This document summarizes dental considerations in autoimmune diseases. It discusses the concepts of immunity and autoimmunity. It classifies and describes common autoimmune diseases that can affect the oral cavity, such as Sjogren's syndrome, Mikulicz's disease, aphthous stomatitis, periodontal disease, pemphigus, and bullous pemphigoid. For each condition, it covers etiology, clinical features, diagnosis, and management. The document provides an overview of how autoimmune diseases can impact oral health.

1. Dental considerations in
AUTOIMMUNE DISEASES
Presented by
AMJATH K
MALABAR DENTAL COLLEGE

2. INTRODUCTION
• CONCEPT OF IMMUNITY AND AUTOIMMUNITY
• Human body has got capacity to resist almost all types of organisms or toxins that tend to damage the
tissues and organs.
• Immunity present at birth or as apart of general process-innate immunity
• Immunity aquired against specific invading organism-acquired immunity(b-cell & t-cell mediated)

3. AUTOIMMUNE DISEASES
• Failure of the immune system to tolerate self
tissues.
• It is a condition in which structural or
functional damage is caused by the
immunologically competent cells or antibodies
against normal components of body.

4. Classification of autoimmune diseases
Ferguson A 1995
• 1.ORGAN SPECIFIC AUTOIMMUNE DISEASES
• Hashimottos thyroiditis
• Primary myxedema
• Thyrotoxicosis
• Pernicious anemia
• Addison’s disease
• Type 1 diabetis mellitus
• Myasthenia gravis
• Good pastures syndrome.
• ITP
• Sjogrens syndrome

5. • Non organ specific
• Rheumatoid arthritis
• Systemic sclerosis
• SLE

6. CLASSIFICATION OF AUTOIMMUNE DISEASES AFFECTING OROFACIAL
REGION.
– AUTOIMMUNE DISORDERS AFFECTING OROFACIAL
REGION PREDOMINANTLY
• Sjogrens syndrome
• Benign lymphoepithelial lesion(mikulicz’s
disease)
• Aphthous stomatitis
• Periodontal disease
• Giant cell arteritis

7. Systemic autoimmune diseases with
oral manifestations
• Pemphigus
• Bullous pemphigoid
• Cicatrical pemphigoid
• Epidermolysis bullosa
• SLE
• Myasthenia gravis
• Dermatomyositis
• Systemic sclerosis
• ITP

8. Sjogrens syndrome
Gougerot-sjogren syndrome
• Chronic inflammatory autoimmune disorder
which is charecterized by dimnished lacrimal
and salivary gland secretion(sicca
complex),resulting in keratoconjunctivitis sicca
and xerostomia.

9. • Primary sjogrens syndrome-xerostomia & xerophthalmia.
• Secondary sjorgrens syndrome-triad of
xerophthalmia,xerostomia& a connective tissue
disorder(rheumatoid arthritis,SLE).
• ORAL MANIFESTATIONS
• Unpleasant taste sore mouth
• Difficulty in eating dry food(cracker sign)
• Mouth mirror or tongue blade adheres to buccal
mucosa(tongue blade sign)
• Shed epithelial cells on the labial surface of maxillary
incisors(lipstick sign)
• Parotid enlargement in 80% patients.
• Tongue may appear lobulated ,usually red with partial or
complete depapillation.

10. • Diagnosis of sjogrens syndrome
• Laboratory findings
• 75% of patients with primary sjogrens syndrome
have polyclonal hyperglobulenemia.
• Cryoglobulins
• Antisalivary duct antibodies
• Rhematoid factor
• Antinuclear antibodies.
• Normocytic normochromic anemia
• Leucopenia

11. • Sialometry-salivary flow rate estimation
• Sialography-cherry blossom appearance
• MRI –salt & pepper appearance
• investigation for occular signs
• 1.Schimmers test-check lacrimation
• 2.Ocular staining-cornea stained using rose
Bengal dye & examined microscopically.
• Slit lamp examinationclearly reveals the stained
corneal cells with their devitalised nuclei
• Such stained areas represents corneal damage
from inadequate lacrimation.

