We report a case of a 43–year–old African American female with a 30 years history of tobacco use who presented with complaints of severe retrosternal chest pain for 4 hours associated with mild non–productive cough. The pain radiate to upper back and both arm. She denied shortness of breath, palipitation, dizziness, nausea or diaphoresis. Physical examination revealed only fine bibasilar crackles. CT scan of the chest showed a reticulonodular infiltrate and cystic lesions predominantly in the upper and middle lung fields. Apart from anthracosis, bronchoscopy with BAL was not diagnostic. Therefore, the patient underwent an open lung biopsy via VATS and the results revealed a positive immunohistochemistry staining for S–100 protein and CD1a, confirming a diagnosis of PLCH. The patient was counseled on the strong association with smoking and PLCH. She was amenable to smoking cessation. One month after discharge, a PFT was performed and showed reversible obstructive pattern along with decreased diffusion capacity that concur to her diagnosis.
Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare, smoking related granoulamtous disorder characterized by focal infiltrate of Langerhans cells mixed with eosinophils. It makes up about 5% of cases of ILD. Approximately, 5–15% of cases of PLCH are reported to have extrapulmonary involvement, most commonly bone. More than 90% of cases of PLCH occur in adults who smoke. The disease can present with non–specific respiratory symptoms such as cough and dyspnea but seldom as chest pain. Often patients are not symptomatic and the disease is detected only on routine chest X–ray. In 10–40% of cases the diagnosis can be made with the use of bronchoscopy, bronchoalveolar lavage (BAL) and transbronchial Biopsy (TBB).
Retrospective studies have shown that minimally symptomatic patients will have a relatively good prognosis especially after smoking cessation. A subgroup of PLCH develop a variety of complications that harbor high morbidity and mortality. Therefore, many studies have described markers to help in identifying high risk patients so as to target them for aggressive smoking cessation and other types of experimental therapy. Despite the advances in understanding of the disease , smoking cessation was merely the evident line of treatment. A multicenter cooperative well designed study, where steroids and immunosuppressive agents are better evaluated, is still highly warranted.