**4.3 Cardiac abnormalities**

**4.3.1** The myocardium may be affected in a similar way to the involvement of skeletal striated muscle. Cardiac complications include arrhythmias, congestive cardiac failure, and pericarditis (O'Brien & Kelleher, 2008).

### **4.4 Gastrointestinal abnormalities**

**4.4.1** Patients may complain of dysphagia and a barium swallow may reveal disordered peristalsis involving the upper oesophagus, that portion with striated muscle. [The lower

**8** 

*Japan* 

*Osaka Medical College* 

**Interstitial Pneumonia in Dermatomyositis** 

Dermatomyositis (DM) and polymyositis (PM) are types of autoimmune inflammatory muscle disease that mainly damage proximal limb muscles, with DM involving characteristic skin findings such as Gottron's sign and heliotrope eruption (Bohan & Peter, 1975a, 1975b). Interstitial pneumonia (IP) is often associated with DM/PM and is one of the important prognostic factors. Above all, rapidly progressive IP (RPIP), which has the worst prognosis, is resistant to corticosteroid drugs and is strongly associated with clinical amyopathic DM (CADM), which is unlikely to show myositis (Kameda & Takeuchi, 2006). In response, combination therapies of corticosteroid drugs and immunosuppressive drugs have recently been administered early in the onset of IP, and outcomes have been improved. Here, we review the pathogenesis, the clinical and laboratory findings, and treatment of IP

IP occurs in association with DM/PM in 40-50% of patients and is an important prognostic factor of DM/PM (Hidano, 1992, Marie, 2002, Fathi, 2004). In 2002, the American Thoracic Society and the European Respiratory Society jointly advocated classifying idiopathic IP into 7 types (American Thoracic Society & European Respiratory Society, 2002), which are applied to IP associated with connective tissue disease. Many cases of IP associated with DM/PM comprise one of 3 types: nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), or diffuse alveolar damage (DAD) (Douglas, 2001, Fujisawa, 2005). Prognoses vary according to these types: cases of DAD are the most critical, but some cases of NSIP and COP also progress to unfortunate outcomes. IP is classified according to clinical course into 1 of 3 groups: acute/subacute IP (A/SIP) with rapidly progression over several weeks or months, chronic IP (CIP) with slowly progression over years, and asymptomatic type with only minor abnormalities on chest CT or in respiratory function

The histological features of muscle biopsies in DM and PM are different, as are the pathological features of the IP associated with each disease. PM mainly is associated with slowly progressive CIP, which histologically presents as fibrotic NSIP and which responds to corticosteroids. A/SIP also is associated with COP or cellular NSIP and shows good response to corticosteroids in many cases. In contrast, almost 50% of DM cases are associated with A/SIP, which histologically presents as fibrotic NSIP or DAD (Hirakata &

**1. Introduction** 

associated with DM/PM.

tests (Frazier & Miller, 1974).

**2. IP Associated with DM/PM** 

Tohru Takeuchi, Takuya Kotani and Shigeki Makino

oesophagus has smooth muscle, and is more likely to be affected by scleroderma.] Because of the progressive weakness of the proximal striated oesophageal muscle, there tends to be atony and dilatation. Aspiration into the tracheobronchial tree may occur during barium swallow examination (Fig. 3a). Atony of the small intestine and colon may furthermore be a feature. It should be emphasized that involvement of the gastrointestinal is not a common manifestation of dermatomyositis.

#### **5. Conclusion**

**5.1** Some of the protean radiological manifestations of dermatomyositis in childhood and adults are illustrated, with the aid of various radiological examinations.

The superficial soft tissue changes may be very extensive and florid. In recent years MRI has become more important to demonstrate activity of disease.

The effect of treatment, for example, with corticosteroids, can be monitored by four-station STIR MRI. The very extensive disease with deformities and contractures, seen previously, are now less common as a consequence of more optimal management.

#### **6. References**

