1. A 35-year-old female with previous history of renal embolisation. CT thorax was done showing diffuse regular cysts involving the lung. The patient also have some facial rashes. What is the most likely diagnosis?
**Correct Answer:** D. Lymphangioleiomyomatosis (LAM)
The key to the question is a history of renal embolisation. Tuberous sclerosis is associated with both LAM and large renal angiomyolipomas, which are prone to haemorrhage and require embolisation for treatment. The cysts of LAM are also classically regular and diffuse.
**A. Birt-Hogg-Dubé syndrome:** While Birt-Hogg-Dubé is also associated with renal manifestations like RCC and oncocytomas, these are typically treated with surgical excision rather than embolisation. The cysts are also lower lobe predominant and irregular.
**B. Lymphoid interstitial pneumonia (LIP):** LIP is usually associated with autoimmune disorders (especially Sjögren's syndrome) or immunodeficiency (e.g., HIV/AIDS). The cysts are typically lower lobe predominant rather than diffuse.
**C. Neurofibromatosis type 1:** The renal association in NF1 is renal artery stenosis. Can cause upper lobe predominant cystic lung disease
**E. Idiopathic pulmonary fibrosis (IPF):** Presents with basal predominant fibrosis and honeycombing rather than as a cystic disease