89. Idiopathic Pulmonary Fibrosis Treatment: RFJC – INPULSIS

Posted on November 12, 2024 by Pulm PEEPs

Our episode today is diving into a broader initiative to discuss the management of interstitial lung disease. In this episode we will be talking about the treatment of Idiopathic Pulmonary Fibrosis through the lens of a journal club discussion of the NEJM 2014 INPULSIS trial. Today’s episode is hosted by Pulm PEEPs Associate Editor Luke Hedrick.

Meet Our Guests

Robert Wharton is a recurring guest on Pulm PEEPs as a part of our Rapid Fire Journal Club Series. He completed his internal medicine residency at Mt. Sinai in New York City, and is currently a first year pulmonary and critical care fellow at Johns Hopkins.

Dr. Nicole Ng is an Assistant Profess of Medicine at Mount Sinai Hospital, and is the Associate Director of the Interstitial Lung Disease Program for the Mount Sinai National Jewish Health Respiratory Institute.

Article and Reference

Today the discussion of IPF treatment centers around the 2014 NEJM publication of the INPULSIS trials investigating the efficacy of Nintedanib for the treatment of IPF.

Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, Cottin V, Flaherty KR, Hansell DM, Inoue Y, Kim DS, Kolb M, Nicholson AG, Noble PW, Selman M, Taniguchi H, Brun M, Le Maulf F, Girard M, Stowasser S, Schlenker-Herceg R, Disse B, Collard HR; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014 May 29;370(22):2071-82. doi: 10.1056/NEJMoa1402584. Epub 2014 May 18. Erratum in: N Engl J Med. 2015 Aug 20;373(8):782. doi: 10.1056/NEJMx150012. PMID: 24836310.

Infographic

Summary of Key Discussion Points

Background and Challenges in ILD Treatment: Interstitial lung diseases (ILDs), particularly idiopathic pulmonary fibrosis (IPF), had historically poor treatment outcomes, with numerous therapies showing either no benefit or even harm. Prior to 2014, effective treatments were extremely limited, and lung transplantation was the primary management option.

INPULSIS I and II Trials: These 2014 trials examined nintedanib, an antifibrotic drug initially tested for cancer, in patients with moderate IPF. The studies were well-structured, involving strict criteria to ensure accurate diagnoses and excluding younger patients or those with more advanced disease.

Nintedanib’s Mechanism and Design of the Trials: Nintedanib acts by blocking multiple tyrosine kinases that mediate fibrotic growth factors. Patients were monitored over a year, with primary endpoints focusing on forced vital capacity (FVC) decline—a common surrogate measure for disease progression in ILD trials due to its correlation with survival.

Outcomes: Both trials showed that nintedanib significantly reduced the rate of FVC decline compared to placebo, suggesting that it slowed disease progression. Secondary endpoints included reduced acute exacerbations (significant only in one trial) and minor improvements in quality of life, though these weren’t statistically or clinically significant.

Adverse Effects: Nintedanib’s side effects included gastrointestinal issues (diarrhea, nausea, vomiting) and, less commonly, liver enzyme elevations and cardiovascular events. While post-marketing data suggested some improvements in tolerability, clinicians still monitor for these side effects closely.

Application in Clinical Practice: The trials support nintedanib as an option for slowing IPF progression, though no cure or disease reversal is achieved. Clinicians weigh the choice between nintedanib and pirfenidone (another antifibrotic) based on each drug’s side effect profile and individual patient needs.

Future Directions: The trials paved the way for further research into multi-therapy approaches for ILD, targeting multiple disease pathways, similar to strategies in asthma or COPD. Upcoming therapies and trials aim to provide more targeted and effective options for IPF and other ILDs.

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59. Top Consults: Lung Transplant 101

Posted on November 28, 2023 by Pulm PEEPs

We’re back with our Top Consults series to talk about Lung Transplant! This is a topic that every pulmonologist should have background knowledge about since it impacts the care of patients with end-stage lung disease of any cause. We will talk about the indications for referral and transplant, how to advise patients and some unique considerations for evaluation. Enjoy, rate and review us, and share your thoughts about the episode!

Meet Our Guests

Dr. Meghan Aversa is an Assistant Professor of Medicine at the University of Toronto and her expertise involves patients with end stage lung disease and lung transplant.

