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Updated: Jul 19, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Hypercapnic respiratory failure in systemic sclerosis.
Muthiah Pugazhenthi1, Dianne Cooper, B Sanjay Ratnakant
1Department of Medicine, University of Tennessee, Memphis, Tennessee 38163, USA. mpugazhenthi@utmem.edu
Summary
Systemic sclerosis can cause rare respiratory failure due to chest wall issues, not intrinsic lung disease. This case highlights the grave prognosis of such hypercapnic respiratory failure.
Area of Science:
- Pulmonology
- Rheumatology
- Critical Care Medicine
Background:
- Respiratory failure is uncommon in systemic sclerosis, especially without intrinsic lung disease.
- Chest wall involvement can lead to respiratory compromise.
Purpose of the Study:
- To report an unusual case of hypercapnic respiratory failure secondary to chest wall dysfunction in diffuse systemic sclerosis.
- To emphasize the potential for severe outcomes in such cases.
Main Methods:
- Case report of a young woman with diffuse systemic sclerosis.
- Clinical presentation, physical examination, pulmonary function tests (PFTs), right heart catheterization, high-resolution computed tomography (HRCT), and muscle biopsy were performed.
- Patient required ventilatory support due to aspiration and persistent hypercapnia.
Main Results:
- The patient presented with proximal muscle weakness, dyspnea, and weight loss, with limited chest wall expansion but normal breath sounds.
- PFTs indicated restrictive lung disease, yet HRCT and catheterization showed no intrinsic lung disease.
- Despite treatment with corticosteroids and IVIG, the patient experienced aspiration, severe hypercapnia, and ultimately expired.
Conclusions:
- Hypercapnic respiratory failure can occur in systemic sclerosis due to chest wall involvement, even without intrinsic lung disease.
- This presentation carries a grave prognosis, as demonstrated by the fatal outcome in this case.
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