Polymyositis is one of a group of diseases termed idiopathic inflammatory myopathies. It is a rare condition with an incidence that is estimated to be from fewer than one to eight cases per million (Ruddy et al., 2001)
Pathophysiology
Polymyositis is classified as autoimmune because autoantibodies are present. However, these antibodies do not cause damage to muscle cells, indicating only an indirect role in tissue damage. The pathogenesis is multifactorial, and a genetic predisposition is likely. Drug-induced disease is rare. Some evidence suggests a viral link.
Signs and symptoms
Symptoms
involve pain, with stained weakness and/or loss of muscle accumulation in the proximal musculature, especially in the shoulder and pelvic girdle. The hip extensors are often sternly influenced, guiding to particular intricacy in ascending stairs and getting up from a seated position. Coagulating of the skin on the fingers and hands (sclerodactyly) is a common characteristic; however, this is non-specific and appears in other autoimmune connective tissue ailments.
Dysphagia (trouble in swallowing) and/or other features of esophageal dysmotility take place in as many as 1/3 of patients. Low grade fever and peripheral adenopathy may be there. Foot drop in one or both feet may be a symptom of progressed polymyositis and inclusion body myositis. Polymyositis is also related with interstitial lung issues.
Polymyositis is connected to enlarge in the incidence of certain cancers especially non-Hodgkin lymphoma, bladder and lung cancers, but the overall involvement is weaker than in the associated condition dermatomyositis. The latter is connected to a significant increase in the risk of a range of malignancies including pancreatic, lung, and ovarian cancer.
Polymyositis tends to become marked in adulthood, representing with bilateral proximal muscle weakness repeatedly distinguished in the upper legs because of early fatigue while walking. Every so often the weakness display itself as an incapability to rise from a seated position without help or incapacity to raise the person’s arm above their head. The weakness is usually advanced, attended by lymphocytic inflammation.
Polymyositis, similar to dermatomyositis, appears in females with larger frequency in comparison with males. The skin participation of dermatomyositis is missing in polymyositis.
Causes of polymyositis-The origin of polymyositis is unidentified and may be multifactorial, maybe correlated to autoimmune features, genetics, and viruses. In exceptional cases, the reason is known to be infectious, related with the pathogens that cause Lyme disease, toxoplasmosis, and other infectious causes. Polymyositis generally is believed non-fatal in the absence of ILD.
It is assumed that an early injury leads to release of muscle auto antigen, which is consequently taken up by macrophages and offered to CD+4 TH cells. Triggered TH cells synthesize IFN-γ that excites added macrophages and inflammatory mediator discharge like IL-1 and TNF-α.
Another significant event in the pathogenesis of polymyositis is the augmented expression of MHC proteins by m/s cells. Auto-Ag is offered in relationship with MHC-I molecules and is accepted by CD8 cytotoxic T cells that consequently commence m/s devastation.
Diagnosis of polymyositis-diagnosis of polymyositis is multiple, including increase of creatine kinase, history and physical inspection, electromyograph (EMG) modification, and a helpful muscle biopsy. Sporadic inclusion body myositis (sIBM): This is often mistaken for polymyositis and dermatomyositis that does not react to treatment is probable IBM.
As IBM progresses on over months to years, polymyositis arrives on over weeks to months. It shows that sIBM and polymyositis communicate familiar features, particularly the preliminary sequence of immune system commencement, however, polymyositis does not show the following muscle deterioration and protein irregularities as observed in IBM. Polymyositis tends to react well to treatments, IBM does not. IBM and polymyositis actually involve different disorder mechanisms that are observed in dermatomyositis.
Laboratory findings may show presence of Anti Jo antibodies in over 65% patients. Raised serum creatine kinase is feature, but not particular to polymyositis.
Treatment for polymyositis-Characteristically, high-dose steroids are the treatment of option. Typically, muscle strength will perk up within four to six weeks. Indifferent patients may be evaluated other immunosuppressive medicines. IVIG has also revealed to be a helpful treatment. Specialized exercise therapy may enhancement treatment to improve quality of life.
