Autoimmune Connective Tissue Disease

Background

• Chronic, inflammatory autoimmune disease
• “Autoantibodies and immune complexes set off an array of immunological reaction, resulting in activation of complement system = vasculitis, fibrosis and tissue necrosis”
• Remission and exacerbation
• Incidence has tripled in the last 50 years
• Survival rates have increased

Medical Management

• Health History
• Prednisone
• Anti-inflammatories
• Aspirin
• Acetaminophen
• NSAIDs

• Identification:
• Small vessel vasculitis- can lead to major organs breaking down
• Joint pain
• Rash (young adult women)
• Family history
• Oral ulcers, lesions- located on the lips or mucosa (striae)
• Environmental triggers
• Stochastic events
• Immune response=tissue damage

Dental Management

• Defer treatment is patient is experiencing a flare up
• Oral Conditions:
• Ulcers
• Xerostomia
• Burning sensation
• Keratosis- vermilion, gingiva, buccal mucosa and palate locations (sometimes confused with linchen planus)
• Screen for leukopenia
• Consider antibotics

Medical Management

• Identifying:
• Appearance of hands, feet, other joints
• 4 Stages (early, moderate, severe, terminal)
• Many tests- blood serum
• 3 Courses of the disease (monocyclic, polycyclic, progressive)
• Treatment:
• Corticosteroids
• NSAIDs
• Immunosuppressants

Dental Management

• Oral Conditions:
• Xerostomia
• Stiff TMJ
• Uncontrolled plaque
• Treatment:
• Oral hygiene instructions
• Diet education
• Antimicrobial mouth rinse- Pg is most likely present
• Will need pillows during tx
• TMJ- give them breaks
• Tools to work with dexterity issues- big handle toothbrushes, floss holder etc.
• Salivary substitute- for xerostomia
• Fluoride tx- high caries risk

Medical Management

• Identifying:
• No symptoms
• Possibly presentation of fatigue, xerostomia, HBP, UTI, Jaundice, Pain
• Blood Tests--elevated serum
• Treatment:
• Cyclosporine
• Immunosuppressive
• Liver transplant

Dental Treatment

• Oral Conditons:
• Xerostomia
• Gingival bleeding
• Petechiae or ecchymosis
• Lichen planus
• Attrition/erosion
• Candidiasis
• Treatment:
• Potential bleeding tendency- take precaution
• INR may be needed
• Use LA precaution- may use live dysfunction

Medical Management

• Identifying:
• Fatigued/agitated
• Widespread pain 3 months +
• Stiffness
• Cognitive difficulties
• Unexplained symptoms
• Treatment:
• Skeletal-muscle relaxants
• Opioids
• Hypnotics
• Psychological therapy

Dental Management

• Oral Conditions:
• TMJ stiffness
• Slow healing- anxiety and depression
• Treatment:
• Temporomandibular disorder- pain moving joint, or lack of movement of joint
• Jaw fatigue
• Give breaks often
• Drug interactions
• Xerostomia- caries, salivary sub. Etc.

Background

• Skin thickening- tissue fibrosis and inflammation within major organs (fibroblasts and endothelium)
• Triggers cytokines and autoantibodies
• 49,000 US adults
• 30-50 years old
• More women than men (4:9)
• Endothelial cell injury, fibroblast activation
• Cellular and humoral derangement
• Vascular malfunction is an early sign (systemically)
• Also, Sjogren's Syndrome

Medical Management

• Identifying:
• Localized- skin on hands and face and it’s slow progressing
• Systemic- skin and major organs
• 1. CREST Syndrome- limited disease, calcinosis (disposition of calcium), Raynaud’s syndrome (inadequate blood flow)
• 2. Diffuse disease
• Symmetric thickening of skin in the fingers
• Limited mobility
• Sclerodactyly, loss of digital pad
• Treatment:
• immunosuppressive

Dental Management

• Oral Conditions:
• Stiff TMJ
• Microstomia- limit mouth opening
• Oropharyngeal cancers
• PDL widening
• Treatment:
• Narrow mouth- difficulty accessing molars
• Masticatory dysfunction
• Xerostomia- fluoride, short recalls etc.

Background

• Blistering that effects stratified squamous epithelium
• Results in cutaneous or mucosal blistering
• Less than 0.5 patients/ 100,000
• Several variants
• Idiopathic
• Diet or drug trigger
• Genetic
• Autoantibodies that target the extracellular adhesion components

Clinical Features

• Oral mucosa is affected at an early stage
• Blisters lead to chronic erosions and ulcers on buccal mucosa, palate, ventral surface of the tongue, and lips
• Erostive gingivitis
• Oral lesions are followed by lesions on the skin
• May be associated with other autoimmune disorders (RA, SLE, Sjogren’s)

Diagnosis

• Firm sliding pressure separates normal looking epithelium
• (Nikolsky’s sign)—not sensitive
• Biopsy is needed
• Serum antibody titers

Treatment

• Aimed at controlling, not fully treating
• Lowest drug dose to prevent disease activation
• Cyclosporine
• Dapsone
• Tacrolimus
• Rituximab

Background

• Chronic, subepithelial blistering disease
• Localized or extensive
• Mucosal and cutaneous surfaces
• 2-5 per 100,000 population a year
• Twice as often in women as men
• Middle aged and older adults
• Autoantibodies attack antigen sites in the molecules connecting the epithelium to the CT and prevent linage of molecules to hemidesmosomes

Clinical Features

• Mucosal site—oral and conjunctival mucosa
• 85% has oral involvement without skin involvement
• Lesions on the gingiva, palate, buccal mucosa—less often on tongue and lips
• The gingiva presents with painful erythematous and tender erosions with toothbrushing
• Inability to maintain oral health with heavy accumulation of plaque
• Small vesicles that rupture easily
• Unlike PV, they are long lasting and well-defined

Treatment

• Management depends on the severity
• Widespread (eye, throat, or skin) needs a specialist
• Oral lesions may be treated with a topical or intralesional corticosteroid
• More severe cases involve systematic corticosteroids, dapsone, tacrolimus
• Excellect oral hygiene to reduce plaque

Citations

• Patton, Lauren. Glick, Michael. Patients with Medical Conditions. Hoboken, NJ. John Wiley & Sons. 2016.
• Mease, Phillip. Fibromyalgia syndrome: review of clinical presentation, pathogenesis, outcome measures, and treatment. Journal of Rheumatology. August 2015.
• Patton, Lauren. Scleoderama: oral manifestations and treatment challenges. SDC. August 2000.
• Darby, Michele L. Walsh, and Denise M. Bowen. Dental Hygiene: Thoery and Practice. St Louis, MO: Elsevier/Saunders, 2015.