**6. References**


was also suggested that CD4+CD25+ cells, forkhead/winged helix transcription factor (Fox P3)+, transforming growth factor+ and IL-10+ cells were reduced in peripheral blood of patients with DM (21). Actually, CD4+CD8+ cells were significantly distributed around vessels in the interstitial tissues of the muscle bundles in our patients with DM. These cells might be CD4+CD 28+ (null) cells and CD8+CD28+ (null) cell infiltration, as reported, and it is of interest that circulating CD4+CD28+ cells and CD8+CD28+ cells were significantly increased in seropositive individuals, responded to human cytomegalovirus antigen

As to treatment for myopathy of patients with pSjS and DM, adequate doses of NSAIDs and/or oral steroids were mainly used in corresponding to their clinical severities and these were considered to be effective. However, in addition to these drugs, the combination with immunosuppressive agents such as azathioprine, cyclosporine, mycophenolate or methotrexate should be used for the autoimmune diseases, if they were not clinically controlled. The biological agent, rituximab, and tacrolimus may offer additional benefit to some patients and emerging agents against T cells, B cells, transmigration or transduction

Myopathies are neuromuscular disorders exhibiting myalgia and muscular weakness due to dysfunction of muscle fibers which are frequently seen in autoimmune diseases. We here discussed about the non-inflammatory myopathy seen in patients with pSjS and the inflammatory myopathy, that is myositis, found in patients with DM is suggested to be Immunological dysfunction in pathogenesis. Although the mechanism of the myopathy is still unclear, it might be due to inflammatory cytokines released from CD4+CD 28+ (null) cells and

Ab, antibody; ALD, aldolase; ANA, anti-nuclear antibody; anti-SSA Ab, anti- Sjögren's syndrome A Ab; anti-SSB Ab, anti- Sjögren's syndrome B Ab; CK, creatine kinase; D-E junction, dermo-epidermal junction; DM, dermatomyositis; IL, interleukin; NSAID, nonsteroidal anti-inflammatory drug; pSjS, primary Sjögren's syndrome; PSL, prednisolone;

[1] NINDS Myopathy Information Page: What is myopathy?, National Institute of Health,

[2] Saito S, Togashi A, Kaneko F, Yamamoto T, Uchida T, Oyama N: Primary Sjögren's

[5] Pendarvis WT, Pillemer SR. Widespread pain and Sjögren's syndrome. J Rheumatol

[3] Koler RA, Montemarano A: Dermatomyositis, Am Farm Physician 2001; 64: 1565-1573. [4] Tishler M, Barak Y, Paran D, Yaron M. Sleep disturbances, fibromyalgia and primary

Sjögren's syndrome. Clin Exp Rheumatol 1997; 15: 71-74.

syndrome with myalgia mimicking dermatomyositis. J Dermatol 2010; 37: 837-839.

CD8+CD28+ (null) cells infiltrated around vessels in the muscles of patients with MD.

stimulation and correlated with disease duration (22).

molecules may be discussed as New treatments (23).

VAS, visual analog scale; WNL, within normal limit

USA, December 10, 2010.

2001; 28: 2657-2659.

**4. Conclusion** 

**5. Abbreviations** 

**6. References** 


**7** 

**Dermatomyositis** 

*University of Otago, Dunedin,* 

*Radiology Department, Dunedin Hospital,* 

*Southern District Health Board & Clinical Senior Lecturer,* 

**1.1** Dermatomyositis is an autoimmune inflammatory myopathy with diffuse nonsuppurative inflammation of the skin and striated muscles. {In polymyositis, skeletal muscles only are

**1.2** The disease is uncommon, occurs twice as often in female patients, and most present between 40 and 60 years of age, with a smaller peak between the ages of 5 and 15. There is a two-to-seven fold increase in the frequency of malignancy, especially in older patients in the adult age group (about 21% for dermatomyositis and 15% for polymyositis) (Hansell, et al.,

**1. Introduction** 

**2. Clinical features** 

**2.1 Nomenclature and classification** 

 Primary idiopathic polymyositis Primary idiopathic dermatomyositis Amyopathic dermatomyositis

Idiopathic inflammatory myopathies:

Juvenile (childhood) dermatomyositis

 Polymyositis Dermatomyositis

i. malignant disease

These different classifications are included:

Polymyositis/dermatomyositis associated with:

Polymyositis/dermatomyositis of childhood

**2.1.1 Polymyositis and dermatomyositis (Hansell, et al., 2005)** 

ii. other collagen vascular disease (overlap syndrome)

**2.1.2 Inflammatory disease of muscle (Resnick & Kransdorf, 2005)** 

involved.]

2005).

Fred van Gelderen

*New Zealand* 

