The Curious Case of Continuous Cough

Brittany Glassberg, B.S., Mackenzie Naert, B.A.,  Stefanie Haft, MS-SLP, & Joseph Truglio, M.D., MPH
November 16, 2019

A 31-year-old cis-gender woman with Juvenile Idiopathic Arthritis (JIA), prior pulmonary embolism (PE) and Zenker’s Diverticulum presented with progressive dyspnea on exertion, cough, and intermittent fever of 2 week’s duration. The patient takes albuterol as needed for shortness of breath, nystatin (15 g) as needed for thrush, esomeprazole (40 mg) once daily for gastroesophageal reflux, prednisone (5 mg) once daily and Rituximab in sodium chloride 375mg/m2 by intravenous route for JIA every three months.  The initial differential diagnosis included atypical pneumonia, Pneumocystis jirovecii due to chronic immunosuppression, aspiration pneumonitis or pneumonia from Zenker’s diverticulum, and interstitial lung disease (ILD) related to Rituximab.  On exam she was afebrile, had a heart rate of 104 and an oxygen saturation of 100% on room air. D-dimer was 2.10 ug/mL (patient’s baseline 3-5 ug/mL) with a WBC count of 17.2 x 103 cells/uL.  CT scan showed patchy ground-glass densities in the right upper, middle, and lower lobes of the lung, consistent with atypical pneumonia vs ILD.  She was initially treated with azithromycin for presumed atypical pneumonia, with some improvement, but not complete resolution of symptoms. A follow-up CT documented near resolution of the initial opacities post-antibiotics, but also demonstrated new, smaller ground-glass opacities.  She subsequently developed intermittent cough with chest pain, along with progressive dysphagia and positional choking. This was consistent with pneumonitis with progressive microaspiration. A videoesophagram demonstrated that the pharyngeal outpouching of her Zenker’s diverticulum had grown from 2cm to 5cm since 2011.  She was referred to ENT for surgical correction.

Question 1

Which of the following could be presenting symptoms of a Zenker’s Diverticulum?

1. Nausea and vomiting 

2. Dysphagia, aspiration, and weight loss

3. Bloody bowel movements 

4. Abdominal pain 

Question 2

Which of the following is a potential pulmonary complication of a Zenker’s Diverticulum?

1. Pneumonia

2. Interstitial Lung Disease 

3. Pleural Effusion 

4. Hemothorax 

Answers and Discussion

Answer 1. B) Dysphagia, aspiratoin and weight loss

Answer 2. A) Pneumonia

A Zenker’s diverticulum is a pseudodiverticulum of the pharyngeal mucosa above the crichopharyngeal muscle.  It usually presents in patients over 40 years old and can manifest as dysphagia, aspiration and weight loss. It also causes silent aspirations presenting as pneumonitis and pneumonia1.  Diagnosis can be made with the aid of a speech language pathologist during a modified barium swallow study.  Management focuses on swallow therapy, with endoscopic or surgical correction recommended for the majority of cases.  While a rare and often asymptomatic condition, Zenker’s diverticulum should be considered in patients with recurrent aspiration or dysphagia.  In patients with a known Zenker’s diverticulum, progressive symptoms may indicate growth in size.  

Our patient has multiple potential causes of cough and dyspnea.  Her JIA created an inflammatory state placing her at risk for recurrent PE.  Rituximab is recognized as potential cause of ILD2.  Chronic immunosuppression placed her at risk for atypical pneumonias, such as Pneumocystis jirovecii or Mycoplasma pneumoniae.  Finally, her Zenker’s diverticulum grew in size, thereby predisposing her to aspiration pneumonitis, as was ultimately diagnosed.  This case highlights the need for a high index of suspicion for a new Zenker’s or progression of a previously asymptomatic Zenker’s in patients with dysphagia, cough and suspected aspiration pneumonitjs. 

References:

1 Ferreira, L., Simmons, D.T., & Baron, T.H.  (8 October 2007). Zenker’s diverticula: pathophysiology, clinical presentation, and flexible endoscopic management. Diseases of the esophagus, 21, (1), 1-8.

2 Alexeeva, E. I., Valieva, S. I., et al. (September 2011). Efficacy and safety of repeat courses of rituximab treatment in patients with severe refractory juvenile idiopathic arthritis. Clinical Rheumatology, 30, (9), 1163-1172.

Authors

Brittany Glassberg earned a B.S. in Neuroscience at Duke University in 2016 and is currently a 2nd year medical student at the Icahn School of Medicine at Mount Sinai.

Mackenzie Naert earned a B.A. in Medical Anthropology at Yale University in 2015 and is currently a 3rd year medical student at the Icahn School of Medicine at Mount Sinai. 

Stefanie Haft earned a B.S in Communication Disorders from the University of Massachusetts, Amherst, and an M.S. in Speech Language Pathology from Seton Hall University. She is a Speech Language Pathologist at the Mount Sinai Hospital on both the outpatient and inpatient rehab units serving a diverse patient population. 

Joseph Truglio, MD MPH, is an Assistant Professor of Internal Medicine, Pediatrics and Medical Education and the course co-director of the Art and Science of Medicine at The Icahn School of Medicine at Mount Sinai.  He sees adult and pediatric patients at the Internal Medicine Associates, Pediatric Associates and the Pediatric Visiting Doctors Program at the Mount Sinai Hospital.