Hypercapnic respiratory failure in systemic sclerosis

Muthiah Pugazhenthi, Dianne Cooper, B. Sanjay Ratnakant, Arnold Postlethwaite, Laura Carbone

Research output: Contribution to journalArticlepeer-review

6 Scopus citations


Respiratory failure from causes exclusive of intrinsic lung disease is rare in systemic sclerosis. We report an unusual case of a young woman with diffuse systemic sclerosis who presented with proximal muscle weakness, dyspnea, weight loss, and nasal regurgitation. On physical examination, she had normal breath sounds but severe limitation of chest wall expansion. Pulmonary function tests (PFTs) were consistent with restrictive lung disease. Pulmonary pressures were normal on right heart catheterization and chest radiography and high-resolution computed tomography showed no evidence of intrinsic lung disease. Quadriceps muscle biopsy was consistent with type II atrophy, without any element of inflammatory myositis. After a meal, the patient aspirated, developed severe persistent hypercapnia, and required ventilatory support. Therapy with corticosteroids and intravenous gamma globulin failed to improve her condition, and the patient subsequently expired. Hypercapnic respiratory failure on the basis of chest wall involvement from systemic sclerosis may occur in the absence of intrinsic lung disease, and the prognosis, as in this case, may be grave.

Original languageEnglish (US)
Pages (from-to)43-46
Number of pages4
JournalJournal of Clinical Rheumatology
Issue number1
StatePublished - Feb 1 2003
Externally publishedYes


  • Hypercapnic respiratory failure
  • Systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology


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