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Lymphoid interstitial pneumonia: clinical features, associations and prognosis

¹ Dept of Internal Medicine, Kyungpook National University Hospital, Daegu, South Korea. ² Dept of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, and ³ Depts of Pathology and Medicine, University of Colorado Health Sciences University, National Jewish Medical and Research Center, Denver, CO, USA.

CORRESPONDENCE: K. K. Brown, ILD Program, National Jewish Medical and Research Center, 1400 Jackson Street, Denver, CO 80206, USA. Fax: 1 3032702240. E-mail: brownk{at}njc.org

Keywords: Corticosteroid, lymphoid interstitial pneumonia, survival

Received: June 30, 2005
Accepted March 13, 2006

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-yr period.

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

In conclusion, histopathological lymphoid interstitial pneumonia is commonly associated with immune system dysregulation, with idiopathic lymphoid interstitial pneumonia being extremely rare. Clinical stability or improvement with corticosteroids can be expected; however, survival remains impaired.

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