Lymphoid interstitial pneumonia: clinical features, associations, and prognosis

¹ Kyungpook National University Hospital, Korea

^* To whom correspondence should be addressed. E-mail: brownk{at}njc.org.

	Abstract

Lymphoid interstitial pneumonia (LIP) is rare, and its clinical course incompletely described. The aim of this study was to examine the clinical features, associations, and prognosis of surgical lung biopsy-proven LIP.

The study group consisted of 15 subjects encountered over a 14-year period.

The majority of subjects were women (n=11), and the mean age was 47 years (17 to 78 years). Underlying systemic immune disorders were frequent, including Sjögren's syndrome (n=8), rheumatoid arthritis, systemic lupus erythematosus, polymyositis, common variable immunodeficiency, and dysproteinemia. Only three patients were classified as "idiopathic". Presenting symptoms were dominated by dyspnoea and cough. Restrictive physiology, a reduced diffusing capacity (62.5±18.4% predicted), and bronchoalveolar lavage lymphocytosis (30.5±29.1%) were noted. Thirteen patients received corticosteroid therapy. Of the nine whose response could be assessed, four clinically improved and four were stable. Overall median survival was 11.5 years. Of the seven patients who died, the primary cause of death was respiratory in three. Conversion to lymphoma was not identified.

In conclusion, histopathologic LIP is commonly associated with immune system dysregulation, with idiopathic LIP being extremely rare. Clinical stability or improvement with corticosteroids can be expected, however survival remains impaired.

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