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Systemic
Lupus Erythematosus |
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Overview |
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Definition |
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Systemic lupus erythematosus (SLE) is a combination of diseases, attributable
to a genetically determined production of pathogenic autoantibodies and immune
complexes. Immune abnormalities, typically involving antibodies to a number of
nuclear or other cellular antigens, result in inflammation and cellular injury.
Lupus is characterized by periods of both chronic disease and remission.
Prevalence is not well established and ranges from 15 to 50 cases per 100,000
people. |
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Etiology |
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There is no known etiology for lupus. However, genetics, immune
abnormalities, hormones, and environmental factors all play a role in the
etiology or aggravation of lupus. |
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Risk Factors |
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- Ultraviolet (UV) light (both UV-A and UV-B) sensitivities
- Females (90% of cases); female:male ratio changes to 3:1 outside of
childbearing years
- Genetic predisposition
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Signs and Symptoms |
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Lupus may involve one organ or many, even at onset.
- Fever
- Fatigue
- Malar ("butterfly") rash
- Skin lesions
- Hair loss
- Nausea, vomiting, abdominal pain
- Muscle pain and stiffness
- Arthritis
- Oral/nasopharyngeal ulcers
- Central nervous system (CNS)—headaches,
migraine, cognitive dysfunction, seizures, stroke, psychosis
- Depression, anxiety, confusion
- Photosensitivity
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Differential
Diagnosis |
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- Rheumatoid arthritis
- Multiple sclerosis
- Psychological disorders
- Dermatitis
- Fibromyalgia
- Chronic fatigue syndrome
- Drug-induced lupus
- Discoid lupus
- Hepatitis
- Vasculitis
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Diagnosis |
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Physical Examination |
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The patient appears pale, may have hair loss, and reports malaise. There is
typically joint swelling, mouth sores, or skin rash. The heartbeat is rapid
(with or without chest pain), and the patient usually has episodic
fever. |
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Laboratory Tests |
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- Cerebral spinal fluid—elevated protein in
50%, increased mononuclear cells in 30%
- Erythrocyte sedimentation rate—assesses
clinical activity
- Urinalysis and increased serum creatinine
levels—proteinuria, cylindruria, hematuria
- Hematology may show anemia, leukopenia, lymphocytopenia,
thrombocytopenia
- Elevated number of antinuclear antibodies (ANAs) indicate:
- Diffuse proliferative glomerulonephritis
- Antibodies (depending on disease expression) to cell surface,
glomerular, and/or tubular antigens, and endothelial and/or neuronal
cells
- Antibodies to double-strand DNA (dsDNA) and
Sm—relatively specific
- Hypoalbuminuria
- Increased prothrombin time
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Pathology/Pathophysiology |
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- Antigen-specific, polyclonal B and T lymphocyte hyperactivity and lack
of regulation
- Immune response switches from IgM to IgA antibodies
- Most common genetic marker—C4AQO, a defective
class III allele fails to encode C4A protein
- Ischemic necrosis of bone, cellular necrosis
- Perivascular mononuclear cells, lumen obliteration, enlarged
endothelial cells, thrombi
- Skin lesions—hyperkeratosis, follicular
plugging, immune complexes (IC) at dermal–epidermal
junction, mononuclear infiltrates in upper dermis, lesions show leukocytoclastic
angiitis
- Mononuclear cells infiltrate pleura and pericardium
- Renal—IC in mesangium and glomeruli basement
membrane
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Imaging |
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- Magnetic resonance imaging—most sensitive
radiographic technique to evaluate CNS changes; reveals avascular
necrosis
- Computed tomography and angiograms—identify
focal neurologic deficits
- Angiograms—shows mesenteric
vasculitis
- X ray—diagnoses lupus pneumonitis; intestinal
perforation and vasculitis
- Echocardiogram—reveals
pericarditis
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Other Diagnostic
Procedures |
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- Diagnosis is determined by the 1982 Revised American College of
Rheumatology criteria for SLE (97% sensitivity, 98% specificity). A patient must
have 4 of the 11 criteria, serially or simultaneously, to be diagnosed with
lupus. Broadly, the criteria include: malar rash, discoid rash,
photosensitivity, oral and nasopharyngeal ulcers, nonerosive arthritis,
serositis (pleuritis, pericarditis), renal disorder (proteinuria), neurological
disorder (seizures, psychosis), hematological disorder (hemolytic anemia,
leukopenia), immunologic disorder, and antinuclear antibodies.
- Renal biopsy assesses extent of disease for accurate treatment, and
lumbar puncture determines presence of infection.