Dr. Hannah Mannem is an Associate Professor of Medicine at the University of Virginia Health. Hannah joined faculty at UVA in 2016 and she has expertise in ILD and Lung Transplant.

Learning Points

Trends in lung transplant:

Global Increase in Lung Transplants: Over the past three decades, there has been a gradual worldwide increase in lung transplants, with approximately 4,500 performed annually. North America conducts over half of these transplants, and the growth is particularly notable in double lung transplants.
Indications and Disease Trends: Interstitial lung disease (ILD) has seen a significant rise in lung transplant indications, surpassing COPD as the leading cause. ILD, especially idiopathic pulmonary fibrosis (IPF), constitutes a substantial portion (40%) of all transplants. However, the trend is primarily observed in North America.
Decline in Cystic Fibrosis Cases: While Cystic Fibrosis is still a significant indication for lung transplant, its percentage has been declining, likely due to improvements in drugs and CFTR modulators.
Evolution of Lung Transplant Candidates: Over the past five years, lung transplant candidates have become sicker, with higher listing scores and increased hospitalization rates at the time of transplant. More patients have antibodies affecting match difficulty. The average age of patients has increased, with 35% being over 65, a demographic that was previously considered contraindicated.
Impact of COVID-19: The COVID-19 pandemic has influenced lung transplant trends. In 2020, UNOS added COVID-19-related ARDS and pulmonary fibrosis as indications. In 2021, these indications constituted about 10% of lung transplants, making it the third most common indication. Two-thirds were due to COVID-19 ARDS, and one-third due to pulmonary fibrosis. The long-term impact, especially with evolving vaccine dynamics, is still uncertain.

Indications for transplant referral:

ISHLT Consensus Document Update (2021): The ISHLT consensus document for lung transplant candidate selection was updated in 2021. It is available on the ISHLT website and serves as a valuable guideline for pulmonologists considering referrals for lung transplant assessment.
General Rule of Thumb for Chronic Lung Diseases: According to the consensus document, a general rule of thumb for all patients with chronic and stage lung diseases is to consider lung transplant if there is a high (more than 50%) risk of death from the lung disease within the next two years. Prognostic markers vary based on the underlying lung disease.
Disease-Specific Recommendations: The consensus document provides disease-specific recommendations. The key diseases highlighted are COPD, ILD, CF, and PH.
- COPD: Referral is recommended when the BODE index is in the range of 5 to 6, with additional factors that increase mortality, such as frequent exacerbations, low FEV1 (20-25%), or rapidly increasing BODE. Referral is also advised for clinically deteriorating patients or those with an unacceptably low quality of life despite maximal medical therapy.
- ILD (Particularly IPF): Early referral is suggested, ideally at the time of diagnosis. For any pulmonary fibrosis, referral is recommended if FEC is less than 80% or declining by 10% in two years, or DLCO is less than 40% or declining by 15% in two years. Other factors for referral include radiographic progression or a need for supplemental oxygen.
- Cystic Fibrosis (CF): Referral is encouraged for those with FEV1 less than 30%, and even 40% if there’s reduced walk distance, hypercapnia, PH, frequent exacerbations, or rapid decline.
- Pulmonary Hypertension (PH): Referral criteria include a REVEAL score of eight, significant RV dysfunction, progressive disease on therapy, need for IV prostacyclin therapy, and specific conditions like PVOD, PCH, scleroderma pulmonary artery aneurysms, which should be referred early due to their rapid progression.

