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04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Interstitial lung diseases associated with amyopathic dermatomyositis
T Suda1, T Fujisawa, N Enomoto
1Second Division, Dept of Internal Medicine, and 2 Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan. suda@hama-med.ac.jp
The European Respiratory Journal
|July 14, 2006
Summary
Interstitial lung disease associated with amyopathic dermatomyositis (ILD-ADM) presents in two distinct forms: acute/subacute and chronic. The acute/subacute form shows poorer prognosis and higher mortality compared to the chronic form.
Area of Science:
- Pulmonology
- Rheumatology
- Clinical Medicine
Background:
- Interstitial lung disease (ILD) can be associated with amyopathic dermatomyositis (ADM).
- Understanding the distinct clinical patterns and outcomes of ILD in ADM is crucial for patient management.
Purpose of the Study:
- To elucidate the clinical characteristics and prognosis of patients with ILD associated with amyopathic dermatomyositis (ILD-ADM).
- To compare the clinical features, therapeutic response, and prognosis between acute/subacute and chronic forms of ILD-ADM.
Main Methods:
- Retrospective analysis of 14 consecutive patients with ILD-ADM.
- Classification of patients into acute/subacute and chronic ILD forms based on clinical presentation.
- Comparison of clinical data, high-resolution computed tomography (HRCT) findings, bronchoalveolar lavage (BAL) results, and histological data.
Main Results:
- Nine patients had acute/subacute ILD-ADM, and five had chronic ILD-ADM.
- Acute/subacute ILD-ADM exhibited lower arterial oxygen tension, more consolidation on HRCT, higher cell counts in BAL, and diffuse alveolar damage histologically.
- Acute/subacute ILD-ADM was therapy-resistant with a 67% mortality rate, contrasting with the chronic form's good response and 0% mortality.
Conclusions:
- ILD-ADM encompasses two distinct clinical forms: acute/subacute and chronic.
- These forms differ significantly in clinical presentation, pathological findings, therapeutic response, and prognosis.
- The acute/subacute form of ILD-ADM carries a substantially higher mortality risk.
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