Lymphoid interstitial pneumonia

This code represents a rare, chronic inflammatory lung disease characterized by diffuse infiltration of the pulmonary interstitium by lymphocytes and plasma cells. It is a form of interstitial lung disease, often associated with autoimmune conditions or immunodeficiency. The condition leads to impaired gas exchange and progressive respiratory symptoms.

Assign this code when documentation explicitly states a diagnosis of lymphoid interstitial pneumonia (LIP). This diagnosis is typically confirmed by lung biopsy showing characteristic lymphocytic infiltrates. It is often seen in patients with HIV/AIDS, Sjögren's syndrome, or other connective tissue diseases.

• Do not confuse with other interstitial pneumonias; ensure the "lymphoid" specificity is documented.
• Always code any underlying associated conditions (e.g., HIV, Sjögren's syndrome) first, as LIP is often a manifestation of these.
• Look for pathology reports confirming lymphocytic infiltration of the lung parenchyma.