Case Presentation: A 27 year-old woman who is approximately 10 weeks pregnant presents with 8 week history of dry cough, shortness of breath, and mediastinal pain, worsened dramatically in the last 3 days. She was evaluated in an urgent care center about 4 weeks prior complaining of the cough and reduced exercise tolerance, and she was given empiric antibiotics and cough syrup without improvement. She has no fevers, no chills, and no known sick contacts. Her cough is non-productive, and both her cough and shortness-of-breath are worse with activity. She currently has to stop and rest after climbing a flight of stairs. Four weeks ago, she would get short of breath carrying in groceries, but 3 months ago she could do all of these activities without difficulty. Her medical history is significant for childhood asthma, which she had outgrown as a teenager. Family history is significant for a mother who died of a stroke at aged 38, attributed to OCPs and smoking. She has 2 young children who are healthy. She is married, smokes cigarettes (about ¾ of a pack per day), drinks alcohol rarely, and denies illicit drug use.
Her blood pressure and heart rate are normal. She is mildly tachypneic to 22bpm, and her oxygen saturation is 92% on room air, which drops to 81% with brief hallwalk. She is uncomfortable appearing, but able to talk in full sentences. Her heart is regular and without murmurs. She has decreased breath sounds on the right without adventitious sounds or egophany. Extremities are warm, with normal capillary refill and no clubbing or edema.
Beta-HCG confirms pregnancy. The remainder of laboratory studies is normal. A chest xray reveals right sided pneumothorax, and extensive cystic lung parenchymal changes on the left. After a chest tube is placed, a high resolution CT demonstrates multiple cysts in the lungs bilaterally of varying shape, as well as scattered lung nodules. Ultimately, she requires a bleb resection with pleurodesis to manage her pneumothorax due to persistent air leak, and a lung biopsy was taken at the time to distinguish between Lymphangioleiomyomatosis and Pulmonary Langerhans Cell Histiocytosis (PLCH), and ultimately the diagnosis of Pulmonary Langerhans Cell Histiocytosis was made.
Discussion: PLCH is an interstitial lung disease affecting young smokers. While the damage can be dramatic, typically smoking cessation is the only required treatment, and is crucial to stop progression of the disease. Rarely, patients progress despite stopping smoking, and then supportive care and lung transplant are the available options.
While PLCH is rare, it is common to see young patients with persistent cough. It is important to recognize the red flags that were missed prior to her development of the pneumothorax: the duration of her symptoms was longer than expected, marked hypoxia, and significantly reduced exercise tolerance in a previously healthy young woman all warrant further investigation. Acknowledging that her disease is uncommon, her presentation could have been consistent with other more common serious diagnoses, including pulmonary embolism, pulmonary hypertension, or decompensated heart failure. In this case, a simple chest xray at initial presentation would have provided evidence of her disease, would have avoided unnecessary antibiotics, and possibly could have prevented her pneumothorax.
Conclusions: It is important to recognize when a persistent cough could represent something more sinister than a post-viral syndrome in an otherwise healthy patient.
To cite this abstract:King J. The Smoking Woman. Abstract published at Hospital Medicine 2016, March 6-9, San Diego, Calif. Abstract 618. Journal of Hospital Medicine. 2016; 11 (suppl 1). https://www.shmabstracts.com/abstract/the-smoking-woman/. Accessed February 25, 2020.