Lymphoid interstitial pneumonia: clinical features, associations and prognosis

• Published in: The European respiratory journal Vol. 28; no. 2; pp. 364 - 369
• Main Authors: Cha, S-I, Fessler, M. B, Cool, C. D, Schwarz, M. I, Brown, K. K
• Format: Journal Article
• Language: English
• Published: Leeds Eur Respiratory Soc 01.08.2006; Maney
• Subjects: Adolescent; Adult; Autoimmune Diseases - complications; Autoimmune Diseases - drug therapy; Autoimmune Diseases - mortality; Autoimmune Diseases - pathology; Biological and medical sciences; Bones, joints and connective tissue. Antiinflammatory agents; Female; Humans; Immune System Diseases - complications; Immune System Diseases - drug therapy; Immune System Diseases - mortality; Immune System Diseases - pathology; Lung Diseases, Interstitial - complications; Lung Diseases, Interstitial - drug therapy; Lung Diseases, Interstitial - mortality; Lung Diseases, Interstitial - pathology; Lymphocytosis - complications; Lymphocytosis - drug therapy; Lymphocytosis - mortality; Lymphocytosis - pathology; Male; Medical sciences; Middle Aged; Pharmacology. Drug treatments; Pneumology; Rare Diseases - drug therapy; Rare Diseases - mortality; Rare Diseases - pathology; Respiratory system : syndromes and miscellaneous diseases; Lung disease; Corticosteroid; Symptomatology; Association; Prognosis; Respiratory disease; lymphoid interstitial pneumonia; Interstitial pneumonitis; Survival
• ISSN: 0903-1936; 1399-3003
• DOI: 10.1183/09031936.06.00076705

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.