PURPOSE: Analysis of acute exacerbations (AEs) of usual interstitial pneumonia (UIP).
METHODS: Retrospective analysis of clinical profiles, laboratory parameters and outcomes of patients with acute exacerbations of chronic interstitial lung disease seen at our center over a 3-year period.
RESULTS: Seven patients met the criteria for acute exacerbations of chronic ILD. All patient had underlying UIP, had acute worsening of dyspnea, and developed new ground-glass opacities without specific evidence for infection. Patients were Caucasian with male to female ratio of 4:3. All but one were non-smokers. In two patients, AEs were the initial presentation of underlying IPF. In six patients, the diagnosis of UIP was made by video-assisted thoracoscopic (VATS) biopsy; in two of these patients, VATS biopsy was done during their AEs and both these patients died being unable to be weaned off mechanical ventilatory support. In the other five patients, two patients died within a few months of their AEs, one required lung transplantation and two improved with steroids. Response of AEs to high-dose steroids was variable and not dependent on severity of underlying UIP. In two patients, ground-glass opacities on HRCT involved one side predominantly. Four patients had laboratory abnormalities that suggested autoimmune phenomena (elevated rheumatoid factor in 2, elevated antinuclear antibodies in 3, increased Scl-70 in 1) but exhibited no other features of a connective tissue disorder. In none of these patients did the underlying UIP respond to steroids or cytotoxic agents.
CONCLUSION: Outcomes from acute exacerbations of UIP are poor. Abnormalities in laboratory autoimmune parameters in patients with UIP may indicate a covert connective tissue disorder; however the lack of other features of a connective tissue disorder and failure to respond to immunosuppressive agents makes these autoimmune phenomena being associated with IPF rather than a specific autoimmune disorder.
CLINICAL IMPLICATIONS: Surgical biopsy during UIP exacerbations may be associated with increased mortality. Whether autoimmune abnormalities on laboratory testing in patients with IPF makes these patients prone to AEs remains to be proven.
DISCLOSURE: Krishna Sundar, None.