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Lymphedema and Autoimmune Diseases

Notice: I am not a doctor nor am I trained in any medical profession. The information presented here is my own opinion based on experience and research

Very often in our many support groups, lymphedema patients will speak of other conditions they have and ask if they are connected. In doing research into that question and the particular disease in question, I look for how many patients have that particular condition. If only one or if only a few have it, then I generally consider it not really connected.

However, if many patients speak of a condition, then there may be a “trend” and I look for possible connections. This is the case with autoimmune diseases. There are so many lymphedema patients that also have autoimmune diseases that I am convinced that there is a connection between the two.

What are autoimmune diseases?

Our bodies have an immune system, which is a complex network of special cells and organs that defends the body from germs and other foreign invaders. At the core of the immune system is the ability to tell the difference between self and nonself: what's you and what's foreign. A flaw can make the body unable to tell the difference between self and nonself. When this happens, the body makes autoantibodies (AW-toh-AN-teye-bah-deez) that attack normal cells by mistake. At the same time special cells called regulatory T cells fail to do their job of keeping the immune system in line. The result is a misguided attack on your own body. This causes the damage we know as autoimmune disease. The body parts that are affected depend on the type of autoimmune disease. There are more than 80 known types.

Overall, autoimmune diseases are common, affecting more than 23.5 million Americans. They are a leading cause of death and disability. Yet some autoimmune diseases are rare, while others, such as Hashimoto's disease, affect many people.

Read more about what an autoimmune disease is below.

Causes of Autoimmune Diseases

Are they inherited? The genes people inherit contribute to their susceptibility for developing an autoimmune disease. Certain diseases such as psoriasis can occur among several members of the same family. This suggests that a specific gene or set of genes predisposes a family member to psoriasis. In addition, individual family members with autoimmune diseases may inherit and share a set of abnormal genes, although they may develop different autoimmune diseases. For example, one first cousin may have lupus, another may have dermatomyositis, and one of their mothers may have rheumatoid arthritis. (1)

Autoimmune Diseases in Children

Autoimmune diseases in children are generally rare, and when they occur they can be challenging to diagnose and difficult to treat. Doctors are still learning about this large group of mostly chronic illnesses—more than 80 in all—and most have no cure yet. If your child has an autoimmune problem, much depends on figuring out what it is and treating it aggressively.

The most common autoimmune diseases that occur in children include: celiac disease; type 1 diabetes; lupus (SLE); juvenile dermatomyositis; scleroderma; juvenile idiopathic arthritis (JIA)

Tips for getting a proper diagnosis of an autoimmune disease

1. Do your own family medical history.

2. Keep a “Symptoms” list.

3. Seek referrals to good physicians.

4. Inquire about the physician and hospital experience with

5. Obtain a thorough clinical examination.

6. Get a second, third, fourth opinion if necessary.

7. Partner with your physicians to manage your disease.

8. Learn to deal early on with the long-term effect of autoimmune disease.

Our thanks to the Society for Women Health Research, “Definitive Diagnosis of Autoimmune Disease Often Difficult To Nail Down,” for additions to AARDA list

How is lymphedema connected to an autoimmune disease?

First, one has to understand the pathophysiology of lymphedema. Lymphedema is an inflammatory process and causes inflammation. It is also a disorder involving the connective tissue. Finally, lymphedema leads the patient susceptible to infections as the cell mediated immune response is deficient or hindered.

As a result of these, all too often our bodies seem to be in a constant state of “fight or flight.” The immune system tries to work overtime to meet the perceived threats.

There are also some of us who have actually had bloodwork done that shows up the possibility of an autoimmune disorder. My sister and I are both in that category.

The connection between Lymphedema and Autoimmune disease

Autoimmune disease has three strong associations with lymphedema.

First, it is a disorder of the connective tissue.

Secondly, it has been clearly documented that inflammation plays a major role in autoimmune disorders.

Thirdly, it has also been shown that infection can play an important role in autoimmune diseases.

Does Lymphedema Cause an Autoimmune Disease?

A flat simple answer would be that at this time, there is no hard evidence to say yes.

This ia an area that desperately needs further research. I personally am convinced there is a tie in. While lymphedema may not cause autoimmune disorders, I believe it can lead you to be more susceptible to getting one.

For extensive links on specific autoimmune diseases, immunity, hypersensitivity and immunologic deficiency syndromes please see our Autoimmune Resource Directory

Pat O’Connor

June 25, 2006


A basic way in which the body reacts to infection, irritation or other injury, the key feature being redness, warmth, swelling and pain. Inflammation is now recognized as a type of nonspecific immune response.

More information: In technical terms, the inflammatory response directs immune system components to the site of injury or infection and is manifest by increased blood supply and vascular permeability which, in technical terms, allows chemotactic peptides, neutrophils, and mononuclear cells to leave the intravascular compartment.

