Scleroderma

CHAPTER 14 Scleroderma



Scleroderma is a diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles and internal organs. It is one type of rheumatic disease and is also called mixed connective tissue disease.



1 Western medical aetiology and pathology


Scleroderma literally means hard skin. It is a symptom of a group of diseases that involves the abnormal growth of connective tissue, which supports the skin and internal organs. In some cases, scleroderma occurs as hard, tight skin; in others, the damage goes deeper, affecting blood vessels and organs, such as the lungs, heart, liver and kidneys.


The cause of scleroderma is generally unknown and it may be the result of several factors working together. However, most researchers think the causes include autoimmunity, environmental exposure, genetics and infections.


T-cell activation plays a pivotal role in the immunopathogenesis. Lymphocyte processes are tightly controlled by molecules that activate either proliferation or apoptosis. Possibly an imbalance in apoptotic function or increasing autoreactive cells may lead to persistent autoreactive phenomena.1


Patients with systemic sclerosis also have altered B-cell homeostasis characterized by increased numbers of native B cells and reduced memory B cells. Although memory B cells are decreased in number, they are chronically activated, possibly because of CD19 overexpression in B cells.2 The immune system and inflammatory activity appears to mirror many other rheumatic disorders. In scleroderma, the disordered immune system stimulates fibroblasts to produce too much of the collagen found in lungs, skin and kidneys. In its milder forms, the effects of this build-up are limited to the skin and blood vessels. In its more serious forms, it also can interfere with the normal functioning of joints and internal organs.


Genetics may also be a predisposing reason why some people are at risk of scleroderma.


Exposure to some environmental factors may trigger the disease in people who are genetically predisposed to it. Suspected triggering factors include viral infections, certain adhesive and coating materials, and organic solvents such as vinyl chloride or trichloroethylene.


Women have a much higher risk of developing scleroderma than men. However, so far, the role of oestrogen or other female hormones has not been proven in the process of scleroderma.


When the lungs are involved, the alveoli and capillaries are damaged and destroyed, leading to pulmonary hypertension. The result is lower oxygen levels in the blood, which may lead to heart failure.




3 Chinese medical aetiology and pathology


Scleroderma is a kind of autoimmune disease caused by sclerosis of the skin. There are two types or scleroderma: localized and systemic. Generally the process has three stages: red skin with swelling, sclerosis and atrophy. Chinese medicine calls this Pi Bi image, or skin hardening.


Wind-Heat or Wind-Cold can invade the body, leading to pathogenic Heat or Cold in the Lung, or Warm diseases develop and impair Yin. This can then lead to deficient Fire singeing the Blood, causing Blood stasis which blocks the channels. As a result, Qi and Blood cannot flow smoothly through the channels, so damaging the skin. These pathogenic factors can also follow the channels into certain organs and impair their function.


Wind-Cold-Dampness can also invade the body. At the start, the pathogens stay in the Lung and cause skin thickness and swelling. The Cold-Dampness directly impairs the Spleen and can cause a Spleen function disorder. This leads to Qi deficiency and loss of the Qi’s function of warming the body and promoting Blood circulation in the blood vessels. If Qi deficiency prevents the Blood from flowing smoothly in the vessels, it can cause the Blood to leave the vessels and become Blood stasis. When the disease lasts for a long time, the pathogens continue to block the channels, affecting the smooth movement of Liver Qi and leading to Liver Qi stagnation. If Qi stagnates for a long time, this will transform to Fire and impair Yin. Yin deficiency and Qi stagnation can aggravate Blood stasis. Eventually, the process of the illness becomes Heat, Yin deficiency, Blood stasis and Qi deficiency.



4 General treatment principle


Pathogenic factors of the illness include Heat, Cold, Yin deficiency, Qi deficiency and Blood stasis. Heat, Cold, and Yin and Qi deficiency all cause Blood stasis or aggravate Blood stasis. Conversely, Blood stasis can aggravate the illness. No matter what the pathogenic factors are, the key pathological change is Blood stasis. Therefore, no treatment will succeed without removing Blood stasis.




Differentiation and treatment






5 Appendix




(2) Pulmonary hypertension







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

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