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[A case of amyopathic dermatomyositis with rapidly progressive interstitial pneumonia]
Y Nanke1, M Tateisi, H Yamagata
1Department of Internal Medicine, National Murayama Hospital, Tokyo.
Abstract:
We report here a case of interstitional pneumonia (IP) associated with amyopathic dermatomyositis (DM). In August, 1998, a 53-year-old Japanese man was admitted to our hospital because of fever, polyarthritis and erythematous heliotrope eruption and Gottron's sign without any symptom of myositis. Serum CK level and EMG were normal. Jo-1 antibody was negative. Chest computed tomography (CT) revealed IP at both lung base areas. The patient was diagnosed as amyopathic DM with IP. When IP rapidly progressed, methylprednisolone pulse therapy and oral high dose prednisolone were not effective. High-dose intravenous cyclophosphamide seemed to be transiently effective, but oral cyclosporine A was not effective. The patient was died of respiratory failure in October 1998. During the course, we measured serum levels of LDH, sIL-2 R, s-ICAM-1 and KL-6, KL-6 could be a sensitive parameter of IP activity.
Insights
This case study details a fatal instance of interstitial pneumonia (IP) linked to amyopathic dermatomyositis (DM). KL-6 levels showed promise as a sensitive marker for interstitial pneumonia activity in this patient.
Area of Science:
- Rheumatology
- Pulmonology
- Clinical Medicine
Background:
- Amyopathic dermatomyositis (DM) is an autoimmune condition characterized by skin manifestations without significant muscle weakness.
- Interstitial pneumonia (IP) can be a severe complication of DM, significantly impacting prognosis.
Observation:
- A 53-year-old male presented with fever, polyarthritis, and characteristic skin lesions (heliotrope eruption, Gottron's sign) but no myositis symptoms.
- Normal serum creatine kinase (CK) levels and electromyography (EMG) ruled out overt myositis.
- Chest CT revealed bilateral interstitial pneumonia at the lung bases.
Findings:
- The patient was diagnosed with amyopathic DM complicated by IP.
- Standard treatments including methylprednisolone and oral prednisolone were ineffective for the rapidly progressing IP.
- Cyclophosphamide showed transient benefit, while cyclosporine A was ineffective.
- The patient ultimately succumbed to respiratory failure.
Implications:
- This case highlights the critical association between amyopathic DM and interstitial pneumonia.
- KL-6 emerged as a potentially sensitive biomarker for monitoring IP activity in this context.
- The limited treatment response underscores the aggressive nature of IP in some amyopathic DM cases.