8. A Case of Dyspnea and Lymphadenopathy

Dave Furfaro, Kristina Montemayor, and Ansa Razzaq are back to tackle another pulmonary case! Listen in and solve the case yourself, and we’ll share some diagnostic pearls along the way. Let us know any additional thoughts on Twitter.

Patient Presentation

The is patient is 57-year-old man with hypertension and asthma who presents with dyspnea and left-sided pleuritic chest pain for 3 weeks. He was in his usual state of health until 3 weeks prior to admission, when he developed dyspnea and sharp left-sided chest pain that worsens with deep breathing. His symptoms are accompanied by unintentional 30-pound weight loss over the past several months as well as an intermittent cough that is nonproductive.

On physical exam, he is mildly tachypneic and saturating well on room air with otherwise normal vital signs. He has decreased breath sounds at the right lung base.

Initial labs

Key Learning Points

**Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below

Physical Exam Pearls

Reasons for decreased breath sounds on physical exam

1. Increased thickness of chest wall

2. Reduced airflow to part of the lung

3. Overinflation to part of the lung

4. Something between the lung and chest wall — air or fluid

Determine bradypnea and tachypnea quickly by matching your breahting rate to the patient’s respiratory rate

Pleural Effusions

Transudative effusion partial differential: heart failure, nephrotic syndrome, hepatohydrothorax, fluid overload, hypoalbuminemia, urinothorax, amyloidosis*, chlothorax*, hypothyrodism*, malignancy*, sarcoidosis*

*these effusions often present as exudates but can be transudates

Exudative effusion partial differential: Infection, hemothorax, malignancy, connective tissue disease, chylothorax, pancreatitis, esophageal perforation.

Sarcoidosis

Key Elements of Diagnosis:

1. Is there a compatible presentation (imaging, physical exam)

2. Detection of non-necrotizing granulomatous inflammation in one or more tissue samples

3. Exclusion of other disease that may present similarily

Pulmonary stages of sarcoidosis:

Key to remember that patients don’t always progress through these stages. The system is useful for prognosticating and determining treatment based on the risk for disease progression.

Image source: Gupta Et Al. J Fam Pract. 2021 April;70(3):E4-E15 | 10.12788/jfp.0177

Treatment for pulmonary sarcoidosis

Patients with stage 1, and even many with stage 2, often don’t require treatment

The first-line agent is oral glucocorticoids and the typical starting dose is prednisone 20 – 40 mg by mouth daily. The patient should be evaluated closely, and ideally, this dose can be tapered starting at about 4 – 6 weeks. Following this, the prednisone dose is tapered slowly over 6 months – 1 year while monitoring for symptom recurrence.

Second-line steroid-sparing agents are methotrexate, azathioprine, or mycophenolate. These are often used if the patient relapses, or is on more then 10mg daily for 3 months after the initial taper with intolerance of steroids

Third-line agents: tumor necrosis factor (TNF)- alpha antagonists (small molecule or monoclonal antibody therapy)

References and links for further reading

  1. Light RW. Pleural Effusion. New England Journal of Medicine. 2002;346(25):1971-1977. doi:10.1056/NEJMcp010731
  2. Feller-Kopman D, Light R. Pleural Disease. New England Journal of Medicine. 2018;378(8):740-751. doi:10.1056/NEJMra1403503
  3. Sarcoidosis: An FP’s primer on an enigmatic disease. MDedge Family Medicine. 2021;70(3). doi:10.12788/jfp.0177
  4. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. New England Journal of Medicine. 2007;357(21):2153-2165. doi:10.1056/NEJMra071714
  5. Drent M, Crouser ED, Grunewald J. Challenges of Sarcoidosis and Its Management. New England Journal of Medicine. 2021;385(11):1018-1032. doi:10.1056/NEJMra2101555

7. Top Consults: Severe Asthma Exacerbation

We are excited to bring you another episode in our Pulm PEEPs Top Consults series! Kristina Montemayor and David Furfaro, are joined by Sandy Zaeh to discuss the assessment and management of a patient with a severe asthma exacerbation. We’ll follow a consult patient from the emergency department to the ICU, and cover everything from the physiology of pulsus paradoxus in asthma to how to manage the ventilator in status asthmaticus. Listen today and please send any questions our way on Twitter @pulmPEEPS.

