Lupus, SLE (Systemic
Lupus Erythematosus)

Lupus, SLE (Systemic Lupus Erythematosus): Overview

Systemic Lupus Erythematosus (SLE) is a chronic, potentially fatal autoimmune disease characterized by exacerbations and remissions with many clinical manifestations, and may mimic infectious mononucleosis, lymphoma or other systemic disease.  SLE is a complex disorder resulting from the production of antibodies that attack the DNA and proteins within healthy cells and the generation of circulating immune complexes.  The complications from this involve multiple organs and are potentially life-threatening.  The hallmark of the disease is recurrent, widespread, and diverse vascular lesions.

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There is clinical involvement of the joints, skin, kidney, brain, and membranes of the lung, heart and gastrointestinal tract.

Incidence; Causes and Development

Women and non-Caucasians are disproportionately affected and SLE is most common in women of child-bearing age although it has been reported in all ages.  The incidence is about 1 in 200 people in America.

Among children, SLE occurs three times more commonly in females than in males.  In the 60% of SLE patients who experience onset between puberty and the fourth decade of life, the female to male ratio is 9:1.  The disorder is three times more common in African American blacks than American Caucasians.  SLE is also more common in Asians.

The cause of SLE remains unknown.  A genetic predisposition, sex hormones, and environmental trigger(s) are strongly implicated in this disordered immune response.  One of many suspected factors is a genetic mutation that disrupts the body's waste disposal mechanism in cells.  The health status of a patient with SLE is related not only to disease activity, but also to the damage that results from recurrent episodes of disease flare-ups.

Signs and Symptoms

The symptoms are often vague, can be mild or severe and are often unrelated to lab tests.  A patient can have many lupus symptoms in a lifetime.

Diagnosis and Tests

A tentative diagnosis can be made through examining a patient's medical history and performing a physical exam and screening tests (positive ANA).  Once SLE is suspected, additional tests are valuable to confirm or rule it out.  These include anti-double stranded DNA, anti-RNP, anti-Sm, anti-Ro, anti-La, C3, and C4.  Some 30 to 70% of patients with SLE will be anti-DNA positive and 30% of patients with SLE will be anti-Sm positive.  The presence of anti-double stranded DNA antibodies and low complement levels strongly suggests the diagnosis of lupus and identifies the patient at increase risk of kidney damage.

Physicians have to gather information from a variety of sources; past medical history, lab tests and current symptoms.  They use a list of 11 criteria to help diagnose SLE.  A person needs to satisfy at least 4 out of the 11 criteria before the diagnosis can be pin-pointed.  Some criteria, such as a biopsy diagnosis of kidney lupus, can carry more weight.

Of the 11 criteria, 7 relate to symptoms, and 4 have to do with lab tests.  The ANA test is used as a screening test for systemic lupus.  We know that 95 % of people with SLE have a positive ANA.  Therefore, if a person has many symptoms of systemic lupus and their ANA test is negative, that's generally regarded as pretty good evidence against lupus being the explanation for the symptoms they are having.

If on the other hand, the ANA comes back positive, that IS NOT proof of lupus.  The positive ANA is only an indicator, it is not diagnostic: many women have positive ANA and not Lupus).

To assist the physician in the diagnosis of lupus, the American College of Rheumatology (ACR) in 1982 issued a list of 11 symptoms or signs that help distinguish lupus from other diseases.  This has recently been revised.  A person should have four or more of these symptoms to suspect lupus.  The symptoms do not all have to occur at the same time.

THE ELEVEN CRITERIA USED FOR THE DIAGNOSIS OF LUPUS

  1. Malar Rash – Rash on cheeks
  2. Discoid Rash – Red, scaly patches on skin which cause scarring
  3. Photosensitivity – Reaction to UV/sunlight, resulting in the development of or increase in skin rash
  4. Oral Ulcers – Ulcers in the nose or mouth, usually painless
  5. Arthritis – Nonerosive arthritis involving two or more peripheral joints (arthritis in which the bones around the joints do not become destroyed)
  6. Serositis – Pleuritis or pericarditis (inflammation of the lining of the lung or heart)
  7. Renal Disorder – Excessive protein in the urine (greater than 0.5 gm/day or 3+ on test sticks) and/or cellular casts (abnormal elements the urine, derived from red and/or white cells and/or kidney tubule cells)
  8. Neurologic DisorderSeizures (convulsions) and/or psychosis in the absence of drugs or metabolic disturbances which are known to cause such effects
  9. Hematologic disorderHemolytic anemia (low red blood cell count) or leukopenia (white blood cell count <4,000/uL), lymphopenia (<1,500/uL) or thrombocytopenia (<100,000/uL) in the absence of offending drug.
  10. Antinuclear Antibody – Positive test for antinuclear antibodies (ANA) in the absence of drugs known to induce it.
  11. Immunologic Disorder – Positive anti-double stranded anti-DNA test, positive anti-Sm test, positive antiphospholipid antibody such as anticardiolipin, or false positive syphilis test (VDRL).