Microorganisms are engulfed by phagocytic cells (e.g., neutrophils and macrophages) in an attempt to contain the infection in a small-tissue space. The response includes attraction of phagocytes in a chemotactic gradient of microbial products, movement of the phagocyte to the inflammatory site and contact with the organism, phagocytosis (ingestion) of the organism, development of an oxidative burst directed toward the organism, fusion of the phagosome and lysosome with degranulation of lysosomal contents, and death and degradation of the organism. When quantitative or qualitative defects in neutrophil function result in infection, the infection usually is prolonged and recurrent and responds slowly to antimicrobial agents. Staphylococci, gram-negative organisms, and fungi are the usual pathogens responsible for these infections.


The growth of a parasitic organism within the body. (A parasitic organism is one that lives on or in another organism and draws its nourishment therefrom.) A person with an infection has another organism (a “germ”) growing within him, drawing its nourishment from the person.

The term “infection” has some exceptions. For example, the normal growth of the usual bacterial flora in the intestinal tract is not usually considered an infection. The same consideration applies to the bacteria that normally inhabit the mouth. (1)

Connective tissue

A material made up of fibers forming a framework and support structure for body tissues and organs. Connective tissue surrounds many organs. Cartilage and bone are specialized forms of connective tissue. All connective tissue is derived from mesoderm, the middle germ cell layer in the embryo. (1)

What is an Autoimmune Disease

Autoimmune disorders are diseases caused by the body producing an inappropriate immune response against its own tissues. Sometimes the immune system will cease to recognize one or more of the body’s normal constituents as “self” and will create autoantibodies – antibodies that attack its own cells, tissues, and/or organs. This causes inflammation and damage and it leads to autoimmune disorders. The cause of autoimmune diseases is unknown, but it appears that there is an inherited predisposition to develop autoimmune disease in many cases. In a few types of autoimmune disease (such as rheumatic fever), a bacteria or virus triggers an immune response, and the antibodies or T-cells attack normal cells because they have some part of their structure that resembles a part of the structure of the infecting microorganism.

Autoimmune disorders fall into two general types: those that damage many organs (systemic autoimmune diseases) and those where only a single organ or tissue is directly damaged by the autoimmune process (localized). However, the distinctions become blurred as the effect of localized autoimmune disorders frequently extends beyond the targeted tissues, indirectly affecting other body organs and systems.

Autoimmune disorders are diagnosed, evaluated, and monitored through a combination of autoantibody blood tests, blood tests to measure inflammation and organ function, clinical presentation, and through non-laboratory examinations such as X-rays. There is currently no cure for autoimmune disorders, although in rare cases they may disappear on their own. Many people may experience flare-ups and temporary remissions in symptoms, others chronic symptoms or a progressive worsening. Treatment of autoimmune disorders is tailored to the individual and may change over time. The goal is to relieve symptoms, minimize organ and tissue damage, and preserve organ function. New treatments and a greater understanding of autoimmune disorders are being researched. Patients should talk to their doctors and to any specialists they are referred to about their treatment options. Lab Tests Online

There are more than 80 types of autoimmune diseases, and some have similar symptoms. This makes it hard for your health care provider to know if you really have one of these diseases, and if so, which one. Getting diagnosed can be frustrating and stressful. In many people, the first symptoms are being tired, muscle aches and low fever. The diseases may also have flare-ups, when they get worse, and remissions, when they all but disappear. The diseases do not usually go away, but symptoms can be treated.

Treatments for autoimmune diseases

Of first importance in treating any autoimmune disease is the correction of any major deficiencies. An example would be replacing hormones that are not being produced by the gland, such as thyroxin in autoimmune thyroid disease or insulin in type one diabetes. In autoimmune blood disorders, treatment may involve replacing components of the blood by transfusion.

Second in importance is the diminishing of the activity of the immune system. This necessitates a delicate balance, controlling the disorder while maintaining the body\ ability to fight disease in general. The drugs most commonly used are corticosteroid drugs. More severe disorders can be treated with other more powerful immunosuppressant drugs, such as methotrexate, cyclophosphamide, and azathioprine. All of these drugs, however, can damage rapidly dividing tissues, such as the bone marrow, and so are used with caution. Intravenous immunoglobulin therapy is used in the treatment of various autoimmune diseases to reduce circulating immune complexes. Some mild forms of rheumatic autoimmune diseases are treated by relieving the symptoms with nonsteroidal anti-inflammatory medications. Drugs that act more specifically on the immune system, for example, by blocking a particular hypersensitivity reaction, are being researched. (2)

What causes Autoimmunity?