Transplant evaluation process

Phases of Lung Transplant Evaluation:
- Referral and Initial Visit: The process begins with a referral, often from a primary pulmonologist. Patients can also self-refer. The initial phase involves insurance authorization and confirming the underlying diagnosis while ensuring all other treatment options are exhausted.
- Assessment of Disease Severity: The severity of end-stage lung disease is assessed to determine the timing of the workup, which varies depending on the patient’s condition and the center’s protocols.
- Diagnostic Steps: A thorough diagnostic workup follows the initial visit, including various tests, imaging, and meetings with multidisciplinary teams to assess medical and social factors influencing transplant success.
- Follow-Up Appointments: Patients typically have multiple follow-up appointments to track the evolution of the disease and ensure health maintenance and vaccinations are up to date.
- Selection Committee: The final phase involves a selection committee that determines if the patient is a candidate. If so, there may be conditional requirements before officially listing the patient.
Multidisciplinary Approach: Lung transplant evaluation involves collaboration with various specialists, including social work, finance, nutrition, pharmacy, physical therapy, and potentially other consult services. The efficiency of this process is optimized for both the patient and the medical team.
Diagnostic Workup:
- Medical Testing: Involves blood work, cardiac testing (echo, left and right heart cath), and imaging, including abdominal imaging, VQ scans, DEXA scans, and 24-hour urine analysis.
- Multidisciplinary Meetings: Patients meet with members of the multidisciplinary team, addressing medical comorbidities as well as social and psychological factors.
- Follow-Up Appointments: Multiple appointments allow for tracking disease progression and ensuring overall health maintenance.
Selection Committee Decision: The patient receives a decision from the selection committee, determining candidacy. Sometimes, patients are considered candidates with conditions (e.g., completing vaccinations or losing weight). Timing of listing is also discussed to ensure optimal candidacy.
Patient Involvement: Patients play an active role, and the process may involve self-referral, understanding and completing requirements, and active participation in follow-up appointments.
Efficiency and Individualization: The evaluation process is tailored to the patient’s condition, and centers aim to efficiently organize diagnostic workup and multidisciplinary meetings to optimize patient care.

Timing of transplant listing for candidates

COPD Patients: For COPD patients, listing is likely when the Bode index is around 7, the FEV1 is under 20%, there is at least moderate pulmonary hypertension (PH), chronic hypercapnia, or severe exacerbations.
ILD Patients: Patients with interstitial lung disease (ILD) are likely to be listed when showing signs of progression or decline in forced expiratory capacity (FEC), diffusing capacity of the lungs for carbon monoxide (DLCO), or six-minute walk distance. Other indicators include hypoxemia, secondary pulmonary hypertension, or hospitalization for complications.
CF Patients: Cystic fibrosis (CF) patients are considered for listing when FEV1 is below 25% or is rapidly declining, and if they experience frequent hospitalizations. Listing criteria also include the presence of pulmonary hypertension, chronic hypoxemia, or hypercapnia.
Pulmonary Hypertension Patients: Those with primary pulmonary hypertension may be listed when the reveal score is above 10 on intravenous therapy, there is progressive hypoxemia, or if there are renal or liver dysfunctions associated with pulmonary hypertension (PH).

Changes from the LAS system to the CAS system

Transition to Composite Allocation Score (CAS):
- Background and Timing: In March 2023, the lung allocation system (LAS) transitioned to the composite allocation score (CAS), a major change in the allocation of lung transplants.
- Reasoning Behind the Change: The change aimed to improve organ matching, prioritize sick candidates, enhance long-term survival, promote equity, increase transplant opportunities for specific patient groups (especially pediatric patients), and manage geographical variation in organ placement.
- Components of CAS:
  - Medical Urgency: Based on waitlist mortality at one year without a transplant and the likelihood of survival post-transplant, now assessed at greater than five years, with equal weighting.
  - Recipient Variables: Includes factors like height discrepancy, blood type matching, sensitization (immune system matching), and other recipient variations.
  - Candidate Biology: Focuses on pediatric patients (less than 18 years old) and individuals are a prior living donor.
  - Donor Variables: Addresses donor characteristics, emphasizing proximity and travel distance from the organ hospital.
- Early Data and Observations: The initial three-month monitoring period has shown changes in O blood type scores, prompting adjustments. Notable outcomes include a 16% increase in the number of lung transplants, a decrease in waitlist deaths and removals, and changes in median distance between donor hospital and transplant center.
- Exception Scores: The number of exception scores has increased, allowing for adjustments when the assigned score may not reflect the patient’s true medical urgency.
- Caution and Early Analysis: Early data, while promising, is subject to caution as centers were aware of the upcoming change. The impact on different age groups and the reasons for exceptions are being closely monitored and may evolve as more data becomes available.
Ongoing Monitoring and Potential Evolution: The data is being closely tracked by medical directors, and further changes to the scoring system may occur based on ongoing analysis and experience with the CAS. The impact on patient outcomes and allocation efficiency will continue to be studied and refined.

