Diffuse cystic lung disease — LAM, LCH, BHD, LIP

CT

First and second year — the floor first, then every step

Cysts are thin-walled and air-filled (unlike emphysema, which has no wall); their shape and distribution identify the cause — round uniform (LAM), bizarre upper-zone (LCH), basal paramediastinal (Birt–Hogg–Dubé), few perivascular with ground glass (LIP/Sjögren).

Orient first

  • LAM: women of reproductive age or tuberous sclerosis; uniform round cysts throughout; renal angiomyolipomas, chylous effusions.
  • BHD: lower-zone, paramediastinal, elliptical cysts; renal tumours and skin fibrofolliculomas.
  • LIP: few cysts with ground glass and centrilobular nodules in Sjögren or HIV.

Acquire the study

  • High-resolution CT chest, thin sections, inspiratory; include the upper abdomen (kidneys).

The manoeuvre

  • Lung window: confirm walls (cyst) vs no wall (emphysema).
  • Size, shape and number of cysts; zonal distribution (upper, lower, diffuse).
  • Associated nodules (LCH), ground glass (LIP), pneumothorax.
  • Upper abdomen on the same series: renal angiomyolipomas (LAM/TSC) or renal masses (BHD).

What confirms it

  • A cyst pattern with its extrapulmonary association (VEGF-D, renal lesions, skin, serology).

What licenses you to exclude it

  • Centrilobular lucencies without walls around a central dot are emphysema.

The classic misread

  • Calling cystic bronchiectasis a cystic lung disease — cysts connect to airways.

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