Systemic lupus erythematosus

CHAPTER 3 Systemic lupus erythematosus

1. Western medical aetiology and pathology 54

(1) Genetic factors 54

(2) Immune system disorder 54

(3) Hormonal factors 55

(4) Environmental factors 55

2. Diagnosis by Western medicine 56

3. Chinese medicine aetiology and pathology 57

(1) Deficiency of Yin is the internal cause of lupus 58

(2) Invasion of pathogenic factors is the external cause of SLE 58

(3) Blood stasis, Phlegm and Fluid are pathogenic substances, as well as one cause of SLE, and an aggravating factor in existing SLE 58

Differentiation and treatment 60

1. Domination of Heat-toxin 60

2. Internal Heat due to Yin deficiency 61

3. Blood stasis and Heat stagnation 62

4. Spleen and Kidney Qi deficiency 62

5. Fluid retention due to Yang deficiency 63

(3) Central nervous system 65

(4) Blood vessels 65

(7) Raynaud’s phenomenon 66

Case studies 66

5. Analysis of the cases 72

6. Lifestyle prescriptions and health issues 76

Systemic lupus erythematosus (SLE), commonly called lupus, is an autoimmune disease in which the immune system attacks the body, leading to inflammation and damage to the joints, skin, kidneys, heart, lungs, blood vessels, nervous system and other tissues.

1 Western medical aetiology and pathology

The cause of SLE, a complex disease, is unknown, but it is likely to be multifactorial, including genetic factors, disorders of the immune system, and environmental and hormonal factors working together. Once the disease has developed, it manifests as injury and dysfunction of specific organs, tissues and cells.

The signs and symptoms of SLE differ from person to person, and the disease can range from mild to life threatening. The initial symptoms of SLE may begin with a fever followed by one or more of these common symptoms: arthralgia or arthritis, constant fatigue, skin rashes, anaemia, swollen ankles due to kidney involvement, chest pain upon deep breathing due to pleurisy, a butterfly-shaped rash across the cheeks and nose, photosensitivity, hair loss, blood clotting, often painless mouth ulcers, Raynaud’s phenomenon, seizures or other neurological disorders, such as mild cognitive dysfunction, organic brain syndrome, peripheral neuropathy, sensory neuropathy, psychological problems (including personality changes, paranoia, mania and schizophrenia), transverse myelitis, and paralysis and stroke. No two patients with SLE have identical symptoms and complaints.

(1) Genetic factors

Genetic factors may play a very important role in SLE. Several different genes may be involved in determining a person’s likelihood of developing the disease, which tissues and organs will be affected, and its severity. However, it is unlikely that genes alone determine who will develop SLE. Other factors that may play a role include infectious agents such as viruses, certain medications, sunlight and immune system disorder.

(2) Immune system disorder

A Autoantibodies

Antibodies circulating in response to a large number of autoantigens characterize SLE. These autoantibodies are blood proteins that act against the body’s own parts. Antinuclear antibodies, usually measured by immunofluorescence, are present in almost all patients with SLE; tests for antinuclear antibodies, which are sensitive but not specific, constitute a screening test for this autoimmune illness. A more disease-specific test for SLE is the measure of antibodies against native DNA. There is good direct evidence that these antibodies play a major role in pathogenic mechanisms leading to systemic and organ-specific disease manifestations. However, they must first join together with complex, described below, to complete the process of tissue injury that triggers SLE.

B Complex

The complement system has important protective functions in both the innate and the adaptive immune systems, but can also, when activated inappropriately, cause tissue damage. ‘Complement deficiency predisposes to infection and also to development of autoimmune disease, especially SLE, and complement is at the same time involved in the pathogenesis of this disease.’¹ The traditional view of the pathogenesis of SLE is that immune complexes containing autoantigens (tissue and cells) and autoantibodies activate complement, and that this causes inflammatory injury to tissues.

Abundant evidence indicates the key role of immune complex deposition in the aetiology of SLE. Many patients have serological findings of raised anti-DNA levels and depressed levels of complement; biopsied tissue examined using immunofluorescence shows evidence of immunoglobulin and complement deposition. Immune complex deposits can provoke an SLE flare-up. Complex formation could occur in the circulation with antibodies binding to DNA or nucleosomes released into the circulation by dead or dying cells.