12. Management
• Frequent sips of water
• Avoid dry foods
• Sour tasting gums,lime ,candy
• Methyl cellulose(salivary substitute)
• Systemic(mucolytic
agents)..pilocarpine,bromhexine

13. Mikulicz’s Disease
(benign lymphoepithelial lesion)
• Symmetric or bilateral chronic ,painless
enlargement of
lacrimal,parotid,&submandibular salivary
glands attributed to chronic infection.
• Goodwin 1952 introduced the term benign
lymphoepithelial lesion.

14. Etiology
• Exact etiology unknown
• Inflammatory or autoimmune or neoplastic
• Related to sjigrens syndrome in which antisalivary gland antibodies
are produced.
• CLINICAL FEATURES
• Unilateral or bilateral salivary gland enlargement.
• Mild pain,local discomfort, & xerostomia.
• Onset associated with fever,respiratory disorders,oral
infection,tooth extraction etc
• The enlargement in size can be varied but generally the size
increases by a few centimetres.
• Occassionally lacrimal glands are enlarged.

15. • Diagnosis
• Incisional & excisional biopsy
• Histopathology
• Focal infiltrates of small lymphocytes that expands
to replace glandular epithelium.
• Hyperplasia & metaplasia of ductal epithelium.
• Lymphoid follicles & germinal centres may or may
not present.
• Appropriate assesment of patient for the presence
of ocular or systemic components of sjogrens
syndrome.

16. • MANAGEMENT
• Surgical excision or radiation
• Mild cases no treatment
• Some cases swelling regresses,persistent
disease may be treated by surgical excision.
• Radiation is not adviced due to possibility of
radiation induced malignancy.

17. Aphthous stomatitis(aphthous
ulcers,canker sores,recurrent aphthous
stomatitis)• Most common cause of oral ulcerations.
Etiology
Hereditary
Trauma
Dietary deficiency
Psychological
Endocrine
Allergic
Infections
Drugs
Immunological

18. • Elevated levels of IgA & IgG in sera of patients with RAS.
• T-lymphocytes from RAS patients had increased
cytotoxicity to oral epithelial cells.
• Suggests autoimmune origin
• CLASSIFICATION
• 1.minor RAS
• 2.major RAS(suttons disease,periadenitis necrotica mucosa)
• 3.Herpetiform ulcer.
• 4.oral ulcers associated with Behcets syndrome.

19. • CLINICAL FEATURES
• Females
• 10-30yrs
• Onset of disease marked by burning sensation, 2-48 hours before
ulcer develops
• Initially localized area of erythema develops within hours,a small
white papule forms,ulcerates and enlarges in next 48-72hrs.
• Ulcers are usually regular and well defined,rimmed by an
erythematous halo.
• Covered by yellowish gray fibrinous pseudo membrane.
• Usually seen on non keratinized oral mucosa(buccal & labial
mucosa),rare on heavily keratinized palate or gingiva.

20. • MINOR RAS
• Commonest variety
• Round or oval ulcers measures less than 5mm
• Heals within 10-14days without scarring
• MAJOR RAS
• Severe form
• Large painful ulcers, 1cm-3cm
• Lips,soft palate,faucial pillars mostly affected
• Severe pain and dysphagia.
• Pesist upto 6weeks,heals with scarring

21. • Recurrent Herpetiform Ulcers
• Crops of multiple small,shallow ulcers often upto
100 in number.
• Numerous small lesion on intraoral mucosal
surface.
• Begin as pinhead sized erosions that gradually
enlarge & coalse
• More painful than suspected by its size.
• Present almost continuesly for one to three years.

22. • Diagnosis
• Based on history of patients complaint & clinical findings.
• Patients reports of bouts of oral ulceration on mobile oral
mucosal surfaces.
• Lasts for few weeks.
• Patients are healthy inspite of ulceration
• Management
• Mouth rinses(Chlorhexidine gluconate,benzydamine
hydrochloride,betadine)
• Topical steroids(hudrocortisone hemisuccinate
pellets,triamcinalone acetonide in adhesive paste)
• Antibiotics-topical tetracyclines
• Immuno modulators-levimasole

23. Periodontal
diseases
• For almost 2decades the concept of autoimmune
pathogenesis for periodontal disease were considered.
• Alphonse VG etal(1981) detected rheumatoid factor in
subgingival plaque,inflamed gingival tissue,stimulated
pooled saliva & serum of patients suffering from
chronic moderate periodontitis.
• Increased levels of antibodies to type1 collagen in
patients with periodontal diseases.
• All these suggested autoimmunity may contribute to
pathogenisis of this common disease.