Advising patients on what to expect in terms of prognosis and survival after lung transplant

Survival Statistics:
- Overall three is approximately 50 percent survival at five years, and the median survival time is approximately six and a half years.
- Significant variations based on factors such as diagnosis, age, and comorbidities.
- Survival outcomes differ for specific groups, e.g., cystic fibrosis (CF) patients, those older than 65, and individuals with interstitial lung disease (ILD).
Quality of Life Emphasis:
- Shift in focus from survival alone to the patient’s goals and quality of life.
- Highlighting the importance of understanding and aligning with the patient’s individual quality of life expectations.
Investment in Healthcare Team and Lifestyle Change:
- Emphasis on the long-term commitment and involvement with the healthcare team post-transplant.
- A substantial investment in healthcare post-transplant, including regular visits, extensive blood work, and medication management.
- Cultural shift for patients to adapt to a new routine of frequent medical visits even when otherwise healthy.
Complications and Side Effects:
- Acknowledgment of potential complications within the first year, making the initial post-transplant period a full-time job.
- Discussion of various complications and medication side effects, ensuring patients are informed.
- Multidisciplinary approach involving nutritionists, physical therapists, and other specialists to address complications and enhance the patient’s quality of life.
Individualized Patient Approach:
- Recognition of the patient’s fight, spirit, and motivation as crucial factors for successful transplantation.
- Encouraging patients to set goals for their post-transplant life.
- Ethical considerations regarding transplanting older patients, with the importance of assessing overall well-being, motivation, and mental health.
Acknowledgment of Averages and Unpredictability:
- Communication of averages, but a reminder of the inherent unpredictability in the post-transplant course.
- Preparing patients for potential complications and the need to adapt to unforeseen challenges.
- Managing expectations by highlighting the unpredictability of individual transplant journeys.
Quality of Life Improvement:
- Despite complications and side effects, lung transplant often results in a significant improvement in the patient’s quality of life.
- Patients generally experience increased satisfaction and happiness post-transplant, outweighing the challenges associated with the procedure and subsequent care.

References for further reading

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54. Top Consults: Solitary Pulmonary Nodule

Posted on September 26, 2023 by Pulm PEEPs

We are thrilled to be back with another episode in our Top Consults series. We are talking about Solitary Pulmonary Nodules, which is something every pulmonologist will encounter in the clinic and on in-patient consults. We go through a number of cases and provide a framework for approaching these cases.

Meet our guests

Dr. Jessica Wang Memoli is board certified in pulmonary disease, critical care medicine and internal medicine. She is the Director of Bronchoscopy and Interventional Pulmonary, as well as the Associate Fellowship Program Director for Pulmonary Critical Care Medicine at the MedStar Washington Hospital Center. Dr. Wang Memoli received her medical degree from the University of Miami Miller School of Medicine. She completed her residency at MedStar Washington Hospital Center and her fellowship training at the Medical University of South Carolina in Charleston.

Dr. Nick Ghionni works at Union Memorial, Good Samaritan, and Franklin Square as an Intensivist and Pulmonologist. He completed his Internal Medicine residency at Mercy Catholic Medical Center in PA serving as Chief Internal Medicine resident. He was a fellow at MedStar Washington Hospital Center where he was the Chief Pulmonary Critical Care Fellow. His specific interests include mechanical ventilation, POCUS, and medical education.

Case Presentations

Case 1:

33 year old woman who came to the emergency department with acute onset of shortness of breath. She states that she had been in her normal state of health until this morning when she developed shortness of breath at rest, and chest pain. She does report a non-productive cough over the last few weeks which she feels may be contributing to her chest pain. She does report a history of asthma during childhood but without any exacerbations or maintenance therapies needed during her adulthood. She does report wheezing when she is sick with a cold but this is infrequent. The ED team sent off an initial work-up including a D-Dimer which was elevated, and she underwent a CTA of the chest for concern for possible PE. On the CT scan, there was no PE but the radiologist did call a “2 mm indeterminate right upper lobe pulmonary nodule.”

Case 2:

We have a 67-year-old male with a past medical history of ischemic cardiomyopathy, chronic systolic heart failure (LVEF 10-15%), s/p AICD, diabetes mellitus type 2, hyperlipidemia, hypertension, chronic kidney disease stage III, prostate cancer s/p seed implantation that was over 15 years ago who presented with acute decompensation of his heart failure and cardiogenic shock. He was successfully managed for that and is now being worked up by advanced HF and as a part of that workup got a chest CT, which found a RUL 6 mm nodule.