[Adapted from: Tan, E.M., et.  al.  The 1982 Revised Criteria for the Classification of SLE.  Arth Rheum 25: pp.1271-77]

Treatment and Prevention

The treatment of infections in lupus patients is basically the same as for other patients.  To prevent possible infections, patients at high risk of infection often benefit from taking antibiotics before dental treatment or surgical procedures.  In general, individuals with lupus should avoid exposure to people with colds or other infections.

Animal studies indicate MSM may be useful.

Prognosis

The idea that lupus is generally a fatal disease is one of the gravest misconceptions about this illness.  In fact, the prognosis of lupus is much better today than ever before.  It is true that medical science has not yet developed a method for curing lupus and some people do die from the disease.  However, with current methods of therapy, deaths from lupus are uncommon, and 80-90% of people with lupus live more than 10 years after diagnosis.

Signs, symptoms & indicators of Lupus, SLE (Systemic Lupus Erythematosus):

Lab Values - Chemistries

Counter-indicators
Normal ANA levels

Any value less than 40 is considered a normal ANA level and called a negative test result.  Normal levels of ANA virtually rule out active SLE.  [Med Clin North Am 81(1): pp.113-28, Jan.  1997]

Symptoms - Environment

Regular sun rash on cheeks

Approximately 80% of patients with SLE have dermatological manifestations during the course of their illness.  The acute skin eruption manifests itself as a photosensitive rash which often has a butterfly appearance and involves the bridge of the nose and cheeks.  A feature of this rash is a sparing of the crease seen on the sides of the mouth when smiling.  Photosensitivity is less common in patients of color but occurs in 50% of all patients with SLE.

Symptoms - General

Fatigue for over 3/major fatigue for 3-12/major fatigue for over 12 months

Prolonged or extreme fatigue is reported by 81% of lupus patients.

(Severe) fatigue after slight exertion

90% of patients with SLE experience fatigue.

Symptoms - Head - Eyes/Ocular

(High) sensitivity to bright light

Sun or light sensitivity (photosensitivity) is experienced by 30% of sufferers.

Symptoms - Head - Mouth/Oral

Occasional/recurring/frequent mouth ulcers

Mouth or nose ulcers have been reported by between 12% and 30% of lupus patients, depending on the study.  They most often occur in the mouth on the hard or soft palate but may also be found on the nasal septum.

Symptoms - Immune System

Regular/frequent infections

Lupus patients have abnormalities in their immune systems that predispose them to develop infections.

Symptoms - Metabolic

Occasional/frequent unexplained fevers

A less common but more serious constitutional feature of SLE is persistent fever.

Unexplained high fevers or unexplained fevers that hit hard

Fever of more than 100F (38C) is reported by 90% of lupus patients.

Symptoms - Muscular

(Very) tender muscles

Muscle pains are a common symptom of SLE.  Less common is actual muscle inflammation which occurs occasionally during the course of SLE.

Symptoms - Nails

(Minor/major) inflamed cuticles

Inflammation of the nail fold (red, puffy skin around the nail) may indicate lupus or another connective tissue disorder.

Symptoms - Respiratory

Chest pain when breathing

Pain in the chest on deep breathing (pleurisy) is experienced by some 45% of lupus patients.

Occasional/regular/frequent sore throats

Sore throat or pain on taking a deep breath may occur with a flare of lupus.

Symptoms - Skeletal

Variable duration morning stiffness or morning stiffness for 45-120 minutes or morning stiffness lasting hours

Lupus patients often complain of prolonged morning stiffness or pain which may last 45 minutes or longer.

