Systemic Lupus Erythematous and Scleroderma (Crest Syndrome)

1. Systemic Lupus Erythematousand Scleroderma (Crest Syndrome) Arodis Arias Michelle Enriquez Kimberly Goris

2. Systemic Lupus Erythematous

3. Systemic Lupus autoimmune disease. • Causes immune system to make antibodies that attack the body’s healthy cells and tissues. • Inflames: • Joints and muscle pain • Tendons • Skin • Other connective tissues and organs like the heart • Symptoms include: • Fatigue • Malaise • Hair loss • Oral sores • Butterfly rash

4. Lupus Symptom: Butterfly rash

5. Etiology • Systemic Lupus Erythematous is unknown. Studies suggest it may be a combination of: • Genetic factors • Environmental factors, which may include: sunlight, (UV rays) • Stress • Viral • Drug induced (methyldopa, procainamide, isoniazid, chlorpromazine, TNF-blocking drugs).

6. Types of Lupus • Systemic Lupus - most common • Cutaneous Lupus – “Discoid Lupus” • Drug Induced: occur in 5% of people with Lupus • Apresoline • Quinidine • Isoniazid • Dilantin

7. Lupus Symptom: Hair Loss

8. Risk Factors • Sex: female to male 10:1 • Age: 20-45 • Race: African American, Native America, Asian and Hispanic.

9. Treatment of Cutaneous SLE • No cure. • Goal of treatment is to control symptoms. • Photo protection: avoid direct sun exposure. • Sunscreens containing dioxide or zinc oxide.

10. Topical Corticosteroids Triamcinoloneacetonide 0.1% Clobetasol propionate 0.05% • Brand Name: Kenalog®, Oralone®, Pediaderm® • MOA : decreases inflammation by suppression of migration of polymorphonuclear leukocytes and reversal of increase capillary permeability, suppress the immune system by reducing activity and volume of the lymphatic system. • Adverse effects: Frequency not defined. • Drug Interaction: Avoid concomitant use with Aldesleukin. • Contraindication: Hypersensitivity to triamcinolone or any component of the formulation; fungal, viral or bacterial infections of the mouth or throat. • Effects on Dental Treatment: ulcerative esophagitis, perioral dermatitis, atrophy of the oral mucosa, burning and irritation.

11. Oral Corticosteroids Prednisone • Brand name: PredniSONEIntensol™ • MOA : decrease inflammation. Inmunosuppresion, adrenal function suppresion at high doses. • Adverse effects: Temporary effects and likely dose related adverse effects like osteoporosis and adrenal suppression. • Drug Interaction: • Avoid ethanol • Prednisone interferes with calcium absorption. Limit caffeine • St. John’s wart may decrease prednisone levels. • Contraindications: Hypersensitivity to any component of the formulation; systemic fungal infections; administration of live attenuated vaccines with immunosuppressive doses of prednisone. • Effects on Dental treatment: No significant effects or complications reported.

12. Topical Calciuneurin Inhibitors Tacrolimus (Protopic® 0.1%) Pimercrolimus • MOA: suppress cellular immunity. (Inhibits T-lymphocyte activation) • Adverse effects: CNS: headache; Dermatologic: skin burning; Respiratory: increased cough; Cardiovascular: peripheral edema; Gastrointesntinal: diarrhea; Ocular: conjuctivitis. • Drug Interaction: avoid use of Immunosuppresants and alcohol. Localized flushing (redness, warm sensation) may occur at applicationsite of topical following ethanol consumption. • Contraindications: hypersensitivity • Effects on Dental Treatment: No significant effects or complications reported.

13. Antimalarials Hydroxychloroquine (Plaquenil®) Aminoquinolone • MOA: Interferes with digestive vacuole function within sensitive malarial parasite. • Adverse effects: Frequency not defined. Cardiovascular: cardiomyopathy; CNS: ataxia, dizziness, emotional changes; Dermatologic: alopecia; Gastrointestinal: abdominal cramping, anorexia, diarrhea, nausea. • Effects on Dental Treatment: no significant effects or complications reported.

