Scleromyositis
Encyclopedia
Scleromyositis or the PM/Scl overlap syndrome is a complex autoimmune disease
(a disease in which the immune system
attacks the body). Patients with scleromyositis have symptoms of both systemic scleroderma and either polymyositis
or dermatomyositis
, 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 synthetase 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
, arthritis
, myositis
and scleroderma
. 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
or immunomodulating drugs.
Autoimmune disease
Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. In other words, the body actually attacks its own cells. The immune system mistakes some part of the body as a pathogen and attacks it. This may be restricted to...
(a disease in which the immune system
Immune system
An immune system is a system of biological structures and processes within an organism that protects against disease by identifying and killing pathogens and tumor cells. It detects a wide variety of agents, from viruses to parasitic worms, and needs to distinguish them from the organism's own...
attacks the body). Patients with scleromyositis have symptoms of both systemic scleroderma and either polymyositis
Polymyositis
Polymyositis is a type of chronic inflammation of the muscles related to dermatomyositis and inclusion body myositis.-Signs and symptoms:...
or dermatomyositis
Dermatomyositis
Dermatomyositis is a connective-tissue disease related to polymyositis and Bramaticosis that is characterized by inflammation of the muscles and the skin.- Causes :...
, and is therefore considered an overlap syndrome
Overlap syndrome
An overlap syndrome is an autoimmune disease of connective tissue in which the patient presents with symptoms of two or more diseases. As much as 25% of all patients with connective tissue disease show signs of 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 synthetase syndrome. Autoantibodies often found in these patients are the anti-PM/Scl (anti-exosome
Exosome complex
The exosome complex is a multi-protein complex capable of degrading various types of RNA molecules...
) antibodies.
The symptoms that are seen most often are typical symptoms of the individual autoimmune diseases and include Raynaud's phenomenon
Raynaud's phenomenon
In medicine, Raynaud's phenomenon is a vasospastic disorder causing discoloration of the fingers, toes, and occasionally other areas. This condition can also cause nails to become brittle with longitudinal ridges. Named for French physician Maurice Raynaud , the phenomenon is believed to be the...
, arthritis
Arthritis
Arthritis is a form of joint disorder that involves inflammation of one or more joints....
, myositis
Myositis
Myositis is a general term for inflammation of the muscles. Many such conditions are considered likely to be caused by autoimmune conditions, rather than directly due to infection It is also a documented side effect of the lipid-lowering drugs statins and fibrates.Elevation of creatine kinase in...
and scleroderma
Scleroderma
Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...
. 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
Immunosuppressive drug
Immunosuppressive drugs or immunosuppressive agents are drugs that inhibit or prevent activity of the immune system. They are used in immunosuppressive therapy to:...
or immunomodulating drugs.