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5. Pulmonary sarcoidosis
Pulmonary cysts are associated with answers 1-4 but are not typically seen in sarcoidosis (5). Pulmonary sarcoidosis typically presents with mediastinal adenopathy and perilymphatic micronodules, although it can present with large scattered nodules (nummular sarcoid). Sarcoid rarely cavitates.
LIP is a rare interstitial lung disease that is often seen in association with Sjogren’s syndrome. Common pulmonary manifestations include lower-lobe predominant ground glass opacities and pulmonary cysts. Pulmonary nodular amyloidosis is a form of systemic amyloidosis where nodular collections of amyloid collect in the lungs. These nodules are of varying sizes and can calcify. Often there are cysts associated with these nodules (as in this case).
Amyloidosis is often a systemic process that can affect any organ system. The myocardial thickening seen on this patient’s CT could be a cardiac manifestation of amyloidosis. LAM typically affects women of childbearing age and has overlap with tuberous sclerosis. Classically, the patients develop innumerable pulmonary cysts of relatively uniform size and distribution. In pulmonary LCH the cysts are often interspersed with nodules and have an upper lung predilection with sparing of the costophrenic angles. LCH is often associated with cigarette smoking and usually improves with smoking cessation.
Biopsy of the patient’s pericardial fat pad demonstrated areas of classic apple-green birefringence with Congo red staining consistent with amyloid deposition.
Figure 3. Representative upper abdominal CT image.
Based on the above image (Figure 3), classify the type of amyloidosis in this patient. (Click on the correct answer to proceed to the next panel)