C Apoptosis

Investigation into the development of autoantibodies has looked at the response to the mechanism of tissue and cell injury that culminates in the inflammatory cascade. Once activated, the complement system promotes inflammation. In the complexes that result, anti-DNA antibodies appear to be important instigators of cell injury. These antibodies are part of a spectrum of antibodies directed at the nucleosome. The nucleosome is the form of DNA found in the cell nucleus and has both protein and nucleic acid components. In this form, DNA is wrapped around a histone core into a complex that is the essential building block of chromatin. As antibodies can bind to intact nucleosomes as well as isolated components, individual patients can concomitantly express antibodies to DNA, histones and nucleosomes. ‘A hereditary defect in the induction of apoptosis as a mechanism of elimination of autoreactive lymphocytes does not seem to be a prerequisite factor for the development of autoimmune disorders.’²

Insight into the pathogenesis of the disease, apoptosis, clearance of apoptotic cells, and how to reduce the occurrence of apoptosis in SLE has shed a new and intriguing light on the development, course and prognosis of the disease.

(3) Hormonal factors

SLE occurs most frequently in women of childbearing years. This may be an effect of oestrogen’s influence on the immune system. Other explanations for a high female to male ratio include an oestrogen-sensitive threshold mechanism ³ and sex differences of exposure to exogenous agents.

(4) Environmental factors

Although infectious and sunlight triggers of SLE have long been suspected, no single infective agent has been found. Universal exposure of children with SLE to Epstein–Barr virus has been noted, suggesting a possible link of this virus with the disease. Autoantibodies can be identified in serum specimens up to a decade before the earliest symptoms of SLE. Autoantibodies first appear as one specific antibody, then generalize into other types just before clinical onset.

2 Diagnosis by western medicine

Diagnosing SLE can be difficult because its symptoms come and go and mimic those of other diseases. There is no single laboratory test that can definitively prove that a person has this complex illness. However, if SLE is suspected, the following generally occur in order to reach a positive diagnosis:

• A thorough and accurate medical history

• Complete physical examination

• Skin biopsy

• Kidney biopsy.

When a person is positive for four or more of these criteria, the diagnosis of SLE is strongly suggested.

The 11 symptoms and signs used for diagnosing SLE are:

1. Malar (over the cheeks of the face) ‘butterfly’ rash

2. Discoid skin rash: patchy redness that can cause scarring

3. Photosensitivity: skin rash in reaction to sunlight exposure

4. Mucous membrane ulcers: ulcers of the lining of the mouth, nose or throat

5. Arthritis: two or more swollen, tender joints of the extremities

6. Pleuritis/pericarditis: inflammation of the tissue lining around the heart or lungs, usually associated with chest pain on breathing

7. Kidney abnormalities: abnormal amounts of proteinuria or clumps of cellular elements called casts

8. Brain irritation, manifested by seizures and/or psychosis

9. Blood count abnormalities: low counts of white or red blood cells or platelets

10. Immunological disorder: abnormal immune tests include anti-DNA or anti-Sm (Smith) antibodies, false-positive blood test for syphilis, anticardiolipin antibodies, lupus anticoagulant, or positive lupus erythematosus (LE) prep test.

11. Antinuclear antibody: positive ANA test.

Note: The LE prep test used in the diagnosis of SLE has relatively low specificity and is insensitive. The ANA test has essentially replaced the LE prep as the test of choice for diagnosis of systemic immune-mediated disease, as it is fairly specific and more sensitive.

There are several kinds of SLE:

• Systemic lupus erythematosus is the most common form of the disease that people are referring to when they say ‘lupus’. The disease can affect many parts of the body. The symptoms of SLE may be mild or serious. Although SLE usually first affects people between the ages of 15 and 45 years, it can occur in childhood or later in life as well. This is the kind of SLE discussed in this chapter.

• Discoid lupus erythematosus is a chronic skin disorder in which a red, raised rash appears on the face, scalp or elsewhere. The raised areas may become thick and scaly, and may cause scarring. The rash may last for days or years and may recur. A small proportion of people with discoid lupus have or develop SLE later.

• Subacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body exposed to sunlight. The lesions do not cause scarring.

• Drug-induced lupus is a form of lupus caused by medications. Many different drugs can cause drug-induced lupus. Symptoms are similar to those of SLE (arthritis, rash, fever and chest pain) and they typically go away completely when the drug is stopped.

• Neonatal lupus is a rare disease that can occur in newborns of women with SLE, Sjögren’s syndrome or no disease at all. Scientists suspect that neonatal lupus is caused by autoantibodies in the mother’s blood, including anti-Ro (SSA) and anti-La (SSB). At birth, the babies have a skin rash, liver problems and low blood counts. In rare instances, babies with neonatal lupus may have a serious heart problem that slows down the natural rhythm of the heart. Neonatal lupus is rare, and most infants of mothers with SLE are entirely healthy. All women who are pregnant and known to have anti-Ro (SSA) or anti-La (SSB) antibodies should be monitored by echocardiography during weeks 16 and 30 of pregnancy.

