Authors: Kameda, Hideto; Takeuchi, Tsutomu

Source: Endocrine, Metabolic & Immune Disorders - Drug Targets, Volume 6, Number 4, December 2006 , pp. 409-415(7)

Publisher: Bentham Science Publishers

Abstract:

Interstitial lung disease (ILD) develops in 30-50% of patients with polymyositis/dermatomyositis (PM/DM) and negatively affects their prognosis. The progression of PM/DM-ILD may be acute, subacute, chronic, or chronic becoming acute. The histopathological classification of PM/DM-ILD includes non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), diffuse alveolar damage (DAD), and usual interstitial pneumonia (UIP) or mixed variations. Some patients with acute/subacute interstitial pneumonia (A/SIP), typically with lung histology of OP or cellular NSIP, respond favorably to corticosteroid treatment, while others do not. Japanese patients with DM, especially those with clinically amyopathic DM (C-ADM) and palmar papules, seem to be at a greater risk of developing fulminant A/SIP with DAD histology resulting in pneumomediastinum and fatal outcome in a few months. An aggressive combination regimen including cyclosporine A (or tacrolimus) and cyclophosphamide should be immediately added to corticosteroid treatment for such patients. Sequential follow-up examination using high-resolution computed tomography (HRCT) of the chest and careful monitoring for bacterial and viral infections are essential. However, intensive immunosuppression alone may not be sufficient to control fulminant A/SIP, and other therapeutic targets, such as fibroblasts, should be considered.

Keywords: Clinically amyopathic dermatomyositis; diffuse alveolar damage; cyclosporine A; cyclophosphamide

Document Type: Research article

DOI: http://dx.doi.org/10.2174/187153006779025775

Affiliations: 1: Division of Rheumatology/Clinical Immunology, Department of Internal Medicine, Saitama Medical Center, Saitama Medical University, 1981 Tsujido-machi, Kamoda, Kawagoe, Saitama 350-8550, Japan.

Publication date: 2006-12-01

More about this publication?

• This journal is devoted to timely reviews of experimental and clinical studies in the field of endocrine, metabolic, and immune disorders. Specific emphasis is placed on humoral and cellular targets for natural, synthetic, and genetically engineered drugs that enhance or impair endocrine, metabolic, and immune parameters and functions. Topics related to the neuroendocrine-immune axis are given special emphasis in view of the growing interest in stress-related, inflammatory, autoimmune, and degenerative disorders.

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