Oral Manifestations of Connective Tissue Disease (CTD)Group B

Mohamad Aiman | Che Ku Farah Najwa | Fatin NabihahLim Shuang Ying | Chan Yunn Yee | Aifa Nadhirah

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

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.

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.

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

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

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.

Chronic palatal lesion in SLE

Discoid lupus lesion on the lower lips

Hard palate ulcer in SLE

Discoid lupus lesion on buccal mucosa

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

Other Features and Complication

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.

• Radiographic finding of TMJ: joint effusion, disc displacement and condylar abnormalities.

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.

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.

Open anterior bite in patient with Juvenile Rheumatoid Arthiritis

Furcation involvement in RA

Increase of pocket depths in RA

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)

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)

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.

Minor aphthous ulcer

Major aphthous ulcer Herpetiform ulcer

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

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

Strawberry tongue

Cracking and bleeding lips

Erythema lips

Kawasaki disease

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

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

• 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

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

• 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)

Mixed Connective Tissue Disease

• MCTD is an overlapping syndrome of SLE, SSc, RA and polymyositis.

• Presence of a distinctive antibody U1-ribonucleoprotein (RNP)

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).

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

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.