Pulmonary Histiocytosis X

Brief Discussion

Langerhans cell histiocytosis (histiocytosis X, eosinophilic granuloma)
- Early stage : nodular collections of Langerhans histiocytes and eosinophils (juxtabronchiolar )
- L
ate stage : fibrosis, honeycombing

-
young or middle-aged adults, male predominance, ciagrette smoking history (> 90%), pneumothorax (14%)
-
variable PFT pattern/ decreased diffusing capacity
- 60% (lung), 40% (lung + bone, other organ)
- Px : 1/2
  favorable, 1/3 stable,  20% progress ( respiratory failure, pulmonary a. HT)

CXR
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nodular, reticulonodular, and cystic abnormalities (upper lung >>)

Evolution on CT
N
odule (1-5mm, centrilobular and peribronchiolar) -> cavitary nodule & thick-walled cyst -> cyst (large confluent, bizarre) -> GGA, irregular lines & honeycombing
 
End-stage lung disease
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UIP (43%), sarcoidosis (15%), HX (13%), asbestosis, EAA, silicosis, LAM

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