments

Turmeric oil Turmeric extracts Decreased multinucleated giant cells TEA – tea polyphenols have anti oxidant properties Septillin, a polyherbal ayurvedic preparation in a dose of 2 tablets per

day for 3 months had shown improvement in mouth opening

Laser treatment

Few studies Lasers used are :

CO2 laser KTP 532 laser

SURGICAL TREATMENT

Simple excision (Fibrotomy)

Simple excision can lead to scarring and exacerbation of the symptoms

Split thickness flap

Used in conjunction with coronoidectomy and temporalis myotomy High contracture rate - high recurrence rate

Tongue flap

Bulky Require additional division surgery Bilateral tingue falps can cause :

Dysarticulation Dysphagia Increased risk of aspiration

Involvement of tongue also precludes tongue flap use

Nasolabial flap

Requires second surgery Facial scar

Buccal fat pad

Simple and easy to use Rich blood supply Epithelialisation complete within 6 weeks Morbidity and failure rates low Generally well accepted by the patient

Radial forearm vascularised flap

Bipaddled radial forearm flap Flap length is 8–9 cm and width 2–3 cm typically Bridge pedicle length is 8–10 cm

conclusion

No single drug regimen can provide complete relief in OSF. First and foremost intervention includes intensive counseling and

cessation of the habit. Although reversal of fibrosis is not possible, it is effective in relieving

the symptoms. A tailor made therapy should be designed depending on the extent,

duration and severity of the disease. Here various combinations of the available medications can be tried

to best suit the individual.

Severe or grade III cases may require surgical intervention. Regular follow-up, assessment of improvement in symptoms, inter-

incisal distance, and quality of life must be reported on weekly basis. Keeping in mind the high malignant potential, any ulceration, sharp

cusps of teeth and ill-fitting prosthesis must be looked upon with suspicion.

Although, no established markers have been identified to detect the risk of malignancy in OSF, necessary corrections and histopatho-logical examination should not be delayed whenever in doubt

Moreover, future research needs to be focused on better standardization and follow-up reporting