Meet Our Guests

Sandy Zaeh is an Instructor of Medicine and Pulmonary & Critical Care Medicine physician at Yale School of Medicine.

Key Learning Points

References and links for further reading

  1. Chung KF, Wenzel SE, Brozek JL, et al. International ERS/ATS guidelines on definition, evaluation and treatment of severe asthma. European Respiratory Journal. 2014;43(2):343-373. doi:10.1183/09031936.00202013
  2. Rodrigo GJ, Rodrigo C, Hall JB. Acute asthma in adults: a review. Chest. 2004;125(3):1081-1102. doi:10.1378/chest.125.3.1081
  3. Godwin HT, Fix ML, Baker O, Madsen T, Walls RM, Brown CA. Emergency Department Airway Management for Status Asthmaticus With Respiratory Failure. Respir Care. 2020;65(12):1904-1907. doi:10.4187/respcare.07723
  4. Althoff MD, Holguin F, Yang F, et al. Noninvasive Ventilation Use in Critically Ill Patients with Acute Asthma Exacerbations. Am J Respir Crit Care Med. 2020;202(11):1520-1530. doi:10.1164/rccm.201910-2021OC
  5. Brenner B, Corbridge T, Kazzi A. Intubation and Mechanical Ventilation of the Asthmatic Patient in Respiratory Failure. Proc Am Thorac Soc. 2009;6(4):371-379. doi:10.1513/pats.P09ST4
  6. Laher AE, Buchanan SK. Mechanically Ventilating the Severe Asthmatic. J Intensive Care Med. 2018;33(9):491-501. doi:10.1177/0885066617740079
  7. Leatherman J. Mechanical ventilation for severe asthma. Chest. 2015;147(6):1671-1680. doi:10.1378/chest.14-1733

6. PEEP in ARDS Roundtable

This week on Pulm PEEPs, Dave Furfaro and Kristina Montemayor are joined by experts in the field of critical care medicine and ARDS to discuss all things PEEP! Drs. Roy Brower, Sarina Sahetya, Todd Rice, and Elias Baedorf-Kassis discuss everything ranging from PEEP basics to their approach to optimizing PEEP in patients with ARDS.

Meet Our Guests

Roy Brower is a Professor of Medicine at Johns Hopkins where he served as the MICU director for over 33 years, and he has been one of the pioneers for lung-protective ventilation for patients with ARDS.

Elias Baedorf-Kassis is an Assistant Professor of Medicine at Beth Israel Deaconess Medical Center and Harvard Medical School. He is the Medical Director of Respiratory Care at BIDMC, and helps lead the VV-ECMO program.

Todd Rice is an Associate Profess of Medicine in the Division of Allergy, Pulmonary, and Critical Care Medicine at Vanderbilt University and Vice President for Clinical Trial Innovation and Operations in the Vanderbilt Institute for Clinical and Translational Research.

Sarina Sahetya is an Assistant Professor of Medicine at Johns Hopkins Hospital and does research in the diagnosis and treatment of ARDS.


Key Learning Points

Driving Pressure figure from Amato et al. 2015. Stress index figure from Hess 2014.
  • The plateau pressure can be measured on the ventilator with an inspiratory hold maneuver
  • Extrinsic PEEP is applied by the ventiilator, while intrinsic PEEP, or auto-PEEP, occurs when there is incomplete emptying of the lungs due to inadequate time for exhalation. This often happens with obstructive lung disease. Intrinsic PEEP can be measured on the ventilator with an end-expiratory hold maneuver
  • We utilize PEEP in all intubated patients to minimize atelectasis. When patients are supine, the heart moves back 2 cm and the diaphragm raises by 2 cm, so often the left lower lobe of the lung is compressed and there is atelectasis there. This is often seen on CXR:

References, Image Sources, and Further Reading

  1. Higher versus Lower Positive End-Expiratory Pressures in Patients with the Acute Respiratory Distress Syndrome. New England Journal of Medicine. 2004;351(4):327-336. doi:10.1056/NEJMoa032193
  2. Amato MBP, Meade MO, Slutsky AS, et al. Driving Pressure and Survival in the Acute Respiratory Distress Syndrome. New England Journal of Medicine. 2015;372(8):747-755. doi:10.1056/NEJMsa1410639
  3. Writing Group for the Alveolar Recruitment for Acute Respiratory Distress Syndrome Trial (ART) Investigators. Effect of Lung Recruitment and Titrated Positive End-Expiratory Pressure (PEEP) vs Low PEEP on Mortality in Patients With Acute Respiratory Distress Syndrome: A Randomized Clinical Trial. JAMA. 2017;318(14):1335-1345. doi:10.1001/jama.2017.14171
  4. Beitler JR, Sarge T, Banner-Goodspeed VM, et al. Effect of Titrating Positive End-Expiratory Pressure (PEEP) With an Esophageal Pressure-Guided Strategy vs an Empirical High PEEP-Fio2 Strategy on Death and Days Free From Mechanical Ventilation Among Patients With Acute Respiratory Distress Syndrome: A Randomized Clinical Trial. JAMA. 2019;321(9):846-857. doi:10.1001/jama.2019.0555
  5. LaFollette R, Hojnowski K, Norton J, DiRocco J, Carney D, Nieman G. Using pressure–volume curves to set proper PEEP in acute lung injury. Nursing in Critical Care. 2007;12(5):231-241. doi:10.1111/j.1478-5153.2007.00224.x
  6. Hess DR. Respiratory mechanics in mechanically ventilated patients. Respir Care. 2014;59(11):1773-1794. doi:10.4187/respcare.03410
  7. Sahetya SK, Hager DN, Stephens RS, Needham DM, Brower RG. PEEP Titration to Minimize Driving Pressure in Subjects With ARDS: A Prospective Physiological Study. Respir Care. 2020;65(5):583-589. doi:10.4187/respcare.07102
  8. Umbrello M, Chiumello D. Interpretation of the transpulmonary pressure in the critically ill patient. Ann Transl Med. 2018;6(19):383. doi:10.21037/atm.2018.05.31
  9. Kenny JES. ICU Physiology in 1000 Words: Driving Pressure & Stress Index. PulmCCM. Published February 13, 2016. Accessed January 1, 2022. https://pulmccm.org/review-articles/icu-physiology-in-1000-words-driving-pressure-stress-index/

5. A Case of Chronic, Productive Cough

The Pulm PEEPs are joined again by Natalie West to discuss a patient who presented with a chronic, productive cough. Listen in today as we work through our differential diagnosis, interpret basic pulmonary testing, and share our clinical reasoning along the way. We have some fantastic diagnostic and treatment teaching points, so once you’ve solved the case check out the takeaways and infographics below. Please let us know any additional insights you have on Twitter!

Patient Presentation

A 50-year-old woman, who is a never smoker, with a past medical history of recurrent pancreatitis presents to the pulmonary clinic with a chronic, productive cough. Her cough has been present for 3 years and has increased in frequency to now being present daily. In the last three months, the cough has also worsened and is productive of small amounts of yellow to green sputum. She has a history of chronic post-nasal drip and sinus infections, and uses intranasal steroids, but has not noted changes in these symptoms. There is no significant family history of pulmonary disease, and an exposure history review of symptoms is negative.

On physical exam, she was a thin woman who appeared her stated age and was breathing comfortably on room air. Her exam was notable for mild expiratory wheezing, primarily on auscultation of the right posterior lung field. She had no cyanosis, clubbing, evidence of volume overload, or abdominal tenderness.

Basic Spirometry Values
Chest X-ray

Key Learning Points

**Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below.

Differential Diagnosis of Chronic Cough

Three most common causes: upper airway cough syndrome, GERD, cough variant asthma

Additional etiologies to consider: chronic bronchitis, post-infectious after a respiratory tract infection, bronchiectasis, ACE inhibitors, lung cancer, eosinophilic bronchitis, interstitial lung disease


Imaging Pearl


Evaluating Bronchiectasis


Making a New Diagnosis of Cystic Fibrosis in an Adult

Sweat testing

Sweat testing should be done in CF accredited center. Inform patients that there are no needles involved. Pilocarpine and electrical stimulation are applied to the arm or leg to stimulate the sweat gland, and then sweat is collected on filter paper, a gauze, or a plastic coil. From there, the amount of chloride in the sweat is calculated

Results

< 30 normal

31 – 60 indeterminate

> 60 is positive and Cystic Fibrosis is likely

What do you do with an Indeterminate test?

