Scleroderma (Crest Syndrome) and Systemic Lupus Erythematous

Pharmacology DEN 2315/Thursday class

Arodis Arias

Michelle Enriquez

Kimberly Goris

Systemic Lupus Erythematous (SLE) is a long term autoimmune disorder that may affect the skin, joints, kidneys, brain and other organs. SLE is a multisystem disease characterized by the presence of multiple autoantibodies particularly antinuclear antibodies. SLE patients have a mortality risk at least double that of the general population. SLE is much more common in women than men. It may occur at any age but appears most often in people between ages 10 and 50. African American and Asians are affected more often than people from other races.

The cause of Lupus Erythematous (LE) is unknown. Studies suggest it may be a combination of genetic factors, environmental factors, stress, viral, drug induced. Symptoms of SLE include: Almost everyone with SLE have joint pain and swelling. Some develop arthritis. Frequently affected joints are the fingers, hands wrists and knees. Fatigue, fever with no cause, hair loss, oral sore, sensitivity to light, skin manifestations and swollen lymph nodes. The skin manifestations include a “butterfly rash” over the cheeks and bridge of the nose. These symptoms affect about half of the people with SLE. The rash gets worse on sunlight.

Treatment: There is no cure for SLE. The goal of treatment is to control symptoms. Mild disease may be treated with NSAID’s which treat arthritis. Corticosteroid creams to treat skin rashes and arthritis symptoms. Protective clothing, sunglasses and sunscreens should be worn in the sun.

Treatment plan for more severe lupus may include high dose of corticosteroids or medication to decrease the immune system response. Cytotoxic drugs (drugs that block cell growth) if the patient does not improve with corticosteroids. These medications have severe side effects. Many people with mild symptoms may need no treatment or only intermittent courses of anti-inflammatory medications. Those with more serious illness involving damage to internal organs require high doses of corticosteroids in combination with other medications that suppress the body’s immune response. NSAIDs are helpful in reducing inflammation and pain in muscles, joints and other tissues. Some examples of NSAIDs are aspirin, ibuprofen, naproxen and sulindac. Corticosteroids are more potent than NSAIDs in reducing inflammation and are particularly helpful when internal organs are affected. Antimalarial medications such as hydroxychloroquine (Plaquenil) are effective for SLE people with fatigue, skin involvement and joint disease. This medication prevents flare-ups of lupus.

Some medications include:

Topical Corticosteroids:Triamcinoloneacetonide 0.1%and Clobetasol propionate 0.05%

Oral Corticosteroids:Prednisone

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.

Topical Calciuneurin Inhibitors: Tacrolimus (Protopic® 0.1%) and Pimercrolimus

Anti-malarials:Antimalarials were originally used to treat malariaduring WWII. It has been proven that they are effective in treating the symptoms of Lupus by reducing muscle and joint pain, skin rashes and pericarditis. Hydroxychloroquine (Plaquenil®) and 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.

Inmunosuppresants: AzaTHIOprine (Azasan®; Imuran®), Methotrexate (Rheumatrex®; Trexall®) and 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

B-cell Suppressor: RiTUXimab (Rituxan®) and Belimumab (Benylsta®)

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.

Crest Syndrome is a connective tissue disease that involves change in the skin, blood vessels, muscle and internal organs. It is a type of autoimmune disorder, a condition that occurs when the immune system mistakenly attacks and destroys healthy body tissue. People with this condition have a buildup of a substance called collagen in the skin and other organs. This bulid up leads to the symptoms of the disease. The etiology of Crest Syndrome is unknown.

This disease usually affects people 30-50 years old. Women get scleroderma more often than men do. Wide spread scleroderma can occur with other autoimmune disease including systemic lupus erythematous and polymyositis. In such cases the disease is referred to as mixed connective disease. Thesymptoms involved in CREST syndrome are associated with generalized form of Systemic sclerosis. CREST is an acronym for the clinical features that are seen in a patient with this disease. The “C” stands for calcinosis, where calcium deposits form under the skin on the fingers or other areas of the body. The “R”, stands for Raynaud’s phenomenon, spasm of blood vessels in the fingers or toes in response to cold or stress. The “E” represents esophageal dysmotility, which can cause difficulty swallowing. The “S” is for sclerodacity, tightening of the skin causing the fingers to bend. The letter “T” is for telangiectasia, dilated vessels on the skin of the fingers, face or inside of the mouth.

Symptoms include: Finger/toes that turn blue or white in response to hot and cold temperature. Hair loss, skin hardness, skin that is abnormally dark or light, skin thickening, stiffness, and tightness of finger, hands and forearm. Small white lumps beneath the skin sometimes oozing a white substance that looks like toothpaste. Sores (ulcers) on the finger or toes; joint pain; esophageal reflux or heartburn are also symptoms related to CREST syndrome.

There is no specific treatment for scleroderma. However the doctor prescribes medicine and other treatment to control the symptoms and prevent complication. Medicines used to treat scleroderma include: Power anti-inflammatory drugs called corticosteroids. Nonsteroidal anti-inflammatory drugs (NSAIDs). Immune Suppressing medications such as methotrexate and Cytoxan. They are used for rheumatoid arthritis. It is an antineoplastic agent.

Effect on Dental Treatment: ulcerative stomatitis, gingivitis, glositis and mucositis. Its mechanism of actionis a folateantimetabolite that inhibits DNA synthesis. The contraindicationsarehypersensitivity to methotrexate or any component of the formulation; breast feeding. The drug interaction is to avoid concomitant use acitretin; BCG, cloapine, natalizumab.

The dental hygienist should obtain a thorough medical history, which include all the medications and side effects. Due to the various medications the patient is under a strict oral regimen and recalls should be maintained. A powered tooth brush should be recommended to the patient due to the lack of dexterity. Advise the patient to use WaterPick to clean interproximally since the patient may not be able to maneuver the dental floss. And recommend the patient to eat a healthy diet.

Resources:

  • You have full text access to this OnlineOpenarticleArthritisRheumatismVolume 62, Issue 12, Article first published online: 30 NOV 2010