American Thoracic SocietyI Know It When I See It

Based on these data, can you diagnose this perplexing case that eludes common respiratory diseases?

Investigating the cause of severe dyspnea and dry cough in a young male smoker presents an interesting diagnostic challenge. This patient’s symptoms are coupled with key findings including bilateral inspiratory crackles, hypoxemia after physical exertion, reticular shadows in both lungs on a chest X-ray, mild pulmonary hypertension on echocardiography, and a chest CT showing upper-lobes predominant bizarre shaped cysts with scattered lung nodules.

Key Data:

• A 24-year-old male active smoker presents with dyspnea on exertion, and dry cough lasting 4 months. The patient cannot walk more than 100m on flat ground without stopping for breath.
• Physical examination reveals bilateral inspiratory crackles, and significant hypoxemia (SPO2 84%) after climbing 2 flights of stairs.
• Chest X-ray shows reticular shadows in both lungs.
• Echocardiography shows normal left ventricular function, normal valves, with mild pulmonary hypertension.
• Pulmonary function tests show mild restriction with mild reduction in diffusion capacity.
• Routine chemistry is within normal limits.
• Chest CT reveals bilateral upper-lobes predominant bizarre shaped cysts with scattered lung nodules.
• Bronchoscopy and bronchoalveolar lavage (BAL) show no evidence of S-100 and CD-1a.

Question for Evaluation

Based on the data presented, what is the most probable diagnosis?

• Metastatic malignancy
• Pulmonary Langerhans cell histiocytosis (PLCH)
• Lymphocytic interstitial pneumonitis
• Atypical infection

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Bilateral upper-lobes predominant bizarre shaped cysts with scattered lung nodules.