14. Immunosuppressants AzaTHIOprine (Azasan®; Imuran®) Methotrexate (Rheumatrex®; Trexall®) Cyclophosphamide (Cytoxa) • MOA: antagonizes purine metabolism and may inhibit synthesis of DNA, RNA and proteins; may also interfere with cellular metabolism and inhibit mitosis. • Adverse effects: frequency not always defined; dependant upon dose, duration, indication, and concomitant therapy. • Drug interactions: avoid conccomitant use with BCG; Febuxostat; Mercaptopurine. • Contraindication: hypersensitivity to azathioprine or any component of the formulation. • Effects on Dental Treatment: No significant effects or complications reported.

15. B-cell Suppresor RiTUXimab (Rituxan®) Belimumab (Benylsta®) • MOA: Its an IgG1-lamba monoclonal antibody that prevents survival of B lymphocytes. Reduces the activity of B-cell mediatoted immunity and the autoimmune response. • Adverse effects: Gastrointestinal: nausea; CNS: fever, insomnia, migrane, depression; Respiratory: bronchitis. • Effects on Dental Treatment: No significant effects or complications reported.

16. NASAIDs • MOA: Management of pain and swelling. • Adverse effects: Cardiovascular: edema; CNS: dizziness; Dermatologic: rash; Endocrine: fluid retention. • Drug interaction: May increase levels of Anticoagulants; Antiplatelet Agent; Bisphosphonate derivatives; Collagenase. • Contraindication: associated with an increased risk of adverse cardiovascular thrombotic events, including fatal MI and stroke. • Effects on Dental Treatment: Ibuprofen can interfere with the antiplatelet effect of low-dose aspirin (81mg/day), diminishing the effectiveness of aspirin as used for cardioprotection and stroke prevention.

17. Scleroderma (Crest Syndrome)

18. A connective tissue disease that involves changes in the: • Skin • Blood vessels • Muscles • Internal organs • An autoimmune disorder when the autoimmune system attacks and destroys healthy body tissues

20. Reynaud’s phenomenon

21. Sclerodactyly

23. Telangiectasia

24. Treatment

25. Medications • Calcium channel blockers used for Reynaud’s phenomenon. • Corticosteroids – Antiinflammatory • NSAIDs • ACE Inhibitors MethotrexateImmune suppressing medication Antineoplasticagaent MOA: Ultrasoft acting IV barbiturate anesthetic. Adverse effects: Frequency not defined. Drug Interaction: None known. Contraindication: Hypersensitivity to barbiturates (porphyria). Effects on Dental Treatment: No significant effects or complications reported.

26. Medications continuation CyclophosphamideAntineoplastic agent MOA: Prevents cell division, and potent immunosuppressive. Adverse effects: Dermatologic: alopecia; may cause sterility; nausea, vomiting, anemia, headache, nasal congestion. Drug Interaction: Increased effect on Vitamin K antagonist. Contraindication: severely depressed bone marrow function. Effects on Dental Treatment: mucositis and stomatitis.

27. Dental Hygienist Role • Obtain thorough medical history which include all the medications and side effects. • Maintain patient on strict oral hygiene regimen and recalls. • Recommend powered toothbrush to patient due to lack of dexterity on their hands. • Advise patient to use WaterPick to clean interproximally since the patient may not be able to maneuver the dental floss. • Recommend patient to eat a healthy diet.

28. Questions • 1. What does Lupus affect? • 2. What type of medications are used? • 3. What is the most significant symptom of Scleroderma? • 4. What does Raynaud’s phenomenon affect?

29. Resources You have full text access to this OnlineOpenarticleArthritis & RheumatismVolume 62, Issue 12, Article first published online: 30 NOV 2010 http://www.nlm.nih.gov/medlineplus/ency/article/000435.htm http://www.medicinenet.com/systemic_lupus/page5.htm#what_is_the_treatment_for_systemic_lupus_erythematosus http://emedicine.medscape.com/article/1064663-treatment