Oral Manifestations of Connective Tissue Disease (CTD
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Transcript of Oral Manifestations of Connective Tissue Disease (CTD
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Oral Manifestations of Connective Tissue Disease (CTD)Group B
Mohamad Aiman | Che Ku Farah Najwa | Fatin NabihahLim Shuang Ying | Chan Yunn Yee | Aifa Nadhirah
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Contents• Introduction• Connective Tissue Diseases with Oral Manifestations
• Systemic Lupus Erythematous• Rheumatoid arthritis• Vasculitides (Bechet’s Disease, Kawasaki Disease)• Systemic Sclerosis• Sjorgen’s Syndrome• Mixed connective Tissue Disease
• References
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Connective Tissue Diseases• Diseases that involved the body’s connective tissues as the
primary target of pathology.• They represent a spectrum of disorders, from single to
multiple organ systems due to systemic autoimmune dysfunction or dysregulation.
• Connective tissues include connective tissue proper, cartilage, bone tissues and blood.
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Systemic Lupus Erythematous• Lupus : wolf (Greek) disease bites the tissue. • SLE is defined as an autoimmune disease characterized by
antibodies that may initiate immune complex reactions, resulting in multisystem disease.
• Susceptibility related to • immune system genes : HLA-DR3 and B8, HLA-DR2• complement C4 genes• polymorphism of T-cell receptor.
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Clinical features• Onset :
• acute, resembling infection• vague symptoms may progress over the years.
• Arthralgia & myalgia• Skin, mouth, hair
• malar rash ( butterfly rash)• Photosensitivity rash ( rash on skin exposed to sunlight)• Oral ulcer• Alopecia ( hair loss)
• Anaemia• Pericardium inflamed, causing
• Pleurisy (pains in the side of chest)• Pericarditis (central chest pain)
• Myocarditis - leads to cardiac failure. Increase risk of infarction. Endocarditis can develop.• Kidney inflamed proteinuria & haematuria (assoc. w. hypertension)• Minor disturbance of mood and depressive or hysterical behaviour• Women w SLE can pass antibodies across placenta (fetal heart block)
Butterfly rash
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Manifestations of SLESkin & Joints • Rash
• Arthritis & Polyarthralgia
Serous membranes • Pericarditis• Pleurisy
Cardiovascular • Endocarditis• Myocarditis• Raynaud's syndrome
Lungs • Pneumonitis
Kidney • Nephritis
Neurological • Cranial nerve palsy• Neuroses• Strokes
Eyes • Conjuctivitis• Retinal damage
Git • Hepatosplenomegaly, Pancreatitis• Sjogren's syndrome
Blood • Anaemia & purpura
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Dental Aspect [SLE]• Oral mucosa lesion seen in 20% of patients• May resemble oral lichen planus lesions.
• 2 predominant types :• Oral ulcer
• Usually occur on palate and oropharynx• Characteristically painless
• Discoid lesion• Similar to those occurring in skin (whitish striae radiating from central
erythematous area –brush border)• Common affected site :buccal mucosa, gingiva, labial mucosa
• Surgery/ extraction may exacerbate the symptom• Bleeding tendency• Antimalarial drug used in SLE may cause oral mucosal melanin
pigmentation.
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Chronic palatal lesion in SLE
Discoid lupus lesion on the lower lips
Hard palate ulcer in SLE
Discoid lupus lesion on buccal mucosa
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Rheumatoid Arthritis• A multisystem disease, but join pain and damage are the most
prominent features.• It is immunologically mediated;
- An abnormal immunoglobulin is form in the join tissue and an auto-antibody to the abnormal immunoglobulin (Rheumatoid factor, RF) is produced in response. - Immune complex (antigen-antibody complex) formation may then lead to the activation of inflammation and lead to synovial damage.
• Women are affected 3:1 men• Onset are typically between age 30-40
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Other Features and Complication
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Orafacial Manifestations
1. Temperomandibular Joint (TMJ) Involvement
• Due to synovial inflammation and connective tissue degeneration
• Can cause bilateral; deep, dull, aching pain exacerbation during movement of TMJ.
• Including tenderness and swelling in the periauricular region, limitation of mandibular range of movement and clicking and tenderness of masticatory muscle.
• Limitation in mouth opening as the disease worsening, anterior open bite due to loss of condylar height and class II malocclusion with heavy posterior occlusal contact.
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• Radiographic finding of TMJ: joint effusion, disc displacement and condylar abnormalities.
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2. Periodontal Disease• Increase in pocket depths• Furcation involvement• Loss of alveolar bone and tooth loss• Limitation on oral hygiene due to arthritic involvement of hands
and limiting opening.
- This all happen due to the connection of inflammatory process in joint and oral tissue.
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3. Xerostomia and Decrease In Salivary Flow
• Occurs in Rheumatoid Arthritis patient with Sjogren Syndrome.
• Leads to: difficulty in swallowing & speaking oral soreness & burning sensation difficulty in function with oral prosthesis increase risk for caries.
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Open anterior bite in patient with Juvenile Rheumatoid Arthiritis
Furcation involvement in RA
Increase of pocket depths in RA
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Vasculitis/Vasculitides• Vasculitis – a group of clinical syndromes characterized by
inflammation that predominately involves the blood vessels of unknown etiology.
