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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

Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases
|October 17, 2006
PubMed
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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.

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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.