Oral Manifestations of

Connective Tissue
Disease (CTD)
Group B
Mohamad Aiman | Che Ku Farah Najwa | Fatin Nabihah
Lim Shuang Ying | Chan Yunn Yee | Aifa Nadhirah

Contents
Introduction
Connective Tissue Diseases with Oral Manifestations

Systemic Lupus Erythematous

Rheumatoid arthritis
Vasculitides (Bechets Disease, Kawasaki Disease)
Systemic Sclerosis
Sjorgens Syndrome
Mixed connective Tissue Disease

References

Connective Tissue
Diseases
Diseases that involved the bodys 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)

Butterfly rash

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)

Manifestations of SLE
Skin & Joints
Serous membranes
Cardiovascular

Rash
Arthritis & Polyarthralgia
Pericarditis
Pleurisy
Endocarditis
Myocarditis
Raynaud's syndrome

Lungs

Pneumonitis

Kidney

Nephritis

Neurological

Eyes
Git
Blood

Cranial nerve palsy

Neuroses
Strokes
Conjuctivitis
Retinal damage
Hepatosplenomegaly, Pancreatitis
Sjogren's syndrome
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

Increase of pocket depths in RA

Furcation involvement 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 (Bechets 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 punchedout, 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
fever5days

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

Ka
wa
sa
ki
dis
ea
se

Strawberry tongue

Erythema lips

Cracking and bleeding

lips

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

Sjogrens 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: Raynauds phenomenon, lung infections,
fibrosis, renal disease, pancreatitis, thyroiditis,

 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 Sjogrens 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 U1ribonucleoprotein (RNP)

Clinical Manifestations
1. Swollen (edema) hands
2. Raynauds 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 Raynauds Phenomenon).

Oral Manifestations

Dry mouth
Oral mucasal ulceration
Focal sialadenitis (90%)
Painful swollen salivary gland
Redness of the overlying skin
Difficulty in opening the mouth
Fever
Swelling of the lymph nodes in the neck region

References
Cawson, Odell. Cawsons 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.