Patients with milder phenotypes of Cystic Fibrosis can have a normal or indeterminate sweat chloride level, and 10% of adults diagnosed with CF have a normal sweat chloride. If the sweat chloride test is indeterminate or normal, but suspicion is high for CF, then genetic testing for the whole array of mutations should be performed

References and links for further reading

  1. Morice AH, Millqvist E, Bieksiene K, et al. ERS guidelines on the diagnosis and treatment of chronic cough in adults and children. European Respiratory Journal. 2020;55(1). doi:10.1183/13993003.01136-2019
  2. Barker AF. Bronchiectasis. New England Journal of Medicine. 2002;346(18):1383-1393. doi:10.1056/NEJMra012519
  3. Bronchiectasis: a case-based approach to investigation and management | European Respiratory Society. Accessed November 23, 2021. https://err.ersjournals.com/content/27/149/180016
  4. Rowe SM, Miller S, Sorscher EJ. Cystic Fibrosis. New England Journal of Medicine. 2005;352(19):1992-2001. doi:10.1056/NEJMra043184
  5. Shteinberg M, Haq IJ, Polineni D, Davies JC. Cystic fibrosis. The Lancet. 2021;397(10290):2195-2211. doi:10.1016/S0140-6736(20)32542-3
  6. Jain R. Diagnosing Cystic Fibrosis in Adults: Better Late Than Never. Annals ATS. 2018;15(10):1140-1141. doi:10.1513/AnnalsATS.201806-432ED

4. Top Consults: Hemoptysis

Pulm PEEPs hosts, Kristina Montemayor and David Furfaro, bring our first episode in our Top Consults series. In this series, we will bring in experts to work through the most common pulmonary and critical care consults. Whether you are the consulting physician, or a pulmonologist responding to the page, these episodes are geared to give you all the information you need to care for your patients!

Today, we are joined by Chris Kapp and Matthew Schimmel, two interventional pulmonologists, to discuss hemoptysis. Chris and Matt will help us work through two hemoptysis consults, and together we’ll provide a framework for thinking about hemoptysis, outline some key components of the evaluation, and delve into treatment options.

Key Learning Points

Hemoptysis Evaluation

Hemoptysis Management

Life-Threatening or Large Volume Hemoptysis

  1. Stabilize the patient! Make sure the airway is protected either by the patient coughing themselves, or intubation if needed. Provide hemodynamic support with IVF, blood products, and pressors if needed. If it is known which lung has the bleeding the patient can be positioned so the lung with the bleeding is down. This protects the non-bleeding lung.
  2. Correct any bleeding diathesis If the patient is on anti-coagulation, or has any reversible bleeding diathesis, these should be corrected immediately to reduce further bleeding.
  3. Localize the bleed If the patient is stable, they should undergo a CTA to localize the bleeding. If they are not stable to make it to a CT scan, a bronchoscopy should be performed.
  4. Bronchoscopic treatment In addition to clearing blood from the airway, bronchoscopy can localize the bleeding. With available expertise, bronchoscopic treatments can be performed such as ice saline, topical epinephrine, or balloon tamponade to isolate the bleed.
  5. Definitive therapy with arteriography and embolization Patients with life-threatening hemoptysis should ultimately undergo arteriography and embolization of any bleeding vessel. If this is not possible, then surgery can be needed in some cases.
  6. A note on diffuse hemoptysis If there is not one distinct bleeding lesion, then localizing and treating the bleed becomes more difficult. For diffuse alveolar hemorrhage, evaluation should be performed for if it is primary, and due to an immunologic cause and capillaritis, or secondary to a systemic disease and / or bleeding diathesis. These investigations will guide available treatment options. Capillaritis from an immunologic cause, such as lupus or vasculitis, can be treated with systemic glucocorticoids and an additional immunosuppressive agent such as cyclophosphamide or rituximab.

