Saturday, August 27, 2011

Comorbid Diseases: Scleroderma (By request)

            I was asked to write an article on the subject of sceroderma, one of the numerous diseases that can be comorbid (occur together) with lupus. Some comorbid autoimmune diseases have enough individual characteristics to warrant separate diagnoses; others have a specific diagnosis that means that the patient has symptoms of two diseases. Scleroderma is one such disease. If it occurs by itself it is called scleroderma. If it occurs in conjunction with lupus, it may be called Mixed Connective Tissue Disease. To make it more confusing, of course, different doctors have different criteria by which they decide on which diagnosis (or diagnoses) to give. So please keep in mind that the dividing lines between the diseases are fuzzy, and your doctor may use a term slightly differently from the way I use it. When in doubt, of course, go with your physician's definition. With that caveat aside, I offer my best attempt at...

Scleroderma 101:

Name: I realize that this probably makes me a huge(r) geek, but I find that knowing the roots of disease names or medical terms is helpful. So please excuse a momentary diversion into linguistics. Scleroderma comes from two words: “sclero,” which comes from the greek word for hardness, and derma, which comes from the greek word for skin. (Hence “dermatologist” --a person who studies  skin). So “scleroderma” literally means “hard skin.”

Cause: It is probably unnecessary  for me to say this at this point, but the root cause of scleroderma is unknown. It is an autoimmune disease that, like lupus, occurs more frequently in women than in men. Such differences in disease incidence generally lead to suspicions that hormone levels may contribute to the disease, but that has yet to be proven.

Symptoms: The major characteristic of any form of scleroderma is a hardening of tissues in the body. This thickening is caused by the fact that the  body is going nuts producing collagen, which is a protein normally found in scar tissue. Basically, the body starts trying to build up scar tissue in random places for no apparent reason. If you think about how different scar tissue is to, for example, your lungs, you can see how having a ton of collagen dumped in organs might cause problems. Generally scleroderma is, again like lupus, divided into two classes:
Localized Scleroderma: This form of the disease is scleroderma’s equivalent to discoid lupus. It occurs on the skin, rather than throughout the body. Localized scleroderma will usually cause a hardening of the skin around the face and hands. This hardening can cause hands to be stiff, and can cause a person to develop Raynaud’s Phenomenon. It can also cause the skin to get darker or lighter, odd sores to develop and (again like lupus) hair loss. The good news is that localized scleroderma rarely causes life-threatening problems.

Systemic Scleroderma: This form is, unsurprisingly, scleroderma’s equivalent to systemic lupus. Like systemic lupus, it shows up differently in different patients. There are subcategories to systemic scleroderma, which I’d be happy to explain in another post if anyone is interested. For now, the most important thing is that instead of just dumping extra collagen protein under the skin, the body is putting it in a variety of places in the body.
            Some of the most common places (other than skin and hands) for systemic sclerosis to occur are the esophagous and the lungs. If the esophagous starts to harden it can make it impossible for someone to swallow. It also makes it difficult for the body to keep stomach acid from traveling back up the digestive tract, which leads to severe acid reflux. When scleroderma occurs in the lungs it can cause life-threatening disease. Your lungs need to be very flexible in order to allow oxygen from the air to enter into the blood stream. If the lungs start being turned into scar tissue, it can block this airflow, making it so that a person cannot get the oxygen (s)he needs in order to live.
            The key to understanding scleroderma is to realize that where the disease strikes, it turns tissues and organs into stiff scar tissue. If you start from there, all you have to do is think, “What would happen if organ/tissue/etc X got turned into one large lump of scar tissue protein.” From there the symptoms of the disease follow.

            Since scleroderma is an autoimmune disease, most treatments aim to either a) decrease the immune system’s ability to do damage in the first place or b) treat the symptoms caused by the disease.
Under category “a,” there are corticosteroids, methotrexate, and many other immunosuppressants with which lupus patients are all too familiar. As in the case of lupus, these treatments run a fine line between trying to knock out the over-active autoimmune response without killing the immune system off so badly that it cannot fight infections.
Under category “b,” there are a very wide range of treatments designed to treat specific symptoms. While this will make photosensitive patients like me cringe at the  very thought of it, sometimes scleroderma patients will undergo light therapy, which can help improve the hardening of the skin. There are also the usual painkillers and NSAIDs (Advil, Celebrex, Aleve, etc). Doctors can also prescribe drugs to change the behavior of blood vessels, make it easier to breathe, etc. It really does depend on where in the body the scleroderma is occurring.

Sources and additional notes:
Most of the information in this post came from the following sources. For more detail, I’d recommend taking a look at them :

My usual disclaimer applies. I am not a physician, nor can I promise that my brain fog will allow me to write without making mistakes. Please do not ever use my explanations as a substitute for talking to your doctor!

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