Case 3:

We have a 66-year-old male with a past medical history of HTN and drug abuse who presented to the ED with acute SOB, likely a COPD exacerbation. He was given bronchodilator and steroids as well as being started on Bipap. He eventually was able to be weaned off Bipap and was able to tolerate nasal cannula. As a part of his initial work up, the patient underwent CT scan for possible PE which demonstrated a new LUL spiculated nodule that is 1.3cm that is new since 2019.

Key Learning Points

Approaching Pulmonary Nodules:

A structured approach is essential due to the complexities of diagnosing pulmonary nodules.
Patient history, including risk factors, past interventions, and imaging, plays a vital role.
Nodules’ appearance, such as location, shape, or characteristics like calcification or spiculation, can provide diagnostic clues.
The nodules history on serial imaging is a key predictive risk factor for determining the likelihood that the nodule represents cancer
Tools like the Mayo Risk Calculator and Fleishner Society guidelines assist in risk assessment and guidance.
It’s essential to assess patient risk, and nodule risk, and prioritize patient concerns and education. Periodic monitoring or follow-up might be necessary based on the nodule’s risk and size.
A multidisciplinary approach involving various specialists ensures comprehensive care.

Key Discussion Points:

PET Scans:

Useful in gauging a nodule or tumor’s metabolic activity.
Large, hypermetabolic nodules are suspicious.
Not every positive PET result means malignancy; other causes like inflammation or scars can produce positive results.

Evaluating Nodules:

Consideration of nodule size, characteristics, patient history, and risk calculators is crucial.
Tumor boards provide a collaborative expertise approach.

Tissue Sampling & Testing:

The method of tissue sampling depends on resources and expertise.
CT-guided biopsy offers a high diagnostic yield but with a risk of pneumothorax.
Bronchoscopic biopsy provides a lower diagnostic yield than CT-guided biopsy but has a significantly reduced risk of complications.
Advanced diseases now often require molecular testing on tissue samples.

Ground Glass Nodules:

Different from solid nodules due to their slow growth rate.
Monitoring is crucial due to the potential for transformations raising cancer suspicions.
The approach for ground glass nodules typically involves more extended monitoring intervals than for solid nodules.

Holistic Evaluation:

Consider the nodule’s characteristics, the patient’s history, and clinical intuition.
Individualized patient assessment is as vital as evidence-based guidelines and clinical expertise.

See the infographic for a summary of key learning points:

References and further reading

Loverdos K, Fotiadis A, Kontogianni C, Iliopoulou M, Gaga M. Lung nodules: A comprehensive review on current approach and management. Ann Thorac Med. 2019 Oct-Dec;14(4):226-238. doi: 10.4103/atm.ATM_110_19. PMID: 31620206; PMCID: PMC6784443.

Mazzone PJ, Lam L. Evaluating the Patient With a Pulmonary Nodule: A Review. JAMA. 2022 Jan 18;327(3):264-273. doi: 10.1001/jama.2021.24287. PMID: 35040882.

MacMahon H, Naidich DP, Goo JM, Lee KS, Leung ANC, Mayo JR, Mehta AC, Ohno Y, Powell CA, Prokop M, Rubin GD, Schaefer-Prokop CM, Travis WD, Van Schil PE, Bankier AA. Guidelines for Management of Incidental Pulmonary Nodules Detected on CT Images: From the Fleischner Society 2017. Radiology. 2017 Jul;284(1):228-243. doi: 10.1148/radiol.2017161659. Epub 2017 Feb 23. PMID: 28240562.

Wahidi MM, Govert JA, Goudar RK, Gould MK, McCrory DC; American College of Chest Physicians. Evidence for the treatment of patients with pulmonary nodules: when is it lung cancer?: ACCP evidence-based clinical practice guidelines (2nd edition). Chest. 2007 Sep;132(3 Suppl):94S-107S. doi: 10.1378/chest.07-1352. PMID: 17873163.

Godoy MC, Sabloff B, Naidich DP. Subsolid pulmonary nodules: imaging evaluation and strategic management. Curr Opin Pulm Med. 2012 Jul;18(4):304-12. doi: 10.1097/MCP.0b013e328354a5f2. PMID: 22575798.

Hammer MM, Hatabu H. Subsolid pulmonary nodules: Controversy and perspective. Eur J Radiol Open. 2020 Sep 4;7:100267. doi: 10.1016/j.ejro.2020.100267. PMID: 32944597; PMCID: PMC7481135.

