The real cause of Dupuytren's contracture is not fully understood, but many have classified it as an idiosyncratic type of autoimmune disease.
What is an autoimmune disease?
An autoimmune disease is one where your body’s own immune system, that usually fights against infections and foreign substances in the body and is our body’s main defense system, mistakes a healthy part of our body as something foreign, and attacks it.
Normally, our immune system is intelligent enough to tell our own cells apart from invaders, but in an autoimmune disease it backfires and starts attacking healthy organs and tissues!
There are many kinds of autoimmune diseases and they affect different areas. Some affect a specific organ while others can even affect the whole body!
Why does this happen?
Surprisingly, no one knows the exact reason for this malfunctioning of the immune system yet.
However, research has noticed patterns in it which show that some people are more susceptible to it. For example, women are two times more likely to get it compared to men. Many autoimmune diseases are more common in particular races and ethnicities, and are also passed on through family trees, generation to generation. All these show thatgenetics can have a major role to play here. Other suspected contributors are environmental factors like nutrition and exposure to chemicals.
What is Dupuytren’s Contracture?
Dupuytren’s contracture is a disorder that affects the “fascia” in the palms of the hands, causing them to become thick, hard and fibrous. The tissue shrinks and loses its elasticity which makes the fingers curl or bend inwards. This usually affects the ring finger and pinky finger and rarely spreads so far as to affect the index finger and thumb too. The contracture can seize either of the two hands.
Initially it appears in the form of a triangular indentation on the palm or a nodule, and eventually creeps upwards with the result that the end fingers are permanently bent inwards or flexed so that the person cannot straighten them. The hand assumes the shape of a constant arch.
Unfortunately, the cause of the disease is not known. All that is understood is that people do have a genetic predisposition for it and the tendency for it is passed on through generations. It is also more prevalent in Northern European men. It sets in with age (almost always above 40) and is progressive, recurring and incurable.
Though there is no complete prevention or cure,treatments are available, both surgical and non-surgical. In the surgery, the hard tissue is cut out from the palm, letting the fingers relax and straighten again. But it has a risk of developing again because surgery is not acure. Non-surgical treatments include physiotherapy like massages and hand exercises that can delay its onset or keep it from progressing into a full contracture.
Is Dupuytren’s Contracture an autoimmune disease?
Research is yet to confirm the cause of this disease and whether it is an autoimmune disorder. However, the majoritydo postulate that it is.
There are two major reasons for this assumption:
We saw above that autoimmune diseases show similar features and they are also suggested to have a strong association with genetics. The real cause for Dupuytren’s contracture is unknown (just like autoimmune diseases), but the mathematical analysis of their prevalence patterns and their genetic origin strongly relates it to autoimmune disorders.
Moreover, according to theMayo Clinic, people with diabetes, which is also an autoimmune disorder, have an increased risk of contracting Dupuytren’s. Now this is similar to autoimmune disease patterns as they are always more likely to occur in people with history of another autoimmune disease.
About half of the people who suffer from a Dupuytren’s contracture also suffer from a “frozen shoulder”. The cause of frozen shoulder is also a mystery, but is again commonly thought to be caused by an autoimmune reaction.
The same source explains that the disease is probably initiated by a series of gene mutations in a stem cell of thelymphatic system.
(The lymphatic system is a part of the body’s immune system. It is a network of vessels, lymph nodes, and other organs like the spleen and thymus and is responsible for producing and transporting a fluid called “lymph” which contains disease-fighting white blood cells.)
The mutations lead to the birth of “forbidden” lymphocytes which probably attack healthy tissue and cause the proliferation of fibroblasts. (Dupuytren’s is also called PalmarFibromatosis.)
Anotherresearch shows a positive correlation between certain human leukocyte antigen (HLA) alleles and Dupuytren’s disease. Studies have suggested the involvement of T and B lymphocytes in the origin of the disease, such that it has even been called a “T-cell mediated autoimmune disorder”. Some experiments claim to find high levels of T-cell saturation in the fibrous tissue affected by Dupuytren’s compared to healthy tissue, while others find higher levels of antibodies in affected tissues including IgM, IgA and collagen autoantibodies. All these suggest an autoimmune reaction.
There have also been studies that have shown no significant correlation between some components of the immune system and Dupuytren’s, even though they did show correlation with other fibrotic diseases.
For example, in onestudy, the link between eosinophils (little chemical sacs which are part of the immune system) and Dupuytren’s contracture was investigated. Although strong evidence of abnormal eosinophil levels was found in other fibrotic disorders like endomyocardial fibrosis and retroperitoneal fibrosis, no link was found with Dupuytren’s.
In short, although there appears to besome immune component in the pathology of Dupuytren’s contracture, evidence is not outstanding and differs from theusual causation mechanism of autoimmune diseases.
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