24. • Anusaksathien O and Dolby AE (1991) postulated
possible explanations to explain the presence of
autoantibodies in periodontal disease.
• 1.enhanced presentation of self antigens through
increased expression of the molecule associated with
antigen presentation namely Ia antigen.
• Altered T-helper or T-suppressor cells.
• Bacterial or viral cross reactivity with self antigen
leading to production of cross reactive antibodies.
• Genetic predisposing factors

25. systemic autoimmune diseases with
orofacial manifestations
• Pemphigus
• Cicatrical pemphigoid
• Bullous pemphigoid
• Epidermolysis bullosa

26. Etiology
• Immunologic predisposition
• Blisters in pemphigus vulgaris is associated with
binding of IgG (G1 and G4)autoantibodies to
keratinocyte cell surface molecules
• PV antibodies bind to keratinocyte
desmosomes .
• Binding results in loss of cell cell adhesion

27. Etiology

28. • Cicatrical pemphigoid
• Basement membrane zone antigens
• Bullous pemphigoid
• Autoantibodies are directed against
hemidesmosomes
• Epidermolysis bullosa
• Autoantibodies against anchoring fibrils

29. Pemphigus
• Pemphigus vulgaris
• Pemphigus vegetans
• Pemphigus foliaceous
• Familial benign pemphigus
• Paraneoplastic pemphigus

30. Clinical features
• Pemphigus vulgaris -70% cases
• Rapid appearance of vesicles or bullae
• Lesions contain thin watery fluid which later
become purulent
• When bullae rupture they form eroded areas.
• Nikolsky sign-loss of epithelium occassioned by
rubbing apparently unaffected skin.
• Asboe hansen sign-or bullae spread phenomenon

31. • Oral manifestations
• First to come last to go.
• Oral lesion begins as a bullae on a non inflamed base
• Ruptures to form a shallow ulcer with tissue tags on
the margins.
• Common sites are buccal mucosa ,gingiva and palate
• Ulcers extend peripherally over a period of time until
they involve large portions of oral mucosa.
• Distal extension from oral cavity causes involvement of
esophagus,pharynx,and larynx causes dysphagia and
hoarseness of voice

32. • Pemphigus vegetans is an uncommon variant of
pemphigus vulgaris.
• Occurs in 1-2% pemphigus vulgaris
• Two types 1.Neumann type-pustules
• 2.Hallopeaue type-bullae and
erosions.
• Cerebriform tongue –charecterised by a pattern
of sulci and gyri on the tongue

33. • Investigations
• Cytology-TZANK CELLS..
Epithelial cells that are free in vesicular spaces and are
charecterised particularly by degenerative changes
which include swelling of nuclei and hyperchromatic
staining.
Immunofluorescent studies
• In PV the antibody will bind the immunoglobulin
deposits in the intercellular substance and exhibit
positive fluorescent under fluorescent microscope.fish
net pattern of binding

34. management
• Topical therapy
• Painful skin lesions and foul odour managed
by 0.01%pottasium permangnate or 0.5 %
silver nitrate
• Topical corticosteroids or procaine
hydrochloride
• Chlorhexidine mouth rinses

35. Systemic steroids
• Corticosteroids
• A.control phase
• Initial high dose of corticosteroids to a point of clinical
improvement
• Lever suggests 180-360 mg of prednisolone daily for 6-
10 weeks
• B.consolidation phase
• In this phase the dosage is reduced.
• C.Maintanence phase
• Dose gradually tapered down to alternate day dose and
ultimately stopped

36. • Immunosuppressive agents-Azathioprine 100-
200mg in conjunction with prednisolone
• Plasmapherisis-patients who are refractory to
corticosteroids.
• Photopherisis-administration of 8-
methoxypsoralen followed by exposure of
peripheral blood to uv radiation.causing
photoinactivation of WBC
• Immunomodulators –levimasole(100mg/week)

37. Pemphigus foliaceus(superficial
pemphigus or fogo selvagum)
• Benign variety of pemphigus.
• Manifested as early bullous lesions which rapidly
rupture and dry to leave masses of flakes or
scales suggestive of exfoliative dermatitis or
eczema
• Brazilian wildfire pemphigus
• Endemic form of pemphigus foliaceus
• Occurs commonly in children.
• Oral lesions are rare.