- Electroencephalograms—abnormal in 70% of
patients
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Treatment Options |
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Treatment Strategy |
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There is no known cure for lupus. Symptoms are managed and complications or
flare-ups are emergently treated. The goal is to prevent organ damage and
maintain organ function. Sunscreen and sun avoidance are recommended for
photosensitivity. |
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Drug Therapies |
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- Glucocorticoids—for disabling and organ
disease; 1 to 2 mg/kg/day bid or tid, taper with improvement; prednisone 15
mg/day before noon to avoid hypothalamic–pituitary axis
suppression; intravenous treatment with acute illness; numerous serious side
effects
- Nonsteroidal anti-inflammatory drugs and
salicylates—to alleviate arthralgias, arthritis,
myalgias, fever, serositis
- Antimalarials—to alleviate arthritis,
dermatitis, pleuritis, fatigue, hair loss; e.g., hydroxychloroquine 400 mg/day
for at least two years, 6- to 12-week response time; side effects uncommon;
monitor retinal toxicity with long-term use
- Cytotoxic agents—renal disease, pulmonary
hemorrhage, vasculitis, anemia, arthritis; reduces flare-ups and need for
steroids; cyclophosphamide (1.5 to 2.5 mg/kg/day)—most
effective but serious side effects; azathioprine (50 to 200
mg/day)—least toxic
- Warfarin—for
thrombosis
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Surgical Procedures |
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Occasionally for serositis, vasculitis, deforming arthritis, avascular
necrosis |
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Complementary and Alternative
Therapies |
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While CAM is occasionally able to control SLE symptoms, treatments are
usually most helpful at minimizing symptoms and/or dosages of pharmaceuticals
and at reducing side effects of pharmaceutical interventions. Goal is to
decrease immune response cross reactivity. |
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Nutrition |
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- Eliminate all suspected allergens, including dairy, wheat (gluten),
soy, chocolate, eggs, corn, and preservatives. An ELISA IgG delayed sensitivity
food allergy panel is helpful in identifying food sensitivities.
- A modified fast of five to seven days at two-week intervals may be
helpful, especially during flare-ups. Modified fast can consist of fruits,
vegetables, and fish or vegetable protein.
- Avoid coffee, alcohol, and smoking to decrease toxic load on the
body.
- Minimize red meat and saturated fats to decrease
inflammation.
- Omega-3 fatty acids such as flaxseed and fish oils (3,000 mg/day)
decrease inflammation.
- Beta-carotene (50 mg tid) has cleared some cases of discoid lupus.
Some controversy exists about whether vitamin A is helpful or harmful in
SLE.
- Vitamin B12 (1,000 mcg IM once or twice a week) increases
healing of lesions.
- Vitamin E (800 IU/day) for both discoid lupus and SLE
- Tryptophan should be avoided in SLE. SLE patients may have difficulty
converting tryptophan, which may lead to auto-antibody production. Eating
alfalfa sprouts has also increased symptoms.
- Hydrochloric acid. SLE sufferers may have a deficiency and
supplementation can decrease symptoms.
- DHEA (start at 5 mg tid and work up to 100 mg/day) is especially
effective in reducing symptoms in mild to moderate SLE with incremental benefits
over 3 to 12 months.
- Melatonin (20 mg before bed) has been shown to be helpful in many
autoimmune diseases. Lower dose if drowsiness occurs.
- Methylsulfonylmethane (MSM) (3,000 mg bid) helps prevent joint and
connective tissue degeneration.
- Iron leads to increased inflammation and should be avoided if patient
is not anemic.
Autoimmune diseases can be viewed as a cross sensitivity to an exogenous
antigen that has similar receptor sites to an endogenous antigen. Reducing the
exposure of the immune system to exogenous antigens (usually through the gut
wall) can help lower the immune response. Agents that strengthen GI mucosa are
beneficial. Consider L-glutamine (3,000 mg tid) or quercetin (500 mg
tid). |
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Herbs |
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Herbs may be used as dried extracts (pills, capsules, or tablets), teas, or
tinctures (alcohol extraction, unless otherwise noted). Dose for teas is 1
heaping tsp. herb/cup water steeped for 10 minutes (roots need 20 minutes).
Herbs may be useful for treating secondary symptoms, such as depression and
insomnia.
Dandelion (Taraxacum officinale), yellowdock (Rumex crispus),
echinacea (Echinacea purpurea), and garlic (Allium sativum) are
historically used as alteratives (balancing herbs) and may be useful. Equal
parts in a tea, 1 cup tid. |
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Homeopathy |
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An experienced homeopath would consider an individual's constitutional type
to prescribe a more specific remedy and potency. Some of the most common acute
remedies are listed below. Acute dose is three to five pellets of 12X to 30C
every one to four hours until symptoms resolve.
