lymphoid interstitial pneumonia LIP etiologies are typically autoimmune

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LIP occurs in middle-aged women (incidence is twice that of men) with underlying autoimmune disease or immunodeficiency, most commonly Sjögren syndrome.

LIP is relatively rare and is seen in both adults and children. LIP is one of the idiopathic interstitial pneumonias and is within a spectrum of pulmonary lymphoproliferative disorders. Others include follicular bronchiolitis (thought to be closely related to LIP), pseudolymphoma, primary pulmonary lymphoma, lymphomatoid granulomatosis, bronchocentric granulomatosis, and IgG4-related disease. Granulomatous and lymphocytic interstitial lung disease (ILD), described in patients with common variable immunodeficiency (CVID), may be a form of LIP, which may be related to human herpesvirus type 8.

Although the cause of LIP is unknown, one of the more commonly identified associations with LIP in adults is Sjögren disease, and with Sjögren disease, the pulmonary manifestations of LIP may precede or follow the disease. Fifteen percent of ILD in patients with Sjögren disease is LIP. Other common associations with LIP in adults include rheumatoid arthritis, systemic lupus erythematosus, and hypogammaglobulinemia. Less common associations include celiac sprue, myasthenia gravis, pernicious anemia, chronic active hepatitis, and biliary cirrhosis. In children, there is a strong association with viral infection such as HIV.1

Footnotes

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