Which factors are associated with adverse outcomes in eosinophilic granuloma (histiocytosis X)?

Updated: Mar 29, 2018
  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

Retrospective studies suggest that the following factors are associated with adverse outcomes in PLCH:

  • Extremes of age

  • Extensive cysts and honeycombing radiographically

  • Prolonged corticosteroid therapy

  • Multiorgan involvement

  • Abnormal pulmonary function, including reduced gas exchange, as measured by diffusing capacity for carbon monoxide; obstructive ventilatory defect (reduced ratio of forced expiratory volume in 1 second (FEV1) to forced vital capacity [FVC], or FEV1/FVC); and/or evidence of airtrapping (high residual volume/total lung capacity)

Young men who have diabetes insipidus have the worst prognosis.

Favorable signs include the radiographic finding of sparing of the costophrenic angles and a cellular, nonfibrotic biopsy specimen.


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