- Arsenicum album for restless exhaustion that comes on quickly,
feels worse with cold
- Calcarea carbonica for overworked, overwhelmed people with poor
stamina and low back pain
- Nux vomica for irritable people with constipation and sharp,
crampy pains that feel better with heat
- Tuberculinum for repeated chest infections and joint pain with
swollen glands
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Acupuncture |
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May help balance immune response during remissions and alleviate acute
exacerbations. |
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Patient Monitoring |
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Patients need to be closely monitored during flare-ups. Evaluation of
specific complications and measurement of erythrocyte sedimentation rates are
performed to determine appropriate treatment and induce
remission. |
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Other
Considerations |
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Prevention |
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- Avoid sun exposure, high-dose oral contraceptives, penicillin, and
sulfonamides, as they can exacerbate lupus
- Exercise regularly
- Flu and pneumococcal vaccines—generally
recommended
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Complications/Sequelae |
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- Arthritis, arthralgias, myalgias, tenosynovitis
- Pericarditis, myocarditis, hypertension, coronary heart disease
(especially in long-term disease)
- Pleurisy, pneumonitis, pulmonary embolus
- Nephritis—mesangial nephritis, diffuse
proliferative nephritis, glomerulonephritis
- Anemia, leukopenia, lymphocytopenia, thrombocytopenia,
splenomegaly
- Fibromyalgia
- Vasculitis—cerebral, intestinal
- Isolated discoid lesions—10% frequency of
developing into lupus
- Subacute cutaneous lupus erythematosus
- Pancreatitis
- Hepatomegaly
- Autophospholipid syndrome—false positive
syphilis tests
- Orbital myositis, conjunctivitis, cataracts
- Raynaud's phenomenon
- Sjögren's syndrome
- Coomb's disease
- Osteoporosis from steroid use
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Prognosis |
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- Poor prognosis correlates with high serum creatinine levels, nephrotic
syndrome, hypertension, anemia, autoalbuminemia, low socioeconomic
status
- Survival rates—10 years, 90%; 20 years, 63%
to 75%
- Half of people who go into remission remain so for decades, but 90% of
patients have complications
- Symptoms decrease after menopause
- Major cause of
death—infection
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Pregnancy |
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- Still births and spontaneous abortions—10% to
30% higher than the general population
- Hypertension, renal disease,
myocarditis—pregnancy contraindicated
- Onset, flare-ups—common
- With disease control and absence of serious cardiac or renal
involvement— two-thirds have safe, full-term
pregnancies
- Newborns—cardiac arrhythmias; rarely,
neonatal lupus (1% to 5%) with skin
lesions
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References |
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Bartram T. Encyclopedia of Herbal Medicine. Dorset, England: Grace
Publishers; 1995:278.
Cecil R, ed. Cecil Textbook of Medicine. 20th ed. Philadelphia, Pa:
W.B. Saunders; 1996.
Dambro MR. Griffith's 5-Minute Clinical Consult. 1999 ed. Baltimore,
Md: Lippincott Williams & Wilkins, Inc.; 1999.
Fauci AS, Braunwald E, Isselbacher KJ, et al, eds. Harrison's Principles
of Internal Medicine. 14th ed. New York, NY: McGraw-Hill; 1998.
Koopman WJ, ed. Arthritis and Allied Conditions. 13th ed. Baltimore,
Md: Williams & Wilkins, Inc.; 1997.
Morrison R. Desktop Guide to Keynotes and Confirmatory Symptoms.
Albany, Calif: Hahnemann Clinic Publishing; 1993:39-44, 82-87, 272-276,
390-392.
Val Vollenhoven RD, Engleman, EG, McGuire JL. An open study of
dehydroepiandrosterone in systemic lupus erythematosus. Arthritis
Rheumatol. 1994;37:1305-1310.
Werbach M. Nutritional Influences on Illness. New Canaan, Conn: Keats
Publishing Inc;1987:292-296. |
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Copyright © 2000 Integrative Medicine
Communications This publication contains
information relating to general principles
of medical care that should not in any event be construed as specific
instructions for individual patients. The publisher does not accept any
responsibility for the accuracy of the information or the consequences arising
from the application, use, or misuse of any of the information contained herein,
including any injury and/or damage to any person or property as a matter of
product liability, negligence, or otherwise. No warranty, expressed or implied,
is made in regard to the contents of this material. No claims or endorsements
are made for any drugs or compounds currently marketed or in investigative use.
The reader is advised to check product information (including package inserts)
for changes and new information regarding dosage, precautions, warnings,
interactions, and contraindications before administering any drug, herb, or
supplement discussed herein. | |