Lymphocytic Interstitial Pneumonia (LIP)

LIP (lymphocytic interstitial pneumonia) is a rare lung disease that is characterized by the presence of numerous cells called lymphocytes in the interstitial regions. There is a variable degree of scarring.

The cause of LIP is not known. LIP may occur in association with autoimmune diseases, most often Sjogren's syndrome. LIP occurs with a two-fold increase among women as compared to men aged 40 to 70 years. Shortness of breath and cough are common symptoms. Crackles are sometimes heard. The chest x-ray shows small linear opacities at the lung bases. The chest CT scan sometimes show cystic structures.

A lung biopsy for tissue is required for the diagnosis. Tissue is obtained from a video-assisted thoracoscopy (VAT) or from a small lung operation. This may be performed on a day-surgical basis if the individual is stable or in the hospital if the individual is severely ill.

Corticosteroid therapy is generally the treatment that is used. It is not clear whether the treatment is effective. In about one-third of individuals, the illness eventually disappears. There are chronic symptoms in others. One-third to one-half of individuals may die from this disorder within 5 years.

LIP References:

1. Teirstein AS, Rosen MJ. Lymphocytic interstitial pneumonia (LIP). Clin Chest Med. 1988 Sep; 9(3): 467-571. PMID: 3044682.

2. Swigris JJ, Berry GJ, Raffin TA, Kuschner WG. Lymphoid interstitial pneumonia (LIP). Chest. 2002 Dec; 122(6): 2150-2164. PMID: 12475860.