Lymphocytic interstitial pneumonia (LIP) is a syndrome secondary to autoimmune and other lymphoproliferative disorders.
Lymphocytic interstitial pneumonia (LIP) is a rare, benign lymphoproliferative disorder characterized by diffuse infiltration of the pulmonary interstitium with lymphocytes, plasma cells, and histiocytes.
It is often associated with autoimmune diseases, particularly Sjögren’s syndrome, and can also be seen in patients with HIV or other immunodeficiencies.
LIP is noted by a polyclonal lymphoid cell infiltrate surrounding airways and expanding the lung interstitium, often with the presence of germinal centers.
CXR may present with ground-glass opacities, centrilobular and subpleural nodules, and thin-walled cysts.
Clinically, patients typically present with symptoms such as progressive cough and dyspnea.
The disease course is variable, ranging from spontaneous resolution to progressive respiratory failure and death.
Approximately 33-50% of patients die within five years of diagnosis, and about 5% of cases may transform into lymphoma.
Symptoms of ILP include fever, cough, and shortness of breath.
LIP disorders ARE associated with both monoclonal or polyclonal gammopathy.
Patients with lymphocytic interstitial pneumonia may present with lymphadenopathy, hepatomegaly, splenomegaly, enlarged salivary gland, clubbing and breathing symptoms such as shortness of breath and wheezing.
Possible causes of lymphocytic interstitial pneumonia include the Epstein–Barr virus, Sjögren’s syndrome, auto-immune, and HIV.
Diagnosis requires lung biopsy.
Treatment
Patients who have no symptoms, and are not affected by the syndrome may not require treatment.
Corticosteroids have been reported to be of benefit in select patients., although the response is unpredictable.
Other immunosuppressive agents may be used, but there are no controlled trials.
Bronchodilators may assist with breathing issues.
Resolution may occur with the use of highly active anti-retroviral therapy used in patients with HIV, which suggests that the virus alone may cause immune cells to proliferate, or immunosuppression caused by HIV may lead to other viruses that induce the proliferation of immune cells, leading to LIP.