Lupus: The Great Imitator

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2010

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lupus: The Great Imitator

upus erythematosus is an “autoimmune” disease in which the patient’s immune system incorrectly attacks the body’s own tissues and organs resulting in inflammation and damage. There are several types of lupus: Discoid, Subacute cutaneous, Neonatal, Drug-induced and Systemic lupus erythematosus (SLE). Discoid lupus is characterized by a persistent, raised, red rash that is commonly found on the scalp or face and can result in scarring. Subacute cutaneous lupus presents as a rash produced by exposure to the sun. Neonatal lupus is a rare condition that may develop because the mother has SLE or Sjogren’s syndrome (dry eyes and dry mouth often noted in patients with SLE or other rheumatic diseases). Some anticonvulsants, high blood pressure and other cardiac medications, antibiotics, some antifungal and anti-thyroid drugs and oral contraceptives can cause Drug-induced lupus. This is associated with many of the classic signs of SLE, but resolves after the involved medication is discontinued. SLE is a systemic (many parts) form of lupus that attacks several tissues and organs. In most cases, when people refer to lupus, they mean SLE. The remainder of this article will focus on SLE.

Cause and Frequency

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The cause of SLE is unknown, but several factors may be responsible. Genetics appears to be involved because individuals are more likely to develop SLE if they have family members with the disease or some other type of autoimmune disorder. Lupus occurs most frequently in women of child-bearing age, but can also be diagnosed in children, adolescents, and men. Some risk factors for acquiring lupus are female gender, age between 15 to 40 years, and race. African American, Hispanic, and Asian women appear to be at much greater risk than Caucasians. In the United States, there are approximately 20-60 cases of SLE per 100,000 persons and a 6-8:1 female to male ratio in those between 10-50 years. Sunlight, EpsteinBarr viral infection and exposure to a variety of chemicals may also be contributing factors in the development of SLE.

Symptoms, Signs, and Organ Involvement SLE is sometimes called the “great imitator” because its symptoms can resemble various disease states including rheumatoid arthritis, blood disorders and a host of other conditions involving the heart, lungs, muscles and immune system. A wide range of symptoms can be seen in a given patient with SLE and their severity can be highly variable. Symptoms range from mild skin rash (typically a facial butterflylike rash across the cheeks and nose) to severe disorders of brain function and kidney failure. The most common signs and symptoms of SLE are pain or swelling in various joints, unexplained fever, extreme fatigue or tiredness, chest pain associated with deep breathing, unusual hair loss, rash after exposure to the sun and pale or purple fingers or toes related to stress or cold temperatures. New or worsening symptoms may appear at any time during the course of SLE. A flare develops when symptoms increase and remission occurs when they resolve or decrease in severity. Most patients with lupus experience mild symptoms with periodic flares that are probably induced by environmental factors such as ultraviolet light, infection, pregnancy or during the period of delivery, injury and emotional stress. Estrogen is another factor that may also play a role because these episodes often occur immediately before a menstrual cycle when estrogen levels are the highest. Drugs associated with drug-induced lupus typically do not produce flares of SLE. The kidneys appear to be one of the most susceptible organs to injury as up to 75% of patients with SLE will experience kidney damage of variable degree and, in some, dialysis may be required. Other patients may develop an inflammation of the tissue covering the heart (pericarditis) or lungs (pleuritis), and nearly 80% of patients with lupus experience cognitive dysfunction (confusion, memory loss, etc.). continued


Lupus: The Great Imitator Diagnosis

Treatment

No single test is sufficiently accurate for the physician to make a diagnosis of SLE. The findings of various blood tests for specific antibodies in combination with evaluation of the patient’s signs and symptoms serve as a cornerstone for the diagnosis of this disease. It may sometimes require months to years for an accurate diagnosis to be made. The American College of Rheumatology considers the patient to have SLE if they possess four or more of the following criteria:

Rheumatologists are physicians who specialize in the treatment of diseases of muscles and joints and usually serve as the primary physician for patients with lupus. A cure for lupus does not currently exist; however, several treatment options are available to control symptoms and prevent or reduce disease progression. Some nondrug measures that may decrease lupus symptoms include adequate rest, limiting sun exposure, regular exercise, smoking cessation and eating a healthy diet. Many of the medications used to treat lupus target the immune system. Treatment is highly variable and dependent on the presence and severity of specific signs and symptoms. Medications listed in the accompanying table are commonly used to accomplish some of the five primary goals in the management

• Malar or butterfly rash – a flat or raised rash on the face • Discoid rash – circular raised patches (often on the face or ears) that become thick and scaly and result in scarring • Photosensitivity – after sun or ultraviolet light exposure • Oral ulcers • Arthritis – pain or swelling in two or more joints • Serositis – inflammation of the lungs (pleuritis) or of the sac around the heart (pericarditis) • Renal disease

class AntiInflammatory Agents

medications

Complications include stomach irritation, bleeding and ulcers; increased risk of heart disease; kidney disease

Corticosteroids

Prednisone Methylprednisolone

Decrease the body’s immune system response and allow for resolution of symptoms associated with inflammation such as swelling, warmth, tenderness and pain

Increased appetite, fluid retention, weight gain, acne, hair growth, increased blood sugar, bruising, irritability, insomnia, osteoporosis, depression

Antimalarials

Hydroxychloroquine (Plaquenil) Chloroquine (Aralen)

Allow for reduction of dosage of other medications and are beneficial for skin lesions and milder joint symptoms

Rare and mild including upset stomach, alterations in skin color, retinal damage (basis for periodic eye examination)

Immunosuppressives

Cyclophosphamide (Cytoxan) Methotrexate (Rheumatrex) Azathioprine (Imuran) Mycophenolate (Cell-Cept)

Control inflammation and decrease organ damage

All increase the risk of infection; toxicities vary, but may involve blood, liver, lungs, kidney and the skin

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Additional information on newsletter topics: Pharmaceutical Information Center 412-396-4600 pic@duq.edu Questions about screenings or programs: Christine O’Neil, Pharm.D, B.C.P.S. 412-396-6417

Newsletter Contributors John G. Lech, Pharm.D. Vincent A. Vidaurri, Pharm.D. Ryan Clement, Pharm.D. Candidate Kristen L. Dominik, Pharm.D. Candidate

role in therapy adverse effects Agents used most commonly because they reduce inflammation, pain and fever

• Antinuclear antibodies (antibodies that attack the nucleus of the body’s cells) in high concentration

A publication of: Mylan School of Pharmacy Center for Pharmacy Care Pharmaceutical Information Center (PIC)

Take an active role in understanding your disease state and be aware of the complications that may arise. Speak with your doctor on a regular basis and monitor signs and symptoms of disease progression. In addition, recognize triggers that cause flares because you could prevent them from occurring and effectively treat them if they do occur. Taking medications as prescribed is a crucial element in improving your quality of life and prognosis. The Lupus Foundation is one of the strongest patient support groups in the country and should be contacted by all patients.

Aspirin Acetaminophen NSAIDs such as ibuprofen & naproxen

• Immunologic disorders

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What You Can Do

Drugs Used in the Treatment of SLE

• Seizures or psychosis with no known cause • Hematologic disorder – hemolytic anemia (decreased red blood cells) or leucopenia (decreased white blood cells)

of lupus: reduce inflammation, suppress an overactive immune system, control symptoms such as joint pain and fatigue, prevent flares (treat them if they occur) and minimize organ damage.

For more information on Lupus, please visit the following websites: • The Lupus Foundation of America (http://www.lupus.org) • The National Institute of Arthritis and Musculoskeletal and Skin Diseases (www.niams.nih.gov) • National Library of Medicine (http://www.nlm.nih.gov/medlineplus/lupus.html)

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