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Journal of Acuherb in Medicine


Systemic Lupus Erythematosus

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By Dr. Kenneth Wang

Systemic lupus erythematosus (SLE) is a chronic, inflammatory disorder of connective tissue that usually arises without obvious cause and is regarded as an autoimmune disease. It affects many different organ systems of the body and varies in severity between patients and, over time, within the same patient.

Typical features of SLE are a symmetrical red rash to the face, arthritis and progressive damage to the kidneys.

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect the joints and many organs of the body, commonly the skin and kidneys. The disease course can range from mildly episodic to severe, and can be fatal. Symptoms vary from individual to individual and are characterized by remission and exacerbation. The complex presentation of the disease can make diagnosis difficult.

No definitive term exists for SLE in the ancient Chinese medical classics. Individuals are diagnosed with different syndromes according to the presentation of symptoms and are treated according to the disharmony patterns displayed under these syndromes.

 

Symptoms of fever and skin rash are diagnosed as yin-yang poisoning, Prurigo, itching dermatitis of the face, cheek sores and solar dermatitis syndromes.
Joint pain, muscle pain and Raynaud’s phenomenon are classified as bi-syndrome.
Heart involvement, causing symptoms of palpitations and shortness of breath, is referred to as palpitation syndrome.
Nervous system symptoms are grouped as hysteria syndrome.
Symptoms resulting from multiple asthenia syndrome organ systems involvement are classified as . This can then lead to consumptive disease.
Kidney involvement is defined as kidney deficiency.

 

The kidneys are very important in TCM and are considered (as in Western medicine) to be commonly involved in SLE.


As SLE is a disease that can affect the whole body and involve several organs, different syndromes or disharmony patterns may occur at the same time and present a multiplicity of symptoms.

Causes

The cause of SLE remains unknown, although it is thought that genetic, hormonal, immune and environmental factors have a role. The reasons that support this are: 

 

Relatives of patients with SLE are at increased risk of developing the disease.
The disease is more often seen in both of a pair of identical twins than it is in fraternal (non-identical) twins.
Prevalence of the disease in certain races also implies a genetic (inherited) influence in the occurrence of SLE.
Evidence for hormonal abnormalities is based primarily on the observation that SLE is much more common among women in their childbearing years.


Many abnormalities of the immune system occur in patients with SLE. The reason for this remains unclear and it is not known which are primary and which are secondary causes. These abnormalities in immune regulation are thought to result from loss of “self” (substances belonging to the body) tolerance. This means that SLE patients are no longer totally tolerant of all their self-antigens and consequently an immune response develops to these antigens. 

From the traditional Chinese medicine (TCM) viewpoint, the major cause of SLE is an inborn insufficiency that disturbs the balance of yin and yang. SLE may be acquired when environmental pathogens (dryness, dampness, cold, summer-heat, wind and fire evils) affect this balance. Inborn insufficiencies or acquired disorders also lead to depletion of vital energy (qi) and yin fluid. This can cause impaired functioning of the body and a defect in resistance to illness.

Ancient TCM classics like the Jinkui Yaolue (Summary from the Golden Chest) refer to diseases that poison the yin-yang balance. Constitutional or bodily weakness enables environmental pathogens to invade the body. This disturbs the harmonious balance of yin and yang and illness results. As an example, over-exposure to sunlight, considered to be a heat evil, causes the accumulation of heat and toxic pathogens in the body, and this in turn triggers organ system damage. Diseases where yang is dominant in the body present with yang-type symptoms, such as flushing of the face, high fever, dislike of heat, dark-colored urine or constipation. Diseases dominated by yin present with yin-type symptoms, such as pallor, cold limbs, an aversion to cold, pale urine or watery stools.

Parallels can be made with Western medicine’s understanding of SLE. For example, SLE is a result of the malfunction of the body’s immune system, which in TCM can be interpreted as a yin and yang imbalance. Genetic and environmental factors are also known to play a role in the development of SLE. It has been suggested that a virus may be partially responsible for its cause. This thinking is in line with the TCM concepts of environmental pathogens and an inborn deficiency being linked to the occurrence of SLE.

