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Systemic Lupus Erythematosu s

Furqan Khan RN BSN CWCN NURS 504 Advanced Pharmacology Liberty University

Systemic Lupus Erythematosus Introduction:


Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease The immune system attacks the bodys cells and tissue, resulting in inflammation and tissue damage. SLE can affect any part of the body, but most often harms the heart, joints, skin , lungs , blood vessels , liver, kidneys, and nervous system. Over 40 different genes predispose to SLE Characterized by remissions and exacerbations

Edmunds, M. W., & Mayhew, M. S. (2009). Pharmacology for the primary care provider. St. Louis, MO: Saunders.

Systemic Lupus Erythematosus


Prevalence:
Almost 90% of all cases occur in women Overall, SLE affects women eight times more often than it does men At age 30 years, the ratio of women to men is 10:1 The ratio at age 65 years, the ratio appears to be about 3:1 The prevalence rate among women between ages 15 and 64 years is 1 in 700 women Symptoms usually appear between ages 15 and 25 years The prevalence in the general population is about 1 in 1000

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information & Education Resource .

Retrieved from http://online.statref.com

The etiology of SLE is currently unknown, but there are environmental and genetic factors involved Environmental Factors: Ultraviolet light ( UVB ), Alfalfa sprouts, chemicals ( hydrazines) ? Drugs (Resprim = Trimethoprim + sulphamethoxazole), Infections (parvovirus, CMV, HCV ), Smoking B cell activation results in increased autoantibody (mainly IgG) production to a variety (up to 2000) of antigens (nuclear, cytoplasmic and plasma membrane), e.g. ANA, anti-dsDNA. Development of and failure to remove immune complexes from the circulation leads to deposition of complexes in the tissue, causing vasculitis and disease (e.g. glomerulonephritis). Immune complexes also from in situ, e.g. kidney glomerular basement membrane. There is impaired T cell regulation of the immune response.
Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information & Education Resource . Retrieved from http://online.statref.com

Systemic Lupus Etiology: Erythematosus

Systemic Lupus Erythematosus Signs & Symptoms: PERCENTAGE (%)


Achy joints / arthralgia 95% Fever of more than 100 degrees F / 38 degrees C - 90% Arthritis / swollen joints 90% Prolonged or extreme fatigue 81% Skin Rashes 74% Anemia 71% Kidney Involvement - 50% Pain in the chest on deep breathing / pleurisy 45% Butterfly-shaped rash across the cheeks and nose - 42% Sun or light sensitivity / photosensitivity - 30% Hair loss / Alopecia - 27% Abnormal blood clotting problems 20% Fingers turning white and/or blue in the cold 17% Mouth or nose ulcers 12%

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information & Education Resource . Retrieved from http://online.statref.com

Systemic Lupus Erythematosus


BUTTERFLY RASH

PHOTOSENSITIV E ERYTHEMA

ERYTHMATOUS BULLOUS LESIONS

SUBACUTE CUTANEOUS RASH

DISCOID LUPUS

Images : dermatlas.org

Systemic Lupus Erythematosus Pathophysiology:


The plasma cells are producing antibodies that are specific for self proteins, namely ds-DNA Overactive B-cells. Estrogen is a stimulator of B-cell activity Suppressed regulatory function in T-cells . Lack of T-cells IL-10, also a B-cell stimulator is in high concentration in lupus patient serum. High concentration linked to cell damage caused by inflammation Increased levels of Ca2+ . Leads to spontaneous apoptosis
Moser, R. (2001). Primary Care for Physician Assistants: Clinical Practice Guidelines. New York . McGraw- Hill Medical Publishing.

Systemic Lupus Erythematosus Pathophysiology:


RBCs lack CR1 receptor. Decreasing the affective removal of complexes IgG is the most pathogenic because it forms intermediate sized complexes that can get to the small places and block them. DNA is the main antigen for which antibodies are formed. Extracellular DNA has an affinity for basement membrane where it is bound by auto-antibodies. Classical thickening of the basement membrane
Moser, R. (2001). Primary Care for Physician Assistants: Clinical Practice Guidelines. New York . McGraw- Hill Medical Publishing.