(Minor/major) joint pain/swelling/stiffness

Most patients with SLE have musculoskeletal symptoms.  The typical clinical manifestations are arthralgia, reported by 95% of patients, and arthritis (swollen joints) by 90%.  The joints most commonly involved are the index finger, wrist and knees.  Lupus is rarely accompanied by actual joint erosion.

Counter-indicators
Morning stiffness that eases rapidly

Lupus patients often complain of prolonged morning stiffness or pain which may last 45 minutes or longer.

Symptoms - Skin - Conditions

Occasional/regular/frequent rashes

Skin rashes are reported by 74% of lupus sufferers.

Conditions that suggest Lupus, SLE (Systemic Lupus Erythematosus):

Autoimmune

Chronic Thyroiditis

Approximately 10% of lupus patients have thyroid antibodies, and autoimmune thyroiditis occasionally coexists with SLE.

Circulation

Hemolytic Anemia

Anemia as a result of chronic inflammation is a characteristic but not especially common feature of active SLE.

Vasculitis

In the vasculitis caused by lupus, the antigens causing the immune complexes are often not known.  In some cases, the complexes contain DNA and anti-DNA antigens, or Ro (also called SS-A) and anti-Ro antigens.  Another antibody, ANCA (anti-neutrophil cytoplasm antibody), can cause vasculitis in some individuals.

Raynaud's Phenomenon

Raynaud's phenomenon has been observed in 17-30% of patients with SLE, depending on the study.

General

Infections

Yeast / Candida Infection

Lupus patients are at an unusually high risk for contracting candida (yeast) infections.

Urinary Tract Infection (UTI)

Infections of the urinary tract are common in lupus patients.

Shingles (Herpes Zoster)

Lupus patients are at an unusually high risk for contracting herpes zoster.

Lab Values

A Low Platelet Count

Lupus is suggested if thrombocytopenia (a low platelet count of under 100,000 platelets per cubic millimeter) is detected in the absence of drugs that are known to induce it.

Low White Blood Cell Count

Active lupus and an infection may share many symptoms.  Further, infection can induce a lupus flare or be difficult to distinguish from a lupus flare.  A low white blood cell count is suggestive of active lupus (although certain viruses can also give a low white count) while a high count suggests infection.

Metabolic

Migraine/Tension Headaches

Vascular or migraine headaches occur in 10% of lupus patients.

Nervous System

Seizure Disorder

Seizures have been found to complicate the course in between 15-25% of patients with lupus, depending on the study quoted.

Nutrients

EFA (Essential Fatty Acid) Type 3 Requirement

There is a possible defect in the metabolism of essential fatty acids (EFAs) in systemic lupus erythematosus (SLE).  In order to verify this possibility, doctors in one study measured the plasma levels of various EFAs and their metabolites in SLE.  These results showed that amongst SLE patients the concentrations of Omega-6 and Omega-3 oils or metabolites were low.  Even small doses of fish oils (which contain EPA and DHA) have been shown to help.

Organ Health

Kidney Disease

Diverse kidney problems can arise from the deposition of circulating immune complexes in the kidneys.  Lupus, being an auto-immune disease, causes the immune system to attack the body's own tissues.  The commonly affected organs/tissues are skin, joints, nervous system and kidneys.

Reproductive

Susceptibility To Miscarriages

The miscarriage rate in SLE patients is much higher than that of the general population.  Although most women who suffer recurrent miscarriages do not have clinical signs of SLE, many exhibit autoimmune phenomena which is similar to that seen in SLE patients.

Respiratory

Acute Bronchitis

Infections of the respiratory tract are common in lupus patients.

Skin-Hair-Nails

Female Hair Loss

Alopecia occurs in 50% of patients.  Typically manifested as reversible hair thinning during periods of disease activity, it is demonstrated by the ease with which hair can be plucked from the scalp and the development of "lupus hairs" (i.e. short strands at the scalp line).  Following an acute attack of SLE, usually with fever, patients may experience much generalized hair loss.  This results from a period of arrested hair growth during the acute episode.

Male Hair Loss

Alopecia occurs in 50% of patients.  Typically manifested as reversible hair thinning during periods of disease activity, it is demonstrated by the ease with which hair can be plucked from the scalp and the development of "lupus hairs" (i.e. short strands at the scalp line).  Following an acute attack of SLE, usually with fever, patients may experience much generalized hair loss.  This results from a period of arrested hair growth during the acute episode.