Symptoms

The onset of SLE can be highly variable, as can be the progression of the disease. Early symptoms may be non-specific and include muscular pain, nausea, vomiting, headaches, depression and easy bruising. Sometimes symptoms are more specific and more than one may be present. Symptoms may be mild or severe, fleeting or persistent.

General symptoms

Fatigue occurs in virtually all patients with SLE and may be experienced with the onset of SLE or only with a relapse of the disorder.
Swelling of the ankles is common.
There is often a gain or loss in body-weight.
Low-grade fever is seen in 80 per cent of patients.
It is usually an episodic disease (occurs from time to time).

Skin and mucous membranes
The skin is affected in over 70 per cent of cases. Often, rashes occur after exposure to ultraviolet light and such exposure may lead to major flares involving other organ systems as well. Abnormal sensitivity to sunlight is experienced by about half of all patients with SLE and is more common in those who are fair and light-skinned.

Typical ”butterfly” distribution facial rash seen in SLE.

A classical characteristic of SLE is the “butterfly” rash that occurs across the face. Hair-loss is also common. Mouth ulcers occur frequently in active disease, but they may be relatively painless. A painful throat may accompany a flare.

Vascular system (blood vessels) 
A disorder of the vascular system known as Raynaud’s phenomenon occurs in 20 to 30 per cent of patients. Classically, the fingers turn white followed by a bluish cyanotic hue, finally replaced by a red flush as dilatation of the blood vessels occurs. The full three-color change does not always happen, but excessive sensitivity of the hands to the cold is common.

Musculoskeletal system
Painful and arthritic joints are the most common symptoms of SLE and experienced by almost all patients. Symptoms can occur in any joint and often clear up in one to three days. Fingers, hands wrists and knees are the most commonly affected. Less frequently, ankles, elbows, shoulders and hips are involved. Stiffness on waking can last several minutes.

Renal system
In 50 per cent of SLE patients, the kidneys become involved. There may be rapid onset of an acute and painful kidney inflammation or traces of blood or protein may be found on analysis of the urine. Urinary tract infections are common.

Nervous system 
Nervous disorders occur in virtually all SLE patients. Anxiety and/or depression are common in response to the illness and the threat of loss of health, family and job. The prospect of disability and dependency can be very distressing.

Symptoms may include insomnia, loss of appetite, constipation, palpitations, diarrhea, over-breathing, memory loss, emotional instability, confusion and decreased concentration. Seizures (convulsions) occur in more than 10 per cent of patients. The incidence of stroke, with resulting abnormalities of sensation and power, is similar.

Headaches are a frequent complaint and are usually due to stress and tension. Migraine occurs in 20 per cent of patients.

Cardiorespiratory system 
Chest pain, cough and breathlessness occur frequently. These result from a variety of processes involving the lungs, the lining of the chest wall and diaphragm. Pain on breathing is commonly experienced and is often accompanied by chest discomfort. It may be worsened by lying down.

Gastrointestinal tract
Involvement of the gastrointestinal tract is common. Up to 25 per cent of patients have esophageal complaints, including difficulty swallowing. Indigestion, abdominal pain, nausea and vomiting are also common.

Blood
Anemia occurs in at least 50 per cent of patients. On laboratory analysis, abnormalities in the blood cells and clotting mechanisms are commonly found.

TCM practitioners will examine the sufferer and categorize the symptoms under special syndrome groups known as “disharmony patterns.” Certain disharmony patterns are present during different stages of the disease. The presentation of SLE is often complicated, but some common patterns can be classified:

Excessive toxic heat accumulation
Individuals present with persistent high-grade fevers, the appearance of a rash on the face or other areas, joint pain, muscle soreness or pain, fatigue, thirst and a desire for cold drinks. Irritability, insomnia, constipation and production of dark-colored, scanty urine are also common.