The immune system normally can distinguish “self” from “non-self.” Some lymphocytes are capable of reacting against self, resulting in an autoimmune reaction. Ordinarily these lymphocytes are suppressed. Autoimmunity occurs naturally in everyone to some degree; and in most people, it does not result in diseases. Autoimmune diseases occur when there is some interruption of the usual control process, allowing lymphocytes to avoid suppression, or when there is an alteration in some body tissue so that it is no longer recognized as “self” and is thus attacked. The exact mechanisms causing these changes are not completely understood; but bacteria, viruses, toxins, and some drugs may play a role in triggering an autoimmune process in someone who already has a genetic (inherited) predisposition to develop such a disorder. It is theorized that the inflammation initiated by these agents, toxic or infectious, somehow provokes in the body a “sensitization” (autoimmune reaction) in the involved tissues. (2)

Autoimmune Disease and Infections

Excerpt from article

To address the question of whether autoimmune diseases can be induced by infections, first autoimmunity needs to be defined. Autoimmune disease occurs when a response against a self-antigen(s) involving T cells, B cells, or autoantibodies induces injury systemically or against a particular organ. Understanding of autoimmune diseases is hindered by the fact that some level of autoimmunity, in the form of naturally occurring autoantibodies and self-reactive T and B cells, is present in all normal persons (6). Thus, on a proportional basis, developing autoimmune disease is the relatively uncommon consequence of a common autoimmune response. Although an autoimmune response occurs in most persons, clinically relevant autoimmune disease develops only in susceptible persons Given those circumstances, how can infections induce autoimmune disease? A mechanism often called on to explain the association of infection with autoimmune disease is “molecular mimicry,” that is, antigens (or more properly epitopes) of the microorganism closely resemble self-antigens. The induction of an immune response to the microbial antigen thus results in cross-reaction with self-antigens and induction of autoimmunity (10). Although epitope-specific cross-reactivity between microbes and self-tissues has been shown in some animal models (11,12), molecular mimicry has not been clearly demonstrated to occur in human diseases (13). Another possibility is that microorganisms expose self-antigens to the immune system by directly damaging tissues during an active infection. This mechanism has been referred to as the “bystander effect” (14,15). However, whether pathogens mimic self-antigens, release sequestered self-antigens, or both, is difficult to determine.

In addition to antigen-specific mechanisms, nonspecific mechanisms could also lead to autoimmunity after infection (9,16). Activation of the innate immune system is essential for a protective adaptive immune response to develop; and vaccines that lack intrinsic activation of innate immunity (e.g., subunit vaccines) require microbial adjuvants to be immunogenic (17). Historically, adjuvants are considered to stimulate immune responses nonspecifically. A renewed understanding of the critical role of innate immunity in influencing the development of an adaptive immune response has led researchers to a better understanding of “the adjuvant effect” (16). Although innate immune cells do not respond to specific antigenic epitopes on pathogens, they do produce restricted responses to particular classes of pathogens through pattern-recognition receptors (PRR), such as Toll-like receptors (TLR) (18).

Interaction of the microorganism component of adjuvants with PRR on innate immune cells results in activation of antigen-presenting cells and upregulation of molecules essential for antigen presentation, such as major histocompatibility complex (MHC) class II and B7–1/2, as well as production of proinflammatory cytokines. This activation of PRR by the microbial components of adjuvants stimulates the immune response in a manner similar to pathogens such as bacteria or viruses (16,18). The pathogen-specific innate immune response is not the same as the nonspecific activation that occurs after mechanical tissue damage, such as during surgery. During mechanical injury, self-antigens and cytokines are released without consistently stimulating pathogen-specific responses. Autoimmune disease rarely develops and usually resolves spontaneously, as seen in postcommissurotomy syndrome (or postcardiotomy syndrome). Adjuvants (usually bacterial, e.g., Mycobacterium in complete Freund's adjuvant) activate the innate immune response in the same pathogen-specific manner when administered with self-antigen; this process results in organ-specific autoimmune disease in animal models (9,16). Adjuvant alone (without self-antigen) does not usually result in autoimmune disease, and microorganisms likely provide not only the adjuvant effect to stimulate the immune response but also the damage necessary to make self-antigens available to the immune system, resulting in autoimmune disease.

To determine whether infection can lead to autoimmune disease, direct evidence (e.g., the ability to transfer autoimmune disease), indirect evidence (e.g., the ability to reproduce autoimmune disease in animal models), and circumstantial evidence (e.g., the association of autoantibodies with disease in appropriate clinical settings) should be considered (3,6). The best evidence so far that infections can induce autoimmune diseases comes from animal models. In most animal models of autoimmunity, including myocarditis, disease has been transferred to naïve animals with autoimmune cells (splenocytes or T cells), autoantibodies (7), or both, which provides compelling evidence that infections induce autoimmune diseases by immune-mediated mechanisms.


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Classification and codes


2008 ICD-9-CM Diagnosis 279.4

Autoimmune disease not elsewhere classified

  • A condition in which the body recognizes its own tissues as foreign and directs an immune response against them.
  • A disorder resulting from loss of function or tissue destruction of an organ or multiple organs, arising from humoral or cellular immune responses of the individual to his own tissue constituents. It may be systemic (e.g., systemic lupus erythematosus), or organ specific, (e.g., thyroiditis).
  • Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.
  • 279.4 is a specific code that can be used to specify a diagnosis
  • 279.4 contains 4 index entries

279.4 also known as: · Autoimmune disease NOS 279.4 excludes: · transplant failure or rejection (996.80-996.89)

OMIM 109100

DiseasesDB 28805

MedlinePlus 000816

MeSH D001327

Resource Directory Autoimmune Diseases

Lymphedema People Internal Links

Lymphedema People Resources

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