3 Chinese medicine aetiology and pathology

Although SLE has multiple and complicated symptoms, Chinese medical theory suggests the primary pathogenic cause is almost always due to Yin deficiency.⁴ Chinese medicine posits that SLE is a deficiency disease due to Zhen Yin, , true Yin, insufficiency – this primary problem can occur in the early or late process of SLE. Specifically, the theory describes the problem as ‘a deficiency in the disease origin and an excess in the disease manifestation’. Internal Heat due to Yin deficiency is the principal cause. When patients with a long history of illness have developed a Qi and Yang deficiency, Yin and Blood deficiency, or both Yin and Yang deficiency, they eventually also develop Yin, Yang, Qi and Blood deficiency coupled with Blood stasis. In the initial stage, deficiency may reside in the Liver, Spleen and/or Kidney. In the later stage, deficiency may reside in all five Yin organs. The disease may damage the Heart, Lung, Liver, Kidney and Spleen, and their corresponding tissues, including the brain, skin, hair, nails, muscles and joints, as well as the Ying, or nutritive Qi, and the Xue, or Blood, and the entire body.

(1) Deficiency of Yin is the internal cause of lupus

When Zhen Yin flourishes, pathogenic factors cannot invade the body easily and cause diseases such as SLE. Deficiency of Zhen Yin, especially Kidney Zhen Yin, is very important in the development and course of SLE. Kidney Essence is the foundation of life, which originates from the conjugation of the innate reproductive essence of both sexes. This means the individual Kidney Zhen Yin and Essence come from both parents. The strength of the Zhen Yin and Essence depends on the quality of parental genes. Congenital deficiency is the internal cause of SLE.

According to Chinese medicine, women are energetically different from men. Women’s bodies belong to Yin and the Liver. The Liver tends to Yin deficiency, which may be the reason why SLE occurs more in women than in men.

According to clinical research at the Traditional Chinese Medical Hospital of Shanghai, in 90% of 142 patients with SLE there was Yin deficiency.⁴ Most researchers in Chinese medicine realize that Yin deficiency may be the primary pre-existing problem in patients prior to the occurrence of SLE symptoms.

(2) Invasion of pathogenic factors is the external cause of SLE

While Zhen Yin deficiency is an important factor in the occurrence and development of SLE, not everyone, including members of the same family who have been diagnosed with SLE, have the same symptoms and signs. Therefore, it seems apparent that there are other triggers of SLE, such as result from an attack by the six exogenous factors that all cause different symptoms and signs.

There is always conflict between vital Qi, which tries to maintain the body’s health, and pathogenic factors. Such conflict is not only related closely to the initial occurrence of a disease but it also affects the development and final outcome of the condition. It has some direct influence on the sthenic or asthenic transformation of the disease, meaning that the strength of vital Qi will influence an individual’s healthy condition and can determine the gravity and prognosis of a disease. Therefore, the way a disease progresses actually depends on the conflict between vital Qi and pathogenic factors.

During the conflict, vital Qi and the pathogenic factors can influence one another. When vital Qi is exuberant, it can suppress pathogenic factors and so prevent the occurrence of disease. However, if vital Qi is deficient or the pathogenic factors are in excess, this can lead to the failure of vital Qi in conquering the pathogenic factors and the person will become sick.

(3) Blood stasis, Phlegm and Fluid are pathogenic substances, as well as one cause of SLE, and an aggravating factor in existing SLE

Blood stasis is a pathological state. It results from: (1) the reverse or impeded flow of Blood in the body; (2) stagnation of Blood flow in local areas, or (3) the abnormal presence of Blood outside the blood vessels that fails to disperse or to be reabsorbed. Once Blood stasis has formed, it can further compromise the circulation of Blood and lead to still other pathological changes and a variety of diseases and syndromes. Therefore, Blood stasis is both a pathogenic factor and also the result of pathological abnormalities.

Retention of Phlegm and Fluid is caused by accumulation of Fluid due to a disturbance of metabolism. Retention of Phlegm and Fluid mostly occurs following either an attack by the six exogenous factors, an improper diet or internal damage from the seven emotions causing Qi not to move smoothly. The result is accumulated Fluid in the body. Once this complex is formed it can follow the same pathways as Qi circulating everywhere in the body, causing a number of diseases and symptoms. For instance, Phlegm stagnation in the Lung can cause cough with sputum and dyspnoea. Phlegm stagnation in the Heart can cause palpitations, chest pain and oppression, and mental confusion, coma and dementia.

