Rapid onset of lung involvement in progressive systemic sclerosis

Area of Science:

• Rheumatology
• Pulmonology
• Pathology

Background:

• Progressive systemic sclerosis (PSS) commonly affects the lungs, typically with chronic onset late in the disease course.
• Skin manifestations usually precede pulmonary findings in PSS.
• The typical presentation involves gradual progression of lung disease.

Observation:

• This report details a rare case of PSS presenting with acute, severe pulmonary interstitial fibrosis.
• Notably, this presentation occurred without preceding or concurrent skin changes.
• The rapid onset of lung disease in this patient was atypical for PSS.

Findings:

• Radiologic and pathologic findings confirmed extensive interstitial fibrosis in the lungs.
• The rapid progression of pulmonary involvement was the most striking feature.
• The absence of skin changes challenged the conventional understanding of PSS progression.

Implications:

• This case expands the spectrum of PSS presentations, particularly concerning its pulmonary manifestations.
• It suggests that PSS can manifest with aggressive lung disease as the initial prominent feature.
• Early recognition of such atypical presentations is crucial for timely intervention and management of pulmonary complications in PSS.