Massive interstitial fibrosis precipitated by pneumonia in a woman with mixed connective tissue disease

The interstitial involvement of lungs during systemic connective tissue diseases is well known: most of connective tissue diseases can evolve, at various degree, into diffuse interstitial fibrosis. The variable clinical presentations of these diseases can lead to underestimate the severity of lung involvement. We describe the case of a 64-year-old woman with ascertained but paucisymptomatic mixed connective tissue disease. Although no sign of previous pulmonary involvement was present, the onset of pneumonia rapidly precipitated the respiratory function, leading to ARDS and subsequent death. The autopsy disclosed a massive and severe interstitial fibrosis, with superimposed pneumonia on resolution. We suggest that patients suffering from systemic connective tissue diseases should be carefully ynonitored for lung involvement.