Other examples of excess conditions include Fire, Heat-Toxin, Fire-Toxin, stagnant Fire, Heat in the Blood, Blood stasis, Wind-Dampness, chronic oedema and Cold-Dampness.

The term lupus has no equivalent in Chinese medicine, but there are records that describe similar conditions, particularly referring to damage of specific tissue.

(1) Skin damage

In the early Han Dynasty, Zhang Zhongjing’s Jin Gui Yao Lue/Synopsis of the Golden Chamber describes Yin Yang Du , Yin and Yang-toxin. The chapter on this topic says that Yin toxin and Yang toxin can occur simultaneously as Yin and Yang toxin. Their main clinical symptom is red spots on the face and pain in the whole body. Other books that address this same condition include Cao Yuangfang’s Zhu Bing Yuan Hou Lun/General Treatise on the Causes and Symptoms of Diseases , written during the Sui Dynasty, and Zhu Danxi’s Dan Xi Xin Fa/Danxi’s Experiential Therapy , written during the Yuang Dynasty, which records Yin Yang Du as having symptoms of fever and also cold hands and feet.

(2) Joint and organ damage

Thousands of years ago there were already records of lupus-like symptoms in medical books and articles. For instance, Huang Di Nei Jing/The Yellow Emperor’s Inner Classic , Jin Gui Yao Lue/Synopsis of the Golden Chamber , Zhu Bing Yuan Hou Lun/General Treatise on the Causes and Symptoms of Diseases , and Jing Yue Quan Shu/Complete Works of Zhang Jingyue have all described lupus-like pathologies.

Chinese medicine identifies these diseases as Feng Shi Bin, Feng Shi Tong, Bi Zheng, Wong Bi and Zhou Bi. These are all different names for Bi syndrome, which is used to describe symptoms of pain in the joints and muscles, swelling of joints and fever. They may be related to autoimmune diseases, such as rheumatoid arthritis and SLE. Jin Gui Yao Lue/Synopsis of the Golden Chamber refers to Xuan Yin pleural effusion or fluid retention in the hypochondrium. It also refers to Yin Ji Xin Xia, or hydropericardium, which is Fluid retention in the pericardium that can cause oedema and heart failure. Jin Gui YaoLue/Synopsis of the Golden Chamber and Zhu Bing Yuan Hou Lun/General Treatise on the Causes and Symptoms of Diseases both mention Shen Zhuo Zheng, which refers to a heavy sensation in the body, caused by Dampness. Its clinical symptoms are lumbago, a cold feeling in the lumbar region, swelling on the face, eyelids and ankles, decreased urination, and a swollen stomach from ascites. These symptoms and signs sound similar to nephritis and hypoproteinaemia, which is caused by protein being lost in the urine resulting in deficient protein in the serum, causing hypotonic oedema.

Clinical symptoms recorded in Chinese medical books over the past several thousand years appear similar to those of SLE and include Fa Re , fever, Wen Du Fa Ban , epidemic disease with eruptions, Xu Lao , consumptive diseases, Bi Zheng , arthralgia, and Shui Zhong , oedema.

Differentiation and treatment

1 Domination of heat-toxin

Clinical manifestations

Sudden onset, high fever, flushed face, red rash or purpura dotted over the skin, irritability, anxiety, thirst, insomnia, arthralgia, constipation, and even coma and delirium as well as seizure in severe cases. The tongue is deep red with a yellow coating. The pulse is rapid and taut or rapid and full.

Treatment principle

Clear pathogenic Heat and Toxin, cool Blood, remove Blood stasis and nourish Yin.

Du-14 Dazhui, LI-11 Quchi, LU-11 Shaoshang and LI-4 Hegu.

Supplementary treatment

If the patient has constipation, add Da Huang Rhei Radix et Rhizoma and Zhi Shi Aurantii Fructus immaturus and needle ST-25 Tianshu and TB-6 Zhigou; if the patient has a temperature, add Chai Hu Bupleuri Radix and Huang Qin Scutellariae Radix and needle LIV-2 Xingjian.

2 Internal heat due to Yin deficiency

Clinical manifestations

Low-grade fever or tidal fever in the afternoon, a feverish sensation in the palms and soles, anxiety, perturbation, red skin eruptions, night sweats, fatigue, irritability, insomnia, thirst with no desire to drink, preference for cold water, aching pain in the joints and baldness. The tongue is red with a fissure and less coating, or it is shiny and mirror-like. The pulse is thready and rapid.

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Tags: Treating Autoimmune Disease with Chinese Medicine

Jan 19, 2017 | Posted by admin in MUSCULOSKELETAL MEDICINE | Comments Off

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