Non-life-threatening or Small Volume Hemoptysis

  1. Monitor for clinical worsening Patient’s should be monitored, either in the in-patient or out-patient setting, for increased volume or frequency of hemoptysis and for any clinical worsening, such as desaturations or decreased ability to clear the airway.
  2. Correct any bleeding diathesis If the patient is on anti-coagulation, or has any reversible bleeding diathesis, these should be corrected immediately to reduce further bleeding. In pattients with non-life-threateneing hemoptysis this requires careful consideration of balancing the risk of bleeding vs the benefits for continuing anti-coagulation.
  3. Evaluate for underlying cause Patient’s should undergo imaging and evaluation for the underlying cause of the hemoptysis. This may be evidence of an underlying infection, a pulmonary embolism, or new lung lesions making the patient at risk. If the source can’t be found on non-invasive imaging, and there is no clear systemic source such as an infection, a bronchoscopy is warranted. Any underlying cause should be treated and investigated further.
  4. Inhaled Tranexamic Acid Nebulized tranexamic acid is well tolerated and can help resolve hemopytysis without invasive procedures.

References and links for further reading

  1. Gagnon S, Quigley N, Dutau H, Delage A, Fortin M. Approach to Hemoptysis in the Modern Era. Can Respir J. 2017;2017:1565030. doi:10.1155/2017/1565030
  2. Radchenko C, Alraiyes AH, Shojaee S. A systematic approach to the management of massive hemoptysis. J Thorac Dis. 2017;9(Suppl 10):S1069-S1086. doi:10.21037/jtd.2017.06.41
  3. Davidson K, Shojaee S. Managing Massive Hemoptysis. Chest. 2020;157(1):77-88. doi:10.1016/j.chest.2019.07.012
  4. Lara AR, Schwarz MI. Diffuse Alveolar Hemorrhage. CHEST. 2010;137(5):1164-1171. doi:10.1378/chest.08-2084
  5. Wand O, Guber E, Guber A, Epstein Shochet G, Israeli-Shani L, Shitrit D. Inhaled Tranexamic Acid for Hemoptysis Treatment: A Randomized Controlled Trial. Chest. 2018;154(6):1379-1384. doi:10.1016/j.chest.2018.09.026

3. A Case of Worsening Episodic Dyspnea

The Pulm PEEPs are excited to bring our first mystery case! Kristina Montemayor and Dave Furfaro hear a fascinating case presentation from Pulm PEEPs senior editor Ansa Razzaq. Join us as we work through this case together to come to a diagnosis, and share our thought process along the way. Come back to these show notes afterward, or once you’ve solved the case yourself, for some key teaching pearls and representative images.

Patient Presentation

A 66-year-old woman with no smoking history and past medical history of previously well-controlled asthma is referred to pulmonary clinic after multiple recent episodes of dyspnea, wheezing, and coughing. The episodes have features consistent with asthma exacerbations; however, they are also associated with migratory infiltrates. She has been treated with multiple courses of antibiotics and steroids, and despite escalating therapy, the episodes are occurring more frequently and she was worsening overall exercise tolerance. Listen in to hear more and try to solve the case!

Key Learning Points

**Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below.

References and links for further reading

  1. Israel E, Reddel HK. Severe and Difficult-to-Treat Asthma in Adults. New England Journal of Medicine. 2017;377(10):965-976. doi:10.1056/NEJMra1608969
  2. Asthma NAE and PP Third Expert Panel on the Diagnosis and Management of. Expert Panel Report 3: Guidelines for the Diagnosis and Management of Asthma. National Heart, Lung, and Blood Institute (US); 2007.
  3. Chung KF, Wenzel SE, Brozek JL, et al. International ERS/ATS guidelines on definition, evaluation and treatment of severe asthma. European Respiratory Journal. 2014;43(2):343-373. doi:10.1183/09031936.00202013
  4. Barker AF. Bronchiectasis. New England Journal of Medicine. 2002;346(18):1383-1393. doi:10.1056/NEJMra012519
  5. Chen T hsu, Hollingsworth H. Allergic Bronchopulmonary Aspergillosis. New England Journal of Medicine. 2008;359(6):e7. doi:10.1056/NEJMicm055764
  6. Agarwal R, Dhooria S, Singh Sehgal I, et al. A Randomized Trial of Itraconazole vs Prednisolone in Acute-Stage Allergic Bronchopulmonary Aspergillosis Complicating Asthma. Chest. 2018;153(3):656-664. doi:10.1016/j.chest.2018.01.005

2. Cystic Fibrosis Roundtable

The Pulm PEEPs (Kristina Montemayor and Dave Furfaro) host a panel of Cystic Fibrosis (CF) providers to discuss the current state of the disease, recent advances in cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, and the evolving faces and voices of Cystic Fibrosis.