Er ythematosus Test

Systemic Lupus Erythematosus


Notes

Laborator y and Other Studies for Systemic Lupus

Complete blood count

The most common anemia in SLE is from chronic disease. Hemolytic anemia will usually have a positive direct Coombs' test, for either IgG or complement, or both. Both Leukopenia and Lymphopenia are found in SLE. Thrombocytopenia can be due to SLE or to Antiphospholipid antibodies Although the ESR is commonly elevated, it is not specific for SLE An elevated Creatinine may be a clue to renal lupus. Mild elevations in liver function tests occur in 30% SLE Myositis presents with proximal muscle weakness. Creatine phosphokinase is usually elevated Glomerulonephritis usually presents with Proteinuria, with or without Hematuria. Erythrocytes or granular casts can be seen Anti-DNA or anti-Sm are specific for SLE. Low C3 and low C4 are common in SLE, but not specific. A negative ANA argues against SLE. Anti-RNP, anti-Ro/SSA, anti-La/SSB, and Antiphospholipid antibodies may be found, but they are not specific for SLE Indicated in patients with laboratory findings suggestive of lupus nephritis

Erythrocyte sedimentation rate Comprehensive metabolic panel Creatine Phosphokinase Urinalysis

Serologies

Renal biopsy
http://online.statref.com

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information & Education Resource . Retrieved from

Systemic Lupus Erythematosus PROGNOSIS


Usually chronic, relapsing, and unpredictable. Remissions may last for years. If initial acute phase is controlled, even if very severe ( with cerebral thrombosis or severe nephritis), the long-term prognosis is usually good. The 10-yr survival in most developed countries is > 95%. Improved prognosis is in part due to earlier diagnosis and more effective therapies. More severe disease requires more toxic therapies, which increase risk of mortality.
Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information & Education Resource . Retrieved from http://online.statref.com

Systemic Lupus Erythematosus TREATMENT GOALS


Use the drug with the: - Least side effects - Lowest dose to control disease - Long term damage prevention Mild disease: Avoid Steroids Severe disease: Aggressive treatment
Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information Education &Resource . Retrieved from http://online.statref.com

Systemic Lupus Erythematosus


PHARMACOLOGY: NSAIDs Acetaminophen CORTICOSTEROIDS Prednisone ANTI-MALARIAL - Hydroxychloroquine

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information & Education Resource . Retrieved from http://online.statref.com

Systemic Lupus Erythematosus PHARMACOLOGY: NSAIDS


NON STEROIDAL ANTI-INFLAMMATORY DRUGS

NON STEROIDAL ANTI-INFLAMMATORY DRUGS Use to control mild to moderate pain, fever, and various inflammatory conditions, such as rheumatoid arthritis and osteoarthritis. NSAIDS have analgesic, antipyretic, and antiinflammatory properties. Analgesic and anti-inflammatory effects are due to inhibition of prostaglandin synthesis. Antipyretic action is due to vasodilation and inhibition of prostaglandin synthesis in the CNS

Turkoski, B. B., Lance, B. R., & Tomsik, E. A. (2009). Drug information handbook. Hudson, OH: Lexi-Comp.

Systemic Lupus Erythematosus PHARMACOLOGY: NSAIDS

NON STEROIDAL ANTI-INFLAMMATORY DRUGS

NON STEROIDAL ANTI-INFLAMMATORY DRUGS Use cautiously in patients with a history of bleeding disorders, GI bleeding, and severe hepatic, renal, or cardiovascular disease. Safe use in pregnancy is not established and, in general, should be avoided during the second half of pregnancy. NSAIDs prolong bleeding time and potentiate the effect of warfarin, thrombolytic agents, some cephalosporins, and anti-platelet agents. NSAIDs may also decrease response to diuretics or antihypertensive therapy.

Turkoski, B. B., Lance, B. R., & Tomsik, E. A. (2009). Drug information handbook. Hudson, OH: Lexi-Comp.

