Clinicopathologic features of myositis patients with CD8-MHC-1 complex pathology

Neurology ◽  
2017 ◽  
Vol 89 (10) ◽  
pp. 1060-1068 ◽  
Author(s):  
Chiseko Ikenaga ◽  
Akatsuki Kubota ◽  
Masato Kadoya ◽  
Kenichiro Taira ◽  
Naohiro Uchio ◽  
...  
Keyword(s):  
Major Histocompatibility Complex ◽  
Inclusion Body Myositis ◽  
Muscle Fibers ◽  
Immunosuppressive Treatment ◽  
Complex Class ◽  
Clinicopathologic Features ◽  
Hepatitis C Virus Infection ◽  
Histocompatibility Complex ◽  
Histopathologic Finding ◽  
Class 1

Objective:To determine the clinical features of myositis patients with the histopathologic finding of CD8-positive T cells invading non-necrotic muscle fibers expressing major histocompatibility complex class 1 (CD8-MHC-1 complex), which is shared by polymyositis (PM) and inclusion body myositis (IBM), in relation to the p62 immunostaining pattern of muscle fibers.Methods:All 93 myositis patients with CD8-MHC-1 complex who were referred to our hospital from 1993 to 2015 were classified on the basis of the European Neuromuscular Center (ENMC) diagnostic criteria for IBM (Rose, 2013) or PM (Hoogendijk, 2004) and analyzed.Results:The 93 patients included were 17 patients with PM, 70 patients with IBM, and 6 patients who neither met the criteria for PM nor IBM in terms of muscle weakness distribution (unclassifiable group). For these PM, IBM, and unclassifiable patients, their mean ages at diagnosis were 63, 70, and 64 years; autoimmune disease was present in 7 (41%), 13 (19%), and 4 (67%); hepatitis C virus infection was detected in 0%, 13 (20%), and 2 (33%); and p62 was immunopositive in 0%, 66 (94%), and 2 (33%), respectively. Of the treated patients, 11 of 16 PM patients and 4 of 6 p62-immunonegative patients in the unclassifiable group showed responses to immunotherapy, whereas all 44 patients with IBM and 2 p62-immunopositive patients in the unclassifiable group were unresponsive to immunotherapy.Conclusions:CD8-MHC-1 complex is present in patients with PM, IBM, or unclassifiable group. The data may serve as an argument for a trial of immunosuppressive treatment in p62-immunonegative patients with unclassifiable myositis.

scholarly journals Overview on Diagnosis and Management of Polymyositis

2021 ◽  
pp. 445-451
Author(s):  
Mohammed Salah Hussein ◽  
Atyaf Khalid Almandeil ◽  
Sarah Shujaa Alsulami ◽  
Amaal Abdulrahman Alwayli ◽  
Amal Abdulrahman Alwayli ◽  
...  
Keyword(s):  
Cytotoxic T Lymphocytes ◽  
Skeletal Muscles ◽  
Inclusion Body Myositis ◽  
Autoimmune Disorder ◽  
Complex Class ◽  
Older Individuals ◽  
Cd8 Cells ◽  
Histocompatibility Complex ◽  
Class 1 ◽  
Residual Muscle Weakness

Polymyositis (PM) is an autoimmune disorder; result from abnormal activation of cytotoxic T lymphocytes (CD8 cells) and macrophages against muscular antigens as well as the strong extrafusal muscular expression of major histocompatibility complex class 1 causing damage to the endomysium of the skeletal muscles.  Polymyositis develops over the months as compared to inclusion body myositis (IBM), which is a slowly progressive chronic myopathy developing in older individuals over a period of months to years with more severe symptoms.  Many patients require treatment for many years. Polymyositis affects the distal musculature of the esophagus in the late stage of disease in up to 70% of the patients leading to the inability to swallow, as well as regurgitation problems that can cause aspiration pneumonia. The principal goals of therapy are to improve strength and improve physical functioning. Many patients require treatment for several years. The 5-year survival rate for treated patients is in the order of 95%. Up to one-third of PM patients may be left with some degree of residual muscle weakness.

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