It is advisable to consult a doctor if any of the above polymyositis symptoms occurs for early diagnosis and subsequent treatment.
Pathophysiology
Polymyositis is classified as autoimmune because autoantibodies are present. However, these antibodies do not cause damage to muscle cells, indicating only an indirect role in tissue damage. The pathogenesis is multifactorial, and a genetic predisposition is likely. Drug-induced disease is rare. Some evidence suggests a viral link.
Signs and symptoms
Symptoms
involve pain, with stained weakness and/or loss of muscle accumulation in the proximal musculature, especially in the shoulder and pelvic girdle. The hip extensors are often sternly influenced, guiding to particular intricacy in ascending stairs and getting up from a seated position. Coagulating of the skin on the fingers and hands (sclerodactyly) is a common characteristic; however, this is non-specific and appears in other autoimmune connective tissue ailments.
Dysphagia (trouble in swallowing) and/or other features of esophageal dysmotility take place in as many as 1/3 of patients. Low grade fever and peripheral adenopathy may be there. Foot drop in one or both feet may be a symptom of progressed polymyositis and inclusion body myositis. Polymyositis is also related with interstitial lung issues.
Polymyositis is connected to enlarge in the incidence of certain cancers especially non-Hodgkin lymphoma, bladder and lung cancers, but the overall involvement is weaker than in the associated condition dermatomyositis. The latter is connected to a significant increase in the risk of a range of malignancies including pancreatic, lung, and ovarian cancer.
Polymyositis tends to become marked in adulthood, representing with bilateral proximal muscle weakness repeatedly distinguished in the upper legs because of early fatigue while walking. Every so often the weakness display itself as an incapability to rise from a seated position without help or incapacity to raise the person’s arm above their head. The weakness is usually advanced, attended by lymphocytic inflammation.
Polymyositis, similar to dermatomyositis, appears in females with larger frequency in comparison with males. The skin participation of dermatomyositis is missing in polymyositis.
Causes of polymyositis-The origin of polymyositis is unidentified and may be multifactorial, maybe correlated to autoimmune features, genetics, and viruses. In exceptional cases, the reason is known to be infectious, related with the pathogens that cause Lyme disease, toxoplasmosis, and other infectious causes. Polymyositis generally is believed non-fatal in the absence of ILD.
It is assumed that an early injury leads to release of muscle auto antigen, which is consequently taken up by macrophages and offered to CD+4 TH cells. Triggered TH cells synthesize IFN-γ that excites added macrophages and inflammatory mediator discharge like IL-1 and TNF-α.
Another significant event in the pathogenesis of polymyositis is the augmented expression of MHC proteins by m/s cells. Auto-Ag is offered in relationship with MHC-I molecules and is accepted by CD8 cytotoxic T cells that consequently commence m/s devastation.
Diagnosis of polymyositis-diagnosis of polymyositis is multiple, including increase of creatine kinase, history and physical inspection, electromyograph (EMG) modification, and a helpful muscle biopsy. Sporadic inclusion body myositis (sIBM): This is often mistaken for polymyositis and dermatomyositis that does not react to treatment is probable IBM.
As IBM progresses on over months to years, polymyositis arrives on over weeks to months. It shows that sIBM and polymyositis communicate familiar features, particularly the preliminary sequence of immune system commencement, however, polymyositis does not show the following muscle deterioration and protein irregularities as observed in IBM. Polymyositis tends to react well to treatments, IBM does not. IBM and polymyositis actually involve different disorder mechanisms that are observed in dermatomyositis.
Laboratory findings may show presence of Anti Jo antibodies in over 65% patients. Raised serum creatine kinase is feature, but not particular to polymyositis.
Treatment for polymyositis-Characteristically, high-dose steroids are the treatment of option. Typically, muscle strength will perk up within four to six weeks. Indifferent patients may be evaluated other immunosuppressive medicines. IVIG has also revealed to be a helpful treatment. Specialized exercise therapy may enhancement treatment to improve quality of life.
It is advisable to consult a doctor if any of the above polymyositis symptoms occurs for early diagnosis and subsequent treatment.
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Unknown - Monday 11 April 2011