Systemic Lupus Erythematosus PHARMACOLOGY: CORTICOSTEROIDS


Used for anti-inflammatory or immunosuppressant effects Dosage of prednisone depends on the condition being treated and the response of the patient. After a satisfactory response is obtained, dosage should be decreased in small decrements to the lowest level that maintains an adequate clinical response. The activity of the drug is thought to result at least in part from binding with a steroid receptor.

Turkoski, B. B., Lance, B. R., & Tomsik, E. A. (2009). Drug information handbook. Hudson, OH: Lexi-Comp.

Systemic Lupus Erythematosus PHARMACOLOGY: CORTICOSTEROIDS


Corticosteroids decrease inflammation by stabilizing leukocyte lysosomal membranes, preventing release of destructive acid hydrolases from leukocytes; inhibiting macrophage accumulation in inflamed areas; reducing leukocyte adhesion to capillary endothelium; reducing capillary wall permeability and edema formation; decreasing complement components; antagonizing histamine activity and release of kinin from substrates; reducing fibroblast proliferation, collagen deposition, and subsequent scar tissue formation; and possibly by other mechanisms as yet unknown.

Turkoski, B. B., Lance, B. R., & Tomsik, E. A. (2009). Drug information handbook. Hudson, OH: Lexi-Comp.

Systemic Lupus Erythematosus PHARMACOLOGY: CORTICOSTEROIDS


Corticosteroids usually are metabolized in the liver and excreted by the kidneys. Some topical corticosteroids and their metabolites are excreted in bile. Muscle wasting, muscle weakness, delayed wound healing, and atrophy of the protein matrix of the bone resulting in osteoporosis, vertebral compression fractures, pathologic fractures of long bones are manifestations of protein catabolism that may occur during prolonged therapy with glucocorticoids .

Turkoski, B. B., Lance, B. R., & Tomsik, E. A. (2009). Drug information handbook. Hudson, OH: Lexi-Comp.

Corticosteroids, especially in large doses, increase susceptibility to and mask symptoms of infection Infections with any pathogen, including viral, bacterial, fungal, protozoan, or helminthic infections in any organ system, may be associated with corticosteroids alone or in combination with other immunosuppressive agents Steroid withdrawal syndrome consisting of lethargy, fever, myalgia can develop following abrupt discontinuance.

Systemic Lupus Erythematosus PHARMACOLOGY: CORTICOSTEROIDS

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information & Education Resource . Retrieved from http://online.statref.com

Systemic Lupus Erythematosus PHARMACOLOGY: CORTICOSTEROIDS


Acute adrenal insufficiency (even death) may occur if the drugs are withdrawn abruptly. Prolonged therapy, may produce various endocrine disorders including hypercorticism and amenorrhea. Prior to initiation of long-term steroid therapy, perform baseline ECGs, blood pressures, chest and spinal radiographs, glucose tolerance tests, and evaluations of HPA-axis function in all patients.

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus :Physicians Information & Education Resource . Retrieved from http://online.statref.com

Systemic Lupus Erythematosus PHARMACOLOGY: ANTI-MALARIALS


ANTI-MALARIALS DRUGS

Hydroxychloroquine is used as an adjunct to corticosteroid therapy in the treatment of discoid lupus erythematosus & systemic lupus erythematosus. Hydroxychloroquine therapy may lead to the regression of skin lesions of discoid or systemic lupus erythematosus and may also have a beneficial effect in patients with systemic lupus erythematosus in whom arthritis is a prominent feature

Moser, R. (2001). Primary Care for Physician Assistants: Clinical Practice Guidelines. New York . McGraw- Hill Medical Publishing.

Systemic Lupus Erythematosus PHARMACOLOGY: ANTI-MALARIALS


ANTI-MALARIALS DRUGS

The usual initial adult dosage of hydroxychloroquine for the treatment of lupus erythematosus is 400 mg once or twice daily for several weeks or months depending on the response of the patient. For prolonged maintenance therapy, 200-400 mg of daily may be adequate. The exact mechanism of anti-malarial action of the drug in the treatment of rheumatoid arthritis and lupus erythematosus have not been determined.