In severe cases, loss of consciousness, delirium, muscle spasms, bruising or petechiae (small, flat blood spots under the skin), the spitting up of blood and the presence of blood in the urine or stool may also occur. Exposure to sunlight and the ingestion of certain foods or drugs are factors that can exacerbate the disease and induce flare-ups.

blood yin deficiency
In this deficiency there is a persistent low-grade fever, a hot sensation in the chest, palms and soles of the feet, irritability, fatigue and a reluctance to speak. There is general swelling of tissues, redness of the face, spontaneous perspiration and night sweats, and loin and joint pain.
Toxic factor attacking the heart
Symptoms include palpitations, shortness of breath, chest fullness with a feeling of oppression and local heat, spontaneous perspiration, pallor and cold limbs.
Kidney yin deficiency
Individuals usually experience pain in the back, legs and feet, limb weakness and a sensation of hotness in the face. In more serious cases, sores appear in the mouth and on the tongue, hot sensations are felt in the chest, palms and soles of the feet, and the individual feels emotionless. Sometimes low-grade fever and swelling may be present.
Impairment of the liver due to heat
In this disharmony pattern, pain in the upper sides of the abdomen, abdominal distension, irregular and painful menstruation in females, skin rashes, bruising, insomnia, dizziness and loss of appetite are commonly experienced.

Diagnosis

The diagnosis of systemic lupus erythematosus can be difficult. The disease may present in many different ways and most of the laboratory tests carried out on patients with SLE are not specific. For these reasons, other diseases have to be carefully considered before a diagnosis of SLE can be made. Physicians should always consider the possibility of SLE in a disease which is affecting many systems of the body, especially those involving the skin and joints of young women.

In 1982, the American College of Rheumatology compiled a list of 11 criteria, at least four of which should be present, not necessarily at the same time, to make a diagnosis of SLE. In some patients, only one or two criteria may be present initially and after several weeks, months or even years do the others appear. These criteria are:

Characteristic “butterfly” rash on the face
Generalized skin rash
Mouth ulcers
Arthritis
Sensitivity to ultraviolet light
Inflammation of the lining of the chest wall or the membrane surrounding the heart
Renal (kidney) disorder
Nervous system disorder
Low red or white blood cell count
Positive antinuclear antibodies
Positive lupus anticoagulant

These last two are immunologic laboratory tests.

 

Diagnosis in TCM places importance on determining the circumstances and manifestations of a disease through inquiry and symptom observation. Diagnosis is based on the traditional four examination techniques:

Questioning: The TCM practitioner will establish the medical history of both the patient and his family.
Observation: Examination of the physical features of the body, such as the face, tongue, hair, nails, sputum (mucus that is coughed up), and location of pain, all offer clues to the problem. The tongue is a particularly useful indicator of the functioning of the internal organs.
Listening and swelling: The smelling of sputum and breath and listening to the sounds produced by the chest offer additional clues to the patient’s health.
Touching: Feeling the pulse is a cornerstone of TCM diagnosis and gives the practitioner much information about any bodily imbalance.

In SLE, the disharmony patterns present at different stages during the course of the disease and can be described as follows: :

Excessive toxic heat accumulation
In TCM, it is held that the occurrence of SLE is closely related to over-heating of the heart meridian. The heart controls the blood and blood vessels. When the heart pumps, blood is transported within the vessels around the entire body. The heart, blood and blood vessels are thus united by their common activities. When heat pathogens are congested in the heart meridian, it means a serious invasion of the internal organs has occurred. The qi and blood bring these pathogens to every part of the body, damaging internal organs, muscles and skin along the way.

On examination, the tongue appears red or dark red and ulcerated, or sores may be present on the inside of the mouth. The tongue may be coated with a fur that is greasy and yellow, dry and yellow, greasy and white, or the fur may be absent altogether. The pulse feels taut and rapid.