38. Paraneoplastic pemphigus
• Anhalt et al first described paraneoplastic pemphigus
in 1990.
• Etiology
Tumour antigens evoke an immune response that leads
to development of an autoimmune response to
intercellular adhesins.
• This autoantibody response leads to blistering in
mucosa and other epithelia.
• Often fatal.

39. • Most common malignancy associated is NON
HODGKINS LYMPHOMA.
• CLL
• GIANT CELL LYMPHOMA
• BRONCHOGENIC SCC
• Oral erosions and ulcers are common

40. Cicatrical pemphigoid
• Benign mucous membrane pemphigoid,ocular
pemphigus
• The word cicatrical means scarring
• Chronic subepidermal blistering and scarring
autoimmune disease with a predilection for
stratified squamous mucous membrane and
occassionally skin.
• Charecterized by vesicles that heals by scar
formation.generally occurs on mucous
membrane of oral cavity and conjunctiva.

41. • Oral lesions have two clinical presentations
• 1.erosions on the non-keratinized
mucosa/keratinized gingiva or desquamative
gingivitis
• Oral lesions have distinct margins..heals by
scarring
• Nikolsky sign positive
• Spontaneous gingival bleeding
Ocular lesions
Sub conjunctival scarring leads to blindness in 15%
patients.

42. Bullous pemphigoid
• Parapemphigus
• Rarely involves mucous membrane
• Elderly people
• Appears as rashes commonly on limbs urticarial
or eczematous.
• Remains for several weeks before appearance of
vesicles and bullae.
• Vesicles are thick walled and rupture occurs
rarely

43. • Oral lesions
• Small bullae,rarely painful
• Buccal mucosal gingiva more commonly involved.
• Generalized edema and inflammation of gingiva.
• Remissions are common
• Management
• Use of systemic steroids

44. Epidermolysis bullosa
• Group of inherited bullous disorders
charecterized by blister formation in response
to mechanical trauma.
• 3 types of presentation
• Classical
• Bullous pemphigoid
• Cicatrical pemphigoid

45. • Classical presentation is non inflammatory bullous
disease heals with scarring
• Patients have erosions blisters and scars over trauma
prone surfaces.
• The bullous pemphigoid like presentation is
widespread inflammatory vesiculobullous eruption
involving the trunk,central body,skin folds and
extremities.
• 10% patients exhibit severe mucous membrane
involvement may present a picture clinically similar to
cicatrical pemphigoid with erosions and scarring in the
oral cavity,conjunctiva,upper oesophagus anus and
vagina

46. • Treatment
• Prevention of trauma
• Antibiotics for secondary infection

47. Systemic lupus erythematosis
• Autoantibodies ,immune complex formation and
immune dysregulation resulting in damage to any
organ including kidney,skin,bloodcells,and CNS
• Etiology
• Genetics
• Hormones
• Environment all these leading to immune
dysregulation.
•

48. Clinical features
• Low grade fever and malaise
• Erythematous rash over malar region,refered
to as butterfly rashes
• Pain on joints,rheumatoid arthritis.
• Renal involvement-nephritis
• Cardiopulmonary-pleuritic chest pain
• CNS-neuropathy,sensory motor incoordination
• GIT-nausea,vomiting,anorexia.

49. • Oral lesions
• Multiple white plaques with dark reddish purple
margins
• Hyperemia and edema are marked
• Bleeding and superficial ulceration
• Xerostomia
• Glossitis
• Dental caries
• periodontitis

50. Management
• Use of corticosteroids
• Dental consideration
• Platelet count measured before oral surgical
procedures
• Prophylaxis against bacterial endocarditis

51. Bibilography
• Shafers textbook ot oral pathology
• Burkets oral medicine