Symptoms - Immune System

Uro-Genital

Vaginitis/Vaginal Infection

Lupus patients are at an unusually high risk for contracting candida (yeast) infections.

Interstitial Cystitis

Interstitial cystitis patients have been found to be 30 times more likely to have systemic lupus erythematosus.

Risk factors for Lupus, SLE (Systemic Lupus Erythematosus):

Autoimmune

Gluten Sensitivity / Celiac Disease

Some patients diagnosed SLE may in fact be suffering the results of gluten intolerance.  In these cases, removing gluten from the diet may completely cure the patient.  [Annals of the Rheumatic Diseases (2004; 63: pp1501-3)]

Circulation

Atherosclerosis

Numerous studies have confirmed premature, accelerated atherosclerosis in SLE patients.  Although the exact cause is not known at this point, atherosclerotic heart disease is a common cause of morbidity and death amongst lupus patients.

Hormones

Low Adrenal Function / Adrenal Insufficiency

Lupus is one of the auto-immune diseases, caused by a hyperactive ("hypervigilant") immune system that attacks a person's own protein as if it were foreign matter.  One reason for this is poor adrenal function.  Adrenal steroids modulate (slow down) the immune system: when there is not enough of these steroids the immune system goes berserk.

Low DHEA Level

Low blood levels of the hormone DHEA have been associated with more severe symptoms in people with SLE.  Preliminary trials have suggested that 50 to 200mg per day DHEA improved symptoms in people with SLE.  One double-blind trial of women with mild to moderate SLE found that 200mg of DHEA per day improved symptoms and allowed a greater decrease in prednisone use, but a similar trial in women with severe SLE found only insignificant benefits.

Symptoms - Immune System

History of non-vaginal candidiasis

Lupus patients are at an unusually high risk for contracting candida (yeast) infections.

History of shingles

Lupus patients are at an unusually high risk for contracting herpes zoster.

History of candidiasis

Lupus patients are at an unusually high risk for contracting candida (yeast) infections.

Symptoms - Mind - General

History of seizures

Seizures have been found to complicate the course in between 15-25% of patients with lupus, depending on the study quoted.

Symptoms - Muscular

History of tender muscles

Muscle pains are a common symptom of SLE.  Less common is actual muscle inflammation which occurs occasionally during the course of SLE.

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Lupus, SLE (Systemic Lupus Erythematosus) suggests the following may be present:

Autoimmune

Hormones

Low Adrenal Function / Adrenal Insufficiency

Lupus is one of the auto-immune diseases, caused by a hyperactive ("hypervigilant") immune system that attacks a person's own protein as if it were foreign matter.  One reason for this is poor adrenal function.  Adrenal steroids modulate (slow down) the immune system: when there is not enough of these steroids the immune system goes berserk.

Low DHEA Level

Low blood levels of the hormone DHEA have been associated with more severe symptoms in people with SLE.  Preliminary trials have suggested that 50 to 200mg per day DHEA improved symptoms in people with SLE.  One double-blind trial of women with mild to moderate SLE found that 200mg of DHEA per day improved symptoms and allowed a greater decrease in prednisone use, but a similar trial in women with severe SLE found only insignificant benefits.

Lupus, SLE (Systemic Lupus Erythematosus) can lead to:

Circulation

Hemolytic Anemia

Anemia as a result of chronic inflammation is a characteristic but not especially common feature of active SLE.

General

Lab Values

Low White Blood Cell Count

Active lupus and an infection may share many symptoms.  Further, infection can induce a lupus flare or be difficult to distinguish from a lupus flare.  A low white blood cell count is suggestive of active lupus (although certain viruses can also give a low white count) while a high count suggests infection.

Metabolic

Migraine/Tension Headaches

Vascular or migraine headaches occur in 10% of lupus patients.

Organ Health

Kidney Disease

Diverse kidney problems can arise from the deposition of circulating immune complexes in the kidneys.  Lupus, being an auto-immune disease, causes the immune system to attack the body's own tissues.  The commonly affected organs/tissues are skin, joints, nervous system and kidneys.

Lupus, SLE (Systemic Lupus Erythematosus) could instead be:

Infections

Lyme Disease

Lyme arthritis is often mistaken clinically for systemic lupus erythematosus.

Organ Health

Retinitis Pigmentosa

Systemic Lupus Erythematosus (SLE) is an autoimmune disease sometimes misdiagnosed as retinitis pigmentosa.  [Am J Ophthalmol, 1996 Dec, 122:6, pp.903-5 Abstract]

Recommendations for Lupus, SLE (Systemic Lupus Erythematosus):

Animal-based

Thymic Factors

Through his clinical experiences with thymic supplementation, Dr. Burgstiner said he observed 12 cases of systemic lupus go into remission.  Some of the patients were using as many as 22 different drugs and are now diagnosed as asymptomatic.

Botanical, Chinese

Lei Gong Teng

Amongst patients with lupus nephritis who were unresponsive to prednisone and other immunosuppressive drugs, combined administration of prednisone and TP (polyglycoside extract of Tripterygium wilfordii Hook F) resulted in reduction or even complete disappearance of protein in the urine in 40-50% of cases.  Many side-effects, however, have been reported.  [Chin Med J (Taipei) 1996; 57: S35]

Diet

Plant-Based Nutrition

Animal-based proteins (beef and milk) seem to be the prime offenders in aggravating the symptoms of Lupus.  However, certain plant-based proteins appear also to be.  These include soy beans, corn, spinach and carrots.  [Scandinavian Journal of Gastroenterology 1982;17: pp.417-24]

Alfalfa sprouts and legumes, to a lesser extent, should also be avoided as the constituent L-canavanine causes SLE-like diseases in primates.  [Acta Medica Scandinavica 1984;216: pp. 67-274] Peas and lima beans are alright to eat in this regard.

Lupus flare-ups have also been reported after the ingestion of large amounts of foods containing psoralens (celery, celery salt, parsnips and figs).

Gluten-free Diet

A one-month trial period of avoiding dairy products and foods containing gluten/gliadin should indicate whether there is going to be any change in symptoms or lab values in individual patients.  If there are improvements then these foods will need to be avoided on a permanent basis.

Digestion

Bromelain

If there is kidney involvement, bromelain can be added as a cleansing agent.  Flax oil or fish oil along with bromelain between meals is a good natural anti-inflammatory combination.

Hydrochloric Acid (Trial)

Stomach acid levels are generally lower in patients with autoimmune diseases.  Inadequate digestion can add to the immune system malfunction.

Drug

Environmental

Chemical Avoidance

Hair dyes contain high levels of hydrazines and other similar chemicals that are absorbed through the scalp, thus increasing the risk of contracting Lupus.  [Am J Med 1983;75: pp.365-70] Hydrazines are also present in mushrooms, some food dyes, tobacco smoke and some cooked foods, especially meats.

Extract

DIM / I3C

There is an acceleration of the testosterone-to-estradiol conversion by an increase in aromatase activity in healthy SLE patients when compared to controls.  According to [Lupus 1992;1(3): pp.191-5], "among SLE patients the aromatase activity varied inversely with the disease activity.  Patients with SLE had decreased androgen and increased estrogen levels.  Aromatase activity in SLE patients had significant direct correlation with estrogen levels.  These data suggest that abnormal regulation of aromatase activity may partially explain the abnormalities of estrogen synthesis in SLE." These patients are relatively testosterone deficient.  Aromatase blockers such as DIM and Chrysin should be considered in such cases.

Lab Tests/Rule-Outs

Test for DHEA

DHEA is almost always low in patients with autoimmune conditions such as SLE.

Mineral

MSM (Methyl Sulfonyl Methane)

"MSM has been shown to be clinically helpful in lupus and may be beneficial in other autoimmune disorders as well." [Stanley W.  Jacob, M.D.]

Nutrient

Essential Fatty Acids

Flax seed oil and/or fish oil have been shown to reduce the severity of the disease in animal studies.  One tablespoon flax seed oil bid is recommended.

Vitamins

Vitamin B6 (Pyridoxine)

Vitamin B6 at a dosage of 500mg tid causes some patients to feel better.  Side effects such as pain, numbness and weakness in the limbs are a possibility at this dose.  If found to be beneficial, supplementation may need to be long term or permanent.

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Weak or unproven link: may be a sign or symptom of; may suggest; may increase risk of; is very occasionally misdiagnosed as
Weak or unproven link:
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