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41. Portopulmonary Hypertension and Hepatopulmonary Syndrome

Posted on May 16, 2023 by Pulm PEEPs

This week we are joined by one of our Associate Editors, Tess Litchman, as well as two guest experts to discuss two disease states that involve both the liver and the lung. Join us as we go through how to differentiate portopulmonary hypertension and hepatopulmonary syndrome.

Meet the Guests

Tess Litchman is a senior resident at Beth Israel Deaconess Medical Center and is one of the Associate Editors for PulmPEEPs. Tess will be continuing her training as a Pulmonary and Critical Care Medicine fellow at Brigham and Women’s Hospital next year.

Tyler Peck is an Instructor in Medicine at Beth Israel Deaconess Medical Center in the Division of Pulmonary and Critical Care Medicine. Tyler’s clinical and research interests are in pulmonary vascular disease and pulmonary hypertension.

Michael Curry is an Associate Professor of Medicine at Beth Israel Deaconess Medical Center and Section Chief of the Hepatology Department at BIDMC.

Further Readings and References

Rodríguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome–a liver-induced lung vascular disorder. N Engl J Med. 2008 May 29;358(22):2378-87. doi: 10.1056/NEJMra0707185. PMID: 18509123

Krowka MJ, Fallon MB, Kawut SM, et al. International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation 2016; 100:1440.

Peppas, S., Nagraj, S., Koutsias, G., Kladas, M., Archontakis-Barakakis, P., Schizas, D., Giannakoulas, G., Palaiodimos, L., & Kokkinidis, D. G. (2022). Portopulmonary Hypertension: A Review of the Current Literature. Heart, lung & circulation, 31(9), 1191–1202. https://doi.org/10.1016/j.hlc.2022.04.056

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37. Top Consults: Approach to Parapneumonic Effusions

Posted on March 21, 2023 by Pulm PEEPs

We continue our Top Consult Series on Pleural Disease and bring you a dedicated episode on Parapneumonic effusions. We are joined by two guest experts, Dr. David Feller-Kopman and Dr. Mihir Parikh. Listen in as we discuss the spectrum of parapneumonic effusions, including simple parapneumonic effusions, complicated parapneumonic effusions, and empyema. You will hear what to look for on imaging, what tests to send with pleural drainage as well as discuss the need for surgical consultation.

Meet our Guests

Dr. Mihir Parikh is currently an Assistant Professor of Medicine and academic interventional pulmonologist at Beth Israel Deaconess Medical Center. He is a highly esteemed educator and has worked to incorporate simulation training to improve procedural training for trainees and is a master of pleural disease.

Dr. David Feller-Kopman is a Professor of Medicine and the Section Chief of Pulmonary and Critical Care Medicine at Darmouth whose clinical and research expertise span the field of interventional pulmonology. Dr. Feller-Kopman is a true master of pleural disease, and has authored more than 225 peer-reviewed manuscripts and has been a leader for both ATS and CHEST committees.

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36. Top Consults Series: Approach to Pleural Effusions

Posted on March 7, 2023 by Pulm PEEPs

Today the PulmPEEPs are joined by two amazing educators as we start off our Top Consult series on Pleural Disease. Join us today as we go through cases to learn a systematic approach for evaluation and management of pleural effusions.

Meet our Guests

Dr. Mira John received her medical degree from Tulane University School of Medicine in New Orleans and completed internal medicine residency at Icahn School of Medicine at Mount Sinai. She is currently a second-year pulmonary and critical fellow at the University of Washington.

Dr. Ylinne Lynch completed her fellowship training at the University of Washington and is currently a Clinical Instructor at the UW. She is a great medical educator and spends her clinical time on the pulmonary consult service as well as in the ICU.

Learning Points

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20. Top Consults: Pulmonary Hypertension Diagnosis

Posted on July 5, 2022 by Pulm PEEPs

This week on Pulm PEEPs, we are continuing our Top Consults series with a discussion on the work-up and diagnosis of Pulmonary Hypertension. See our prior Radiology Rounds on signs of PAH on CT scan, and listen to our follow-up episode on right heart catheterizations for some background before this episode… or dive right in! We’ll cover everything from history and physical, to recent guideline changes in the definition of PH, and much, much more!

Meet Our Guests

Erika Berman Rosenzweig is a Professor of Pediatrics and the Director of the Pulmonary Hypertension Center and CTEPH Program at Columbia University Medical Center / New-York Presbyterian Hospital. She is an active member of the Pulmonary Hypertension Association, was the Editor-in-Chief of Advances in Pulmonary Hypertension and is on the Scientific Board of the World Symposium on PH.