Moser, R. (2001). Primary Care for Physician Assistants: Clinical Practice Guidelines. New York . McGraw- Hill Medical Publishing.

Systemic Lupus Erythematosus PHARMACOLOGY: ANTI-MALARIALS


ANTI-MALARIALS DRUGS

Ophthalmologic examinations should be performed prior to initiation of hydroxychloroquine therapy and periodically (every 3 months) during therapy whenever long-term use of the drug is contemplated. Hydroxychloroquine should be discontinued immediately, if there is any indication of abnormalities in visual acuity or visual field May exacerbate psoriasis and precipitate a severe attack in patients with the disease. Use in psoriasis patients only if potential benefits outweigh risks.

Moser, R. (2001). Primary Care for Physician Assistants: Clinical Practice Guidelines. New York . McGraw- Hill Medical Publishing.

Systemic Lupus Erythematosus PHARMACOLOGY: ANTI-MALARIALS


ANTI-MALARIALS DRUGS

May concentrate in the liver; use with caution in patients with hepatic disease or alcoholism and in patients receiving other hepatotoxic drugs. Hydroxychloroquine and its metabolites are slowly excreted by the kidneys. Hydroxychloroquine may exacerbate porphyria in patients with the condition, drug should not be used in patients with porphyria unless potential benefits outweigh risks.

Moser, R. (2001). Primary Care for Physician Assistants: Clinical Practice Guidelines. New York . McGraw- Hill Medical Publishing.

Systemic Lupus Erythematosus NURSING CARE / PATIENT EDUCATION


Take medication as directed per physician order Do not stop taking medication without discussing it with primary care physician Bed rest is indicated for patients with active SLE. Because emotional and physical stress can have a negative impact on the immune system, patients should be encouraged to avoid highpressure situations Educate patient about SLE and when to call the physician

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information Education Resource . Retrieved from http://online.statref.com

Systemic Lupus Erythematosus NURSING CARE / PATIENT EDUCATION


Because all SLE-related skin lesions are either precipitated or exacerbated by sun exposure, patients should be cautioned against exposure without sun block. Patients must be taught that sunlight exposure can also cause flare-ups in other organ systems, not only the skin. When exposure to sunlight cannot be avoided, patients should be instructed to use sunscreens that contain paraaminobenzoic acid (PABA) and also have a high sun protection factor (SPF) rating of at least 30.

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information Education Resource . Retrieved from http://online.statref.com

Systemic Lupus Erythematosus NURSING CARE / PATIENT EDUCATION


Patients with SLE should be encouraged to remain as physically active as possible. Patients should be taught that active exercise may strengthen the immune system, but that too much exercise will produce unhealthy stress. Patients should also be taught relaxation techniques to use during periods of stress. It is also important for patients to understand the need for early intervention when infections occur. When starting treatment with prednisone, inform patients about weight control, low-fat diet, and exercise.

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information Education Resource . Retrieved from http://online.statref.com

Systemic Lupus Erythematosus NURSING CARE / PATIENT EDUCATION


Educate patient about the importance of developing a social support system that will provide feedback about lupus self-management behaviors, problem solving, and alternate solution planning. Patient support organization : www.lupus.org Instruct patient to talk to primary care physician before taking any herbal or OTC medication since they may counteract the effect of other thyroid agents medications Keep all physician appointments and follow up care as scheduled

Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information Education Resource . Retrieved from http://online.statref.com

References
Edmunds, M. W., & Mayhew, M. S. (2009). Pharmacology for the primary care provider. St. Louis, MO: Saunders. Moser, R. (2001). Primary Care for Physician Assistants: Clinical Practice Guidelines. Newyork . McGraw- Hill Medical Publishing. Petri, M., Lazaro, D. (2009). Systemic Lupus Erythematosus: Physicians Information & Education Resource . Retrieved from http://online.statref.com Turkoski, B. B., Lance, B. R., & Tomsik, E. A. (2009). Drug information handbook. Hudson, OH: Lexi-Comp.

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