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Other interstitial pneumonitis / fibrosis

Lymphoid interstitial pneumonia (LIP)

Reviewers: Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 25 September 2011, last major update September 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.


● 1/3 associated with Sjogren’s syndrome; also associated with AIDS, pulmonary drug reactions
● May occur in children with AIDS
● May be an IgG4 related disease
● May be EBV related, particularly in non-HIV related cases
● Symptoms: shortness of breath, cough and polyclonal gammopathy
● Often progressive, long standing cases may culminate in lymphoma


● Poor response to steroids or chemotherapy

Micro description

● Lymphocytes with germinal centers, plasma cells, macrophages and epithelioid granulomas in lung interstitium
● No effacement of alveolar architecture
● No invasion of parietal pleura, although visceral pleura may have mild inflammation
● Late - diffuse interstitial fibrosis

Molecular description

● Polyclonal

Differential diagnosis

● Follicular bronchitis and bronchiolitis
● Non-specific inflammatory reaction

End of Lung-nontumor > Other interstitial pneumonitis / fibrosis > Lymphoid interstitial pneumonia (LIP)

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