Presentation
Fever, malaise and generalized weakness in a patient with Sjögren's Syndrome. On mycophenolate and prednisone for membranous glomerulonephritis.
Patient Data
Multiple bilateral rounded, thin-walled perivascular cysts with lower lobe predominance. A right basal cyst contains internal tissue strands.
Lungs otherwise clear. No nodules or opacities.
No lymphadenopathy or effusions.
Case Discussion
Lymphocytic interstitial pneumonia (LIP) is a benign lymphoproliferative disorder characterized by alveolar septal infiltration of polyclonal lymphocytes, plasma cells and histiocytes. Sjogren's syndrome accounts for about 25% of cases. Prior to the introduction of antiretroviral agents LIP was an uncommon complication in AIDS patients 1.
The main radiological findings are bilateral thin-walled perivascular cysts with lower lobe predominance and round margins 2. Cysts sometimes occur in areas of ground glass opacity and often have internal septa. Nodules may also be present.
In our case, the history of Sjögren's syndrome associated with the characteristic radiological findings make the diagnosis of lymphoid interstitial pneumonia highly probable. Pulmonary infection was ruled out. Typical presenting symptoms of LIP are fever, cough, dyspnea and weight loss.
An incidental gastrointestinal infection was diagnosed on clinical grounds and laboratory test results.