Spontaneous Resolution of Pulmonary Langerhans Cell Histiocytosis Despite Smoking

Excerpt

A 27-year-old woman currently smoking 10 cigarettes a day, 10 pack-years, presented with non-productive cough. A postero-anterior chest X-ray (a) was performed followed by a chest CT scan, demonstrating multiple cysts and nodules with an upper to middle lung zone predominance (lung window; b, axial section; c, coronal section). Spirometry, lung volumes, and diffusion capacity for carbon monoxide were normal. The patient underwent video-assisted thoracoscopic lung biopsy showing numerous Langerhans cells (d, g, h) and positive immunohistochemical staining for CD1a (i) and S-100 protein. A diagnosis of Pulmonary Langerhans Cell Histiocytosis (PCLH) was made. The patient was advised to quit smoking but continued to smoke. During 18 months of follow-up, the patient’s cough resolved and pulmonary function tests were stable. Imaging of the brain and skeleton did not show any evidence of extrapulmonary involvement. After 18 months, a follow-up chest CT scan showed nearly complete resolution of the pulmonary lesions (lung window; e, axial section; f, coronal section). PLCH is usually associated with cigarette smoking and the initial focus of any therapeutic regimen in these patients is cessation of smoking [1]. Although spontaneous resolution has been described [2], prognosis in patients who continue smoking is usually less favorable. In this case, spontaneous resolution of PCLH upon radiological findings occurred despite continued smoking. The presence of nodules, while classic, is typically absent in most cases of PLCH (Fig. 1).

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