True regarding lymphangioleiomyomatosis – Pathology MCQ

Which of the following is true regarding lymphangioleiomyomatosis?
A. Primarily seen in old age
B. Commonly associated with persistent cough
C. Proliferation occurs in perivascular epithelioid cells
D. Disease is rapidly progressive

Correct answer: C. Proliferation occurs in perivascular epithelioid cells

Normal lung vs lung with lymphangioleiomyomatosis

• Lymphangioleiomyomatosis is primarily seen in young women of childbearing age.
• The commonest symptom is dyspnoea.
• Proliferation of perivascular epithelioid cells results in distortion of lungs with cystic dilation of terminal air spaces.
• It is a slowly progressing disorder.

Ref: Robbins & Cotran, Pathologic basis of disease, South Asia edition, Vol 2, p720, 721.
Image credits: National Heart Lung and Blood Institute. https://en.wikipedia.org/wiki/File:Lymphangioleiomyomatosis.jpg