• According to size of the vessels affected, it can be classified into:• Large vessel• Medium vessel (Kawasaki Disease)• Small vessel (Bechet’s Disease)
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Behcet's Disease• An auto inflammatory disease resulting in damage to blood
vessels throughout the body.• Common in men, onset: middle 20-50 y/o• Most common symptoms:
• Oral ulcerations• Genital ulcerations• Inflammation of the internal portions of the eye (uveitis)
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Oral Manifestations • Oral ulceration tends to be the earliest manifestation of
Behcet's Disease.• Can be major, minor or herpetiform ulcers. • Predominantly found on non-keratinized mucosa (tongue, lips,
buccal mucosa, gingiva)• Indistinguishable from common apthous ulcer (more
extensive, more painful, more frequent and evolve quickly from a pinpoint flat ulcer to a large sore)
• Lesions can be shallow or deep, and usually have a central, yellowish, necrotic base and a punched-out, clear margin.
• Appear singly or in crops, persists in 1-2 weeks, and subside without leaving scars.
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Minor aphthous ulcer
Major aphthous ulcer Herpetiform ulcer
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Kawasaki Disease• Aka mucocutaneous
lymph node syndrome.• Idiopathic multisystem
disease characterized by vasculitis of small and medium blood vessels.
• Unknown aetiology, occurs in children <5 years.
• Characteristics: • Conjunctivitis• Rash• Cervical
lymphadenopathy • Strawberry tongue • Changes in peripheral
extremities• High grade
fever≥5days
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Oral Manifestations
• Erythema, dryness, fissuring, peeling, cracking and bleeding of lips
• “Strawberry tongue” it is indistinguishable from that associated with streptococcal scarlet fever with erythema
• Prominent fungiform papillae• Diffuse erythema of the oropharyngeal mucosa
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Strawberry tongue
Cracking and bleeding lips
Erythema lips
Kawasaki disease
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Systemic sclerosis (Scleroderma)
• ‘skleros’ = hard, ‘derma’= skin• Characterised by :
• sclerotic skin changes (subcutaneous and submucous fibrosis) that are often accompanied by multisystem disease.
• Progressive stiffening of skin.• Associated with antibodies.
• Female predominance (F:M = 4:1)• Onset : 30-50 years of age
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Oral Manifestations
• Periodontal involvement• Widening periodontal ligament space (<10%)• No tooth mobility• Increaseing pocket depth• Gingivitis
• Tongue involvement• Due to involvement of oral submucosa• Become stiff/rigid• narrowed
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• Temporomandibular Joint Dysfunction + Microstomia• Limit mouth opening (due to fibrosis)• Gross change in mandibular• Resorption of condyle, angle and body of mandible
• Xerostomia• 25 % cases• With/ without xeropthalmia
• Lips involvement• Constricted oral orifice (fish mouth)• Pursed with radiating furrows
• More decayed, filled or missing teeth
80% patients have manifestations at head and neck regions, 30% starts from here
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Sjogren’s Syndrome• Etiology :
• Chronic inflammatory disease of unknown etiology characterized by lymphocytic infiltration and destruction of exocrine
• Destruction of exocrine gland can cause absence of physiologic secretions, dryness of mucus membranes and conjunctiva, keratoconjunctivitis sicca, xerostemia, and other manifestation.
• Sign/Symptoms :• Mucosal dryness• Eyes: Dry, burning, red eyes(photosensitivity)• Mouth: Dysphagia, enlarged salivary glands, cavities• Systemic: Raynaud’s phenomenon, lung infections, fibrosis, renal
disease, pancreatitis, thyroiditis, athralgias, myositis
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• Treatment :• No specific cure• Treat dry eyes with ophthalmic drop PRN• Emergent referral for patient with cornea ulceration• Dry mouth: Pilocarpine; maintain excellent oral hygiene to avoid
cavities• Treat athralgias with hydroxychloroquine• May need steroids for systemic symptoms
• Oral manifestations of Sjogren’s Syndrome:• Mouth: Dysphagia, enlarged salivary glands, cavities• Decreased salivary flow• Xerostomia• Burning of tongue (glossodynia)
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Mixed Connective Tissue Disease
• MCTD is an overlapping syndrome of SLE, SSc, RA and polymyositis.
• Presence of a distinctive antibody U1-ribonucleoprotein (RNP)
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Clinical Manifestations
1. Swollen (edema) hands2. Raynaud’s phenomenon (particular series of discolorations of
the fingers and/or toes after exposure to changes in temperature)
3. Synovitis > joint pain (arthralgia)4. Myositis > muscle pain (myalgia)5. Acrosclerosis (special form of Scleroderma characterized by
skin thickening and stiffness affecting the extremities, head and face and is associated with Raynaud’s Phenomenon).
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Oral Manifestations • Dry mouth • Oral mucasal ulceration• Focal sialadenitis (90%)
Painful swollen salivary glandRedness of the overlying skinDifficulty in opening the mouthFeverSwelling of the lymph nodes in the neck region
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References• Cawson, Odell. Cawson’s Essentials of Oral Pathology and Oral
Medicine. 8th Edition. Elsevier.• Kumar, Clark. Kumar&Clark Clinical Medicine. 6th Edition.
Elsevier Saunders.• Scully, Cawson. Medical Problems in Dentistry. 5th Edition.
Elsevier.