Scleromyositis
   HOME

TheInfoList



OR:

Scleromyositis, is an autoimmune disease (a disease in which the
immune system The immune system is a network of biological processes that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to parasitic worms, as well as cancer cells and objects such as wood splinte ...
attacks the body). People with scleromyositis have symptoms of both
systemic scleroderma Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. There are two ...
and either
polymyositis Polymyositis (PM) is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis. Its name means "inflammation of many muscles" ('' poly-'' + '' myos-'' + '' -itis''). The inflam ...
or
dermatomyositis Dermatomyositis (DM) is a long-term inflammatory disorder which affects skin and the muscles. Its symptoms are generally a skin rash and worsening muscle weakness over time. These may occur suddenly or develop over months. Other symptoms may inc ...
, and is therefore considered an overlap syndrome. Although it is a rare disease, it is one of the more common overlap syndromes seen in scleroderma patients, together with MCTD and Antisynthetase syndrome. Autoantibodies often found in these patients are the anti-PM/Scl (anti- exosome) antibodies. The symptoms that are seen most often are typical symptoms of the individual autoimmune diseases and include
Raynaud's phenomenon Raynaud syndrome, also known as Raynaud's phenomenon, eponymously named after the physician Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862, is a medical condition in which the spasm of small arteries cau ...
, arthritis,
myositis Myositis is a rare disease that involves inflammation of the muscles. This can present with a variety of symptoms such as skin involvement (i.e., rashes), muscle weakness, and other organ involvement. Systemic symptoms such as weight loss, fatigue ...
and
scleroderma Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs, as well. Symptoms may include areas ...
. Treatment of these patients is therefore strongly dependent on the exact symptoms with which a patient reports to a physician and is similar to treatment for the individual autoimmune disease, often involving either
immunosuppressive Immunosuppression is a reduction of the activation or efficacy of the immune system. Some portions of the immune system itself have immunosuppressive effects on other parts of the immune system, and immunosuppression may occur as an adverse reacti ...
or immunomodulating drugs.


Signs and symptoms

Symptoms vary but they mostly involve skin disorders. The signs to look for include
Raynaud's phenomenon Raynaud syndrome, also known as Raynaud's phenomenon, eponymously named after the physician Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862, is a medical condition in which the spasm of small arteries cau ...
, arthritis,
myositis Myositis is a rare disease that involves inflammation of the muscles. This can present with a variety of symptoms such as skin involvement (i.e., rashes), muscle weakness, and other organ involvement. Systemic symptoms such as weight loss, fatigue ...
and
scleroderma Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs, as well. Symptoms may include areas ...
. Visual symptoms include discoloring of the skin and painful swelling.


Cause

* There is no distinct cause for scleromyositis. * Scleroderma can develop in every age group from infants to the elderly, but its onset is most frequent between the ages of 25 to 55. * In most cases it is observed that the disease involves an overproduction of collagen.


Diagnosis

Diagnosis is by skin tests. Typically, after a consultation with a rheumatologist, the disease will be diagnosed. A dermatologist is also another specialist that can diagnose. Blood studies and numerous other specialized tests depending upon which organs are affected.


Scleroderma overlap syndrome

People with scleroderma overlap syndrome have symptoms of both systemic scleroderma and/or
polymyositis Polymyositis (PM) is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis. Its name means "inflammation of many muscles" ('' poly-'' + '' myos-'' + '' -itis''). The inflam ...
and
dermatomyositis Dermatomyositis (DM) is a long-term inflammatory disorder which affects skin and the muscles. Its symptoms are generally a skin rash and worsening muscle weakness over time. These may occur suddenly or develop over months. Other symptoms may inc ...
: *
Scleroderma Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs, as well. Symptoms may include areas ...
: a group of rare diseases that involve the hardening and tightening of the skin and connective tissues. *
Polymyositis Polymyositis (PM) is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis. Its name means "inflammation of many muscles" ('' poly-'' + '' myos-'' + '' -itis''). The inflam ...
: a rare inflammatory disease that causes muscle weakness affecting both sides of your body. *
Dermatomyositis Dermatomyositis (DM) is a long-term inflammatory disorder which affects skin and the muscles. Its symptoms are generally a skin rash and worsening muscle weakness over time. These may occur suddenly or develop over months. Other symptoms may inc ...
: an inflammatory disease of skin and muscle marked especially by muscular weakness and skin rash. Scleroderma is a connective tissue disease that causes
fibrosis Fibrosis, also known as fibrotic scarring, is a pathological wound healing in which connective tissue replaces normal parenchymal tissue to the extent that it goes unchecked, leading to considerable tissue remodelling and the formation of perma ...
and vascular abnormalities, but that also has an autoimmune component, and can include connective tissues complications.


Treatment

There is no current cure. The only way to treat this disease is by treating symptoms. Commonly people are prescribed immunosuppressive drugs. Another route would be to take collagen regulation drugs.


Recent research

As of 2006 it is unclear which antibodies will best treat connective tissue diseases. One study from 2014 showed some potential of the synthetic drug
Rituximab Rituximab, sold under the brand name Rituxan among others, is a monoclonal antibody medication used to treat certain autoimmune diseases and types of cancer. It is used for non-Hodgkin lymphoma, chronic lymphocytic leukemia (in non-geriatric pa ...
in treating this class of overlap syndromes.


References


Further reading



{{Autoimmune diseases Autoimmune diseases Rheumatology