Autoimmune diseases


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Autoimmune diseases

  1. 1. Dental considerations in AUTOIMMUNE DISEASES Presented by AMJATH K MALABAR DENTAL COLLEGE
  2. 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. 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. 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. 5. • Non organ specific • Rheumatoid arthritis • Systemic sclerosis • SLE
  6. 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. 7. Systemic autoimmune diseases with oral manifestations • Pemphigus • Bullous pemphigoid • Cicatrical pemphigoid • Epidermolysis bullosa • SLE • Myasthenia gravis • Dermatomyositis • Systemic sclerosis • ITP
  8. 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. 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. 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. 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. 12. Management • Frequent sips of water • Avoid dry foods • Sour tasting gums,lime ,candy • Methyl cellulose(salivary substitute) • Systemic(mucolytic agents)..pilocarpine,bromhexine
  13. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 25. systemic autoimmune diseases with orofacial manifestations • Pemphigus • Cicatrical pemphigoid • Bullous pemphigoid • Epidermolysis bullosa
  26. 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. 27. Etiology
  28. 28. • Cicatrical pemphigoid • Basement membrane zone antigens • Bullous pemphigoid • Autoantibodies are directed against hemidesmosomes • Epidermolysis bullosa • Autoantibodies against anchoring fibrils
  29. 29. Pemphigus • Pemphigus vulgaris • Pemphigus vegetans • Pemphigus foliaceous • Familial benign pemphigus • Paraneoplastic pemphigus
  30. 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. 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. 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. 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 net pattern of binding
  34. 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. 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. 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. 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. 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. 39. • Most common malignancy associated is NON HODGKINS LYMPHOMA. • CLL • GIANT CELL LYMPHOMA • BRONCHOGENIC SCC • Oral erosions and ulcers are common
  40. 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. 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. 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. 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. 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. 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. 46. • Treatment • Prevention of trauma • Antibiotics for secondary infection
  47. 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. 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. 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. 50. Management • Use of corticosteroids • Dental consideration • Platelet count measured before oral surgical procedures • Prophylaxis against bacterial endocarditis
  51. 51. Bibilography • Shafers textbook ot oral pathology • Burkets oral medicine