Cystic Fibrosis is an autosomal recessive disorder caused by mutations in the CFTR gene that affects over 30,000 individuals in the United States and 70,000 people worldwide. Absence or dysfunction of the CFTR protein leads to abnormal secretion of mucus, sweat, and digestive fluids, which impacts the lungs, digestive tract, and reproductive system.

From the first formal publication on Cystic Fibrosis in 1938 by Dorothy Hansine Andersen, to the discovery of the delta F508 mutation and CFTR gene in 1988 -1989 by Lap-Chee Tsui, Francis Collins, and John R. Riordan, to the approval of the first CFTR modulator therapy, Ivacaftor, in 2012, our knowledge about Cystic FIbrosis has been advancing in leaps and bounds. As therapies have improved, they have dramatically impacted the lives of patients with Cystic Fibrosis. Join us today as we explore what this evolution in care has looked like from the perspective of Cystic Fibrosis providers, and hear about the new questions and challenges on the horizon.


Meet our guests

Emily DiMango is a Professor of Medicine at Columbia University Medical Center and the Director of the John Edsall-John Wood Asthma Center and the Gunnar Esiason Adult Cystic Fibrosis Program

Terri Laguna is an Associate Professor of Pediatrics at Northwestern Medicine / Feinberg School of Medicine and the Chief of Pulmonary and Sleep Medicine in the Department of Pediatrics

Patrick Sosnay is a Senior Medical Director at Vertex Pharmaceuticals and specializes in Cystic Fibrosis

Natalie West is an Assistant Professor of Medicine at Johns Hopkins Hospital and specializes in Cystic Fibrosis.


References and links for further reading

  1. Cystic Fibrosis Foundation
  2. Shteinberg M, Haq IJ, Polineni D, Davies JC. Cystic fibrosis. The Lancet. 2021;397(10290):2195-2211. doi:10.1016/S0140-6736(20)32542-3
  3. Rowe SM, Miller S, Sorscher EJ. Cystic Fibrosis. New England Journal of Medicine. 2005;352(19):1992-2001. doi:10.1056/NEJMra043184
  4. Davis PB. Cystic Fibrosis Since 1938. Am J Respir Crit Care Med. 2006;173(5):475-482. doi:10.1164/rccm.200505-840OE
  5. Barry PJ, Mall MA, Álvarez A, et al. Triple Therapy for Cystic Fibrosis Phe508del–Gating and –Residual Function Genotypes. New England Journal of Medicine. 2021;385(9):815-825. doi:10.1056/NEJMoa2100665
  6. Middleton PG, Mall MA, Dřevínek P, et al. Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. New England Journal of Medicine. Published online October 31, 2019. doi:10.1056/NEJMoa1908639

1. Meet the PEEPs!

We are excited to introduce Pulm PEEPs to the world! Our mission is to provide learners of all levels with multiple formats to engage in pulmonary and critical care education that you can access anytime. In addition to podcast episodes, we will be sharing helpful infographics and guides to shed light on topics throughout pulmonary and critical care from the bread-and-butter to the esoteric and complex. We will be gathering insights from world experts in the hope to share their knowledge and make it accessible for all. This is only the beginning and please join us each step of the way!

In this introductory episdoe you get to meet the PEEPs!

Kristina “Monty” Montemayor and Dave “Furf” Furfaro are the Co-Founders of Pulm PEEPs. They first met during residency at Johns Hopkins and share a love for medical education and all topics pulmonary and critical care related.

Ansa Razzaq is a Chief Resident in the internal medicine residency at New York-Presbyterian Hospital / Columbia University. She is interested in medical education and pulmonary and critical care medicine. She’ll be bringing amazing cases and helping us work through them from presentation to treatment.