Catherine Simpson is an Assistant Professor of Medicine at Johns Hopkins Hospital and is one of the faculty members in our Pulmonary Hypertension group. Her clinical and research areas of expertise are in pulmonary vascular disease and right heart function. Her research is focused on novel biomarker discovery and metabolomics in pulmonary vascular disease.

Cyrus Kholdani is an Instructor in Medicine at Beth Israel Deaconess Medical Center and Harvard Medical School. He is also the director of the Pulmonary Hypertension Program at BIDMC, and is actively involved in clinical care and clinical research in a variety of pulmonary vascular disease domains.

Consult Patient

Ms. Pamela Harris (PH) is a 47-year-old woman with PMH of migraines, obesity s/p gastric sleeve (BMI now 33), and a history of remote DVT in her 20s while on OCP s/p 6 months of AC who is referred to pulmonary hypertension clinic for evaluation of dyspnea on exertion. She has actually had dyspnea for some time and previously it has been attributed to her weight. Based on this, she pursued a gastric sleeve and has lost 55 pounds, but continues to have shortness of breath. She has no cough, and does not get dyspnea at rest, but notes that after 1 flight of stairs, or 2-3 blocks on flat ground she has shortness of breath. She saw her PCP and had basic labs, basic spirometry, and an echocardiogram. He did not note anything significant on examination in the notes.

The labs had no anemia, and normal renal and liver function. Her serum bicarbonate was 25 and there was no blood gas. Spirometry showed an FVC 82% predicted, FEV1 83% predicted, and FEV1/FVC was 99% predicted. The echocardiogram had normal LVEF, mild LVH, normal RV size and function qualitatively. There was mild TR with tricuspid valve peak regurgitant velocity of 3.4 m/sec. The estimated PASP + RA pressure (based on normal IVC diameter 2.1 cm) was 46 mmHg.

RHC: Systemic BPs 140s/90s, with O2 saturations 97-98% on RA throughout. RA mean pressure was 9, RV was 48 with an RVEDP of 17, PA was 48/27 with mean of 34, and PCWP mean was 11. CO/CI by Fick was 5.56 / 2.42, and by thermo was similar, 5.8 / 2.52. Her PA sat was 62%, and PVR was 3.97 WU.

Key Learning Points

History

Understand the constellation of symptoms and the functional limitation
- The goal is to assign a WHO functional class by the end of the visit
Evaluate the time course and evolution of the symptoms
Concerning symptoms that need to be addressed
- Palpitations
- Pre-syncope
- Syncope
- Chest pain
- LE edema
Evaluate for risk factors to explain or contribute to pulmonary hypertension
- Signs or symptoms of OSA
- Signs or symptoms of auto-immune disease
  - Raynauds
  - Skin changes
- Family history
  - Heritable lung disease
  - Clotting disorders
  - Auto-immune disease
- Social history
  - Exposure history
  - Smoking

Physical Exam

Look for signs that confirm PH
- Loud P2
  - Accentuated with elevated PVR
  - Can hear pretty early on. Could be one of the earliest findings
- TR murmur – pansystolic murmur at RUSB
- Diastolic murmur if severe pulmonary insufficiency
Look for signs of right heart failure
- JVD
- S4 gallop – later in course
- RV heave – later in course
- Peripheral edema
- Pulsatile liver or hepatosplenomegaly
Look for signs of other secondary causes of PH
- Mitral regurgitation or aortic stenosis murmur
- Asymmetric lower extremity edema
- Pulmonary edema
- Skin findings concerning for auto-immune disease or liver disease
- Arthritis

Work up for etiology of PH

CBC with diff – myeloproliferative and hemolytic anemia
CMP – renal function, liver function
Serologies – lupus, scleroderma, vasculitis – broad evaluation
HIV, hepatitis
Liver duplex if concerned
ECHO with bubble
Consider cardiac MRI
History of toxin and anorexigenic use
CT scan of the chest
PFTs including lung volumes and DLCO to evaluate for lung disease
Pulse oximetry at rest and with exercise
A sleep study or nocturnal oximetry
V/Q scan for all patients

References and links for further reading

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17. Top Consults: Pneumothorax

Posted on May 24, 2022 by Pulm PEEPs

This week on Pulm PEEPs we are resuming our Top Consults series with a common pulmonary presentation that can range from incidental to life-threatening: pneumothorax. We will talk through three different cases and review assessments and common management strategies. Make sure to subscribe to our show wherever you listen to podcasts, rate and review us, and visit our website to catch up on all our old content.

