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Updated: Jul 5, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Erdheim-Chester disease: pulmonary presentation in a case with advanced systemic involvement
Charalambos Protopapadakis1, Katerina M Antoniou, Andrew G Nicholson
1Departments of Thoracic Medicine and Radiology, Medical School, University of Crete, Heraklion, Greece Department of Pathology, Royal Brompton Hospital, London, UK.
Summary
Erdheim-Chester disease (ECD), a rare histiocytosis, can aggressively affect the lungs, leading to poor prognosis. Early diagnosis is crucial but challenging, especially with pulmonary symptoms masking systemic involvement.
Area of Science:
- Rare diseases
- Pulmonary medicine
- Histiocytosis
Background:
- Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis.
- It typically affects bone but can involve multiple organs.
- Pulmonary involvement signifies a poor prognosis.
Observation:
- A 48-year-old man presented with progressive dyspnea and cough.
- High-resolution CT revealed interstitial lung thickening.
- Initial biopsies were non-diagnostic, necessitating surgical lung biopsy.
Findings:
- Surgical lung biopsy confirmed Erdheim-Chester disease.
- Systemic involvement included bone, heart (left heart failure), and renal/perirenal disease.
- Treatment with corticosteroids and cyclophosphamide was ineffective, leading to death within 6 months.
Implications:
- Pulmonary ECD presentation can be aggressive and mimic malignancy.
- Diagnostic challenges arise with pulmonary manifestations and asymptomatic systemic disease.
- This case underscores the critical need for timely diagnosis and effective therapeutic strategies for advanced ECD.
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