Open or thoracoscopic lung biopsy is the most sensitive and specific test. Electron microscopy of tissue samples may demonstrate the characteristic Birbeck granules. Inflammatory cell infiltrate of Langerhans cells Eosinophils, neutrophils, and lymphocytes with focal lesions around the smaller bronchioles producing the stellate lesions. However, Eosinophils are not always present, thus the common name eosinophilic granuloma is a misnomer. Evidence of pulmonary vascular involvement and respiratory bronchiolitis are often present.
Smoking cessation is the most important medical intervention.
Refer patients with suspected PHX to a pulmonary disease specialist. Exercise and pulmonary rehabilitation are encouraged. These activities may improve the patient's functional status, even if there is no effect on disease progression. Neither corticosteroids nor cytotoxic agents are useful treatments. Supportive therapy is recommended.