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[Interstitial pneumonia complicating amyopathic dermatomyositis: a case report]
S K Jamoussi1, B B Dhaou, F Boussema
1Service de médecine interne, hôpital Habib-Thameur, 8, rue Ali-Ben-Ayed, Montfleury, Tunis 1008, Tunisie.
Revue De Pneumologie Clinique
|December 10, 2009
Summary
Amyopathic dermatomyositis (ADM) with interstitial pneumonia presents a severe challenge, often progressing rapidly. This case highlights a fatal outcome despite aggressive treatment, underscoring the critical need for early diagnosis and novel therapies.
Area of Science:
- Rheumatology
- Pulmonology
- Dermatology
Background:
- Amyopathic dermatomyositis (ADM) is a subset of dermatomyositis lacking muscle weakness.
- ADM can be associated with rapidly progressive interstitial pneumonia or neoplasm, both linked to poor prognosis.
Observation:
- A 56-year-old woman presented with arthralgia, weight loss, dyspnea, cough, and photosensitivity.
- Physical exam revealed Gottron sign and heliotropic rash, but no muscle weakness. Chest auscultation noted fine crackles.
- Laboratory results showed lymphopenia; creatine kinase and lactate dehydrogenase were normal. Autoantibodies were negative except for a weakly positive antinuclear antibody.
Findings:
- High-resolution computed tomographic chest scans revealed diffuse ground-glass opacities with basilar predominance.
- Diagnosis was ADM complicated by rapidly progressive interstitial pneumonia.
- Despite treatment with IV methylprednisolone and cyclophosphamide, the patient succumbed to respiratory failure.
Implications:
- This case underscores the severe prognosis of ADM-associated interstitial pneumonia.
- It highlights the diagnostic challenges in ADM when muscle involvement is absent.
- Further research into early detection and effective treatments for ADM-related lung disease is crucial.
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