Meet Our Guests

Christine Argento is an Associate Professor of Medicine at Johns Hopkins Hospital and specializes in Interventional Pulmonology.

Charlie Murphy received his medical degree from LSU School of Medicine in New Orleans and completed his internal medicine residency at the Montefiore-Einstein Internal Medicine Residency Program. He is currently a Pulmonary and Critical Care fellow at New York-Presbyterian Hospital / Columbia University Medical Center, where he is one of the chief fellows.

Consult Patients

Barry is a 26-year-old man who came to the emergency department with acute onset of shortness of breath. He is tachypneic to 26, saturating 88% on RA so he was put on NC and is now 95% at 4L, HR 120, BP 145/85. There is only limited history but he reports he has never had anything like this before. His CXR shows a pneumothorax 5cm from the apex.

Larry is a 22-year-old man with normal HR and BP, saturating 96% on RA and breathing 14 x a minute. He has a CXR that shows a small pneumothorax. He has no past medical history and has never had a pneumothorax before, but he is a 1 PPD smoker and smokes marijuana.

Carrie is a 54-year-old woman who has been admitted with a COPD exacerbation. She has a history of emphysema, is not on home oxygen, and came in 2 days ago with worsening dyspnea and increased productive cough. She has been being treated with nebulizers every 4 hours, azithromycin, steroids, and supplemental O2 at 2L NC/ minute and never required NIPPV. This morning she had a coughing spell and significant chest pain and a CXR shows a moderate-sized left-sided pneumothorax. She is on 10L NC now with tachypnea to 26, and HR 105 but stable blood pressure.

Key Learning Points

Management options for a persistent air leak

— Conservative management: continue chest tube to suction

— Heimlich valve – can discharge a patient with this valve if they are stable to water seal, but don’t tolerate clamping

— Blood patch – inject the patient’s own blood into the chest tube to try to heal any pleural defect

— Chemical pleurodesis – inject talc powder, doxycycline, or another substance through the chest tube to cause pleural irritation and closure of the pleural space

— Endobronchial valve – off-label use

— VATS – surgical pleurodesis, resection of blebs

References and links for further reading

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9. Top Consults: Interstitial Lung Disease Diagnosis

Posted on February 15, 2022 by Pulm PEEPs

This week we are absolutely thrilled to be joined by three Interstitial Lung Disease experts to discuss the workup and differential for a patient with a new presentation of suspected ILD. This is also our first episode in a collaboration between the Pulm PEEPs and the American Thoracic Society Clinical Problems Assembly. In a series of episodes, we will be joined by pulmonary experts from around the country who are leaders in the ATS CP Assembly to provide content on common and cutting-edge topics in PCCM.

Meet Our Guests

Sonye Danoff is an Associate Professor of Medicine at Johns Hopkins and is Co-Director of the John Hopkins Interstitial Lung Disease and Pulmonary Fibrosis program. She also serves as the Assembly Chair of the Clinical Problems Assembly for the American Thoracic Society.

John Kim is an Assistant Professor of Medicine at UVA and has both clinical and research expertise in interstitial lung disease with a focus on pulmonary fibrosis.

Shweta Sood is an Assistant Professor of Medicine at Penn Medicine whose expertise is in Interstitial Lung Disease. She is an integral part of fellowship training where she leads the monthly ILD conference for fellows as well as provides didactics for ILD cases.

Consult Patient

A 66-year-old man who is a never smoker with a past medical history of hypertension and osteoarthritis was admitted to the hospital after presenting with progressive dyspnea on exertion to dyspnea at rest and was found to be hypoxemic. He reports 4 months of progressive dyspnea on exertion but on further questioning, thinks he was last normal about 1.5 years ago when he could walk 2 miles at a time. Currently, he can only walk 0.25 to 0.5 miles before needing to stop. He reports an intermittent, dry cough throughout the day that is not associated with eating, position, or sleeping. A full ROS is negative including for rashes or joint pains. His family history is notable only for hypertension and hyperlipidemia. He is a never smoker, drinks in moderation 1-2 x a week, and lives