A 20‐year‐old female smoker with history of bulimia presented to the emergency department with a syncopal episode. Computerized tomography (CT) of the cervical spine was performed, which incidentally revealed cavitary pulmonary nodules in the lung apices. She reported a dry cough and mild shortness of breath. She denied chest pain, wheezing, hemoptysis, fevers, night sweats, and weight loss. Routine blood work was unremarkable. A high‐resolution CT of the chest was performed, which revealed numerous cysts in the apices and midlung zones with relative sparing of the lung bases. Pulmonary function tests were normal. A bronchoscopy with transbronchial lung biopsies was performed. Tissue immunostaining for S‐100 protein and CD‐1 antigen confirmed the diagnosis of pulmonary Langerhans cell histiocytosis (PLCH).
PLCH is a rare disorder of unknown etiology that occurs predominantly in young smokers, with an incidence peak at 20–40 years of age. The exact incidence and prevalence of PLCH is unknown; the diagnosis is made in approximately 2%–5% of lung biopsy specimens from patients with interstitial lung disease. It usually occurs as a single‐system disease and is characterized by focal Langerhans cell granulomas infiltrating and destroying distal bronchioles. PLCH is pleomorphic in its presentation. Symptoms can be minor or absent, and patients often initially attribute their symptoms to smoking. The clinical course of the disease is unpredictable. A large percentage of patients experience stable, persistent disease, but many have a progressive course. Treatment consists of smoking cessation. Corticosteroid therapy may be useful in selected patients. Use of chemotherapeutic agents remains experimental. Lung transplantation may be considered in the case of unresponsive disease; however, the disease may relapse in the transplanted lung. Patients with PLCH require long‐term follow‐up to detect potential disease progression and relapse.
Although PLCH remains a rare disease, its prevalence may be greater than previously recognized. Clinicians should be aware of this disease in the differential diagnosis of interstitial lung disease, especially in patients who have exposure to cigarette smoke.
A. Tiwari ‐ none; T. Gravelyn ‐ none
To cite this abstract:Tiwari A, Gravelyn T. Cough, Dyspnea, and Interstitial Lung Disease in a 20‐Year‐Old Smoker. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 1015. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/cough-dyspnea-and-interstitial-lung-disease-in-a-20yearold-smoker/. Accessed May 26, 2019.