- On the spectrum of lymphoproliferative disorders.
- Associated with autoimmune diseases (Sjogren most common) and HIV (especially paediatric HIV)
- Broad spectrum of disease, with some patients recovering completely, while others progress to respiratory failure and interstitial fibrosis.
- Increased risk of lymphoma (5% prevalence).
- Five-year mortality is 33% to 50%
- Female predilection, peak age 50-60
- Commonly have indolent course, but can also be rapid.
- Radiographic features:
– CXR: basilar reticular pattern
I) basal distribution of thin walled cysts (cysts tend to remain even after treatment)
II) GGO & centrilobular nodules (will resolve after treatment)
- Tx: immunosuppression or HAART if HIV