Desquamative interstitial pneumonia as the initial manifestation of systemic sclerosis

J Clin Rheumatol. 2010 Sep;16(6):284-6. doi: 10.1097/RHU.0b013e3181eed86d.


Interstitial lung disease (ILD) is a frequent pulmonary complication of systemic sclerosis (SSc), and nonspecific interstitial pneumonia is the most commonly recognized pattern of lung injury in these patients. In this report, we describe a never-smoker female presenting with Raynaud phenomenon and ILD that demonstrated desquamative interstitial pneumonia (DIP) on surgical lung biopsy. After 8 months, she was diagnosed with pulmonary hypertension at which time clinical examinations and serologic findings established the diagnosis of SSc. This case report expands the spectrum of patterns of ILD seen in association with SSc to include DIP.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Biopsy
  • Female
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology
  • Lung / pathology
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / drug therapy
  • Lung Diseases, Interstitial / etiology*
  • Scleroderma, Systemic / complications*
  • Treatment Outcome


  • Adrenal Cortex Hormones