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Look Up > Conditions > Systemic Lupus Erythematosus
Systemic Lupus Erythematosus
Overview
Definition
Etiology
Risk Factors
Signs and Symptoms
Differential Diagnosis
Diagnosis
Physical Examination
Laboratory Tests
Pathology/Pathophysiology
Imaging
Other Diagnostic Procedures
Treatment Options
Treatment Strategy
Drug Therapies
Surgical Procedures
Complementary and Alternative Therapies
Patient Monitoring
Other Considerations
Prevention
Complications/Sequelae
Prognosis
Pregnancy
References

Overview
Definition

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.


Etiology

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.


Risk Factors
  • 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

Signs and Symptoms

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

Differential Diagnosis
  • Rheumatoid arthritis
  • Multiple sclerosis
  • Psychological disorders
  • Dermatitis
  • Fibromyalgia
  • Chronic fatigue syndrome
  • Drug-induced lupus
  • Discoid lupus
  • Hepatitis
  • Vasculitis

Diagnosis
Physical Examination

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.


Laboratory Tests
  • 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

Pathology/Pathophysiology
  • 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

Imaging
  • 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

Other Diagnostic Procedures
  • 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

Treatment Options
Treatment Strategy

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.


Drug Therapies
  • 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

Surgical Procedures

Occasionally for serositis, vasculitis, deforming arthritis, avascular necrosis


Complementary and Alternative Therapies

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.


Nutrition
  • 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).


Herbs

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.


Homeopathy

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

Acupuncture

May help balance immune response during remissions and alleviate acute exacerbations.


Patient Monitoring

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.


Other Considerations
Prevention
  • Avoid sun exposure, high-dose oral contraceptives, penicillin, and sulfonamides, as they can exacerbate lupus
  • Exercise regularly
  • Flu and pneumococcal vaccines—generally recommended

Complications/Sequelae
  • 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

Prognosis
  • 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

Pregnancy
  • 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

References

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.


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.