Blood yin deficiency
When the body accumulates too many heat pathogens over a period of time, the body fluids dry up and organs will not function properly. This decreases the production of blood and kidney essence and leads to a morbid condition of general weakness with sweating, low-grade fevers and hot sensations in the palms and soles of the feet.On examination, the tongue appears red with a white fur coating or with no coating. The pulse sign feels thready, rapid and weak.
Toxic factor attacking the heart
Impairment of the functioning of several internal organs signals involvement of the heart and a serious effect to the bloodflow. Individuals present with pallor, palpitations and cold extremities.On examination, the tongue is pale and coated with thin white fur. The pulse feels thready, weak, or choppy.
Kidney yin deficiency
This occurs usually in a late stage of the disease when the pathogens finally invade the kidney. Kidney-yin refers to the fluid and essence stored in the kidneys. The invading pathogens cause a deficiency in the amount of this fluid and essence and the kidneys begin to over-function. This is the yang part of the kidney compensating for the loss of yin and creates a virtual fire in the kidneys. This produces back pain, limb weakness and heatsymptoms such as fever or tongue sores.On examination, the tongue appears bulky, moist and pale. The tip of the tongue may also be red. The pulse feels deep, thready and weak.
Impairment of the liver due to heat
Heat pathogens dry up body fluid and this leads to inadequate nourishment for the liver. In TCM thinking, the liver helps regulate qi and blood movement, and the digestive function of the spleen. Individuals with this disharmony pattern present with pain in the liver region, bleeding problems or loss of appetite.On examination, an enlarged liver and spleen may be palpable.

Treatment

The management of SLE should be comprehensive and include drug and non-drug treatment. Therapy is individualized for each patient depending on the manifestations of disease and their severity. 

Corticosteroids
Systemic corticosteroid therapy is the mainstay of treatment in SLE. However, not all patients require steroids, and steroids can do more harm than good. When needed, oral maintenance doses of prednisone or prednisolone are usually prescribed.

Severe symptoms affecting the whole body, such as sustained high fevers and severe blood disorders, often need to be treated with higher doses of corticosteroids. Involvement of organs such as the heart, lungs, and central nervous system also usually requires treatment with corticosteroids.

A topical steroid is usually prescribed for skin rashes.

NSAIDs
Mild symptoms of arthritis and muscular pain are usually managed with non-steroidal anti-inflammatory drugs (NSAIDs), such as indomethacin, naproxen and ketoprofen. More severe symptoms may need an antimalarial agent and occasionally low doses of corticosteroids may be required.

Antimalarials
The antimalarials chloroquine and hydroxychloroquine possess anti-inflammatory properties and have been found of benefit in treating inflammatory conditions which have an immunologic basis. They are usually used when other first-line therapies (such as NSAIDs) fail. They are prescribed for more severe skin and joint manifestations of SLE.

Immunosuppressants
As long-term corticosteroid therapy, particularly at the higher doses, tends to cause side-effects, immunosuppressants such as azothiaprine, cyclophosphamide and methotrexate are sometimes added in an attempt to lower the dose of corticosteriod required. These drugs suppress the immune response. As immunosuppressants themselves pose a risk of long-term toxicity they need to used with care, but can be useful in the treatment of acute and severe organ involvement.

Psychiatric support
The threat of a chronic disease can be very stressful. Visiting a doctor frequently and having many laboratory tests with long waits for the results can be an emotional burden. Emotional support in the treatment of SLE is essential. It is also important that patients have extensive rest.

Preventive measures 
All possible means to prevent provocation or exacerbation of SLE should be undertaken.  
As SLE is an episodic disease and is characterized by exacerbation and remission, it is extremely important to withdraw therapy slowly when an exacerbation has quieted down. This avoids unnecessary drug toxicity.

Patients with SLE need a to see their doctors at least once every three to six months, even if the disease is inactive. Patients with active disease must be assessed more frequently.

Treatment of SLE from a TCM perspective will depend on the symptoms of the individual patient. In addition to herbal decoctions used to alleviate disharmony pattern symptoms, treatment may include acupuncture for relieving pain and stiffness in the joints, and qi-gong to help focus the energy of the body in fighting the disease and strengthening the mind.

Examples of decoctions used for the different disharmony patterns

Excessive toxic heat accumulation


Therapeutic aim:
To clear away heat and toxins, cool down the blood, protect the yin, remove obstructions in the meridians and clear up rashes.

Prescription:
 Qingying decoction

shi gao gypsum
shu di huang processed rehmannia root
mai dong dwarf lilyturf tuber
zhi mu common anemarrhena root
niu xi achyranthes root

For individuals with high fever, poor concentration and delirium, the dosages of heat-clearing drugs should be increased or the following added:

shang shi gao unprocessed gypsum
huang lian golden thread
da huang rhubarb

Some patent Chinese medicines like Purple Snowy Pill, Bezoar Bolus for Resurrectionand Treasured Bolus can be taken at the same time.

 

Yin blood deficiency

Therapeutic aim:To replenish the yin, nourish and cool down the blood, and eliminate toxins.

Prescription:
 Modified Zhibai Dihuang pill

zhi mu common anemarrhena root
huang bai amur cork-tree
shu di processed rhemannia root
shan yu rou Asiatic cornelian cherry fruit
shan yao common yam root
ze xie oriental water-plantain root
dan pi tree peony bark
fu ling Indian bread

For individuals with spleen deficiency add:

bai zhi large head atractylodes root
chen pi dried tangerine peel
fu ling Indian bread

In cases of very high fever add:

yin hua honeysuckle flower

For dizziness add:

ju hua chrysanthemum flower
chuan xiong Szechwan lovage root

 

Toxic factor attacking the heart

Therapeutic aim:To nourish the yin, replenish the qi, calm the mind, and clear away heat and toxins.

Prescription:
 Modified Huanglian antidote decoction

huang lian golden thread
huang qin baical skullcap root
huang bai amur cork tree
zhi zi Cape jasmine fruit

For spleen deficiency add:

dang shen pilose asiabell root
bai zhi large head atractylodes root

For individuals with a feeling of chest fullness add:

hou po magnolia bark
su geng perilla stem

For yin deficiency add:

yu zhu fragrant Solomon’s seal rhizome
huang jing agastache or Solomon’s seed
dong chong xia cao Chinese caterpillar fungus

 

Kidney yin deficiency

Therapeutic aim:To nourish the yin, tonify the kidneys, activate the blood circulation and eliminate toxins.

Prescription:
 Modified Siwu decoction

dang gui Chinese angelica
chuan xiong Szechwan lovage root
shu di processed rehmannia root
bai shao white peony root

For dampness-heat symptoms in the lower burner add:

che qian zi plantain seed
Bi xie long yam
qu mai Chinese pink herb

For individuals with backache add:

tu si zi dodder seed
chuan duan Himalayan teasel root

For joint pain add:

ji xue teng suberect spatholobus stem
shen jin cao common club-moss herb
liu ji nu diverse wormwood herb
xu chang qing paniculated swallow-wort root

 

Impairment of the liver due to heat

Therapeutic aim:To nourish the yin, cool down the blood, eliminate toxins, activate the blood circulation, clear away blood stasis and remove obstructions in the meridians.

Prescription:
 Modified Xiefu Zhuyu decoction

tao ren peach seed
hong hua safflower flower
dang gui Chinese angelica
sheng di common yam root
chuan xiong Szechwan lovage root
chia hao red peony root
niu xi achyranthes root
jie geng platycodon root
chai hu Chinese tororwax root
zhi qiao orange fruit
gan cao liquorice root

For individuals with severe blood stasis add:

san leng bur-reed tuber
e zhu zedoary rhizome
yi mu cao motherwort herb
chuan xin lian andrographis herb
gui jian yu winged spindle tree

For severe abdominal distention and pain below the ribs add:

hou po magnolia bark
chen pi dried tangerine peel
xiang fu nut-grass

For qi deficiency and residual heat toxins add:

huang qi milk-vetch root
qin jiao large-leafed gentian
wu she black snake

For over heated blood and a well-defined rash add:

ji guan hua cockscomb
mei gui hua rose
ling xiao hua trumpet creeper flower

Dr. Wang who is expert on treatment of SLE  RA with his spacial prepared herb medicine in over thirty year experience in clinic and  with Acupuncture treatment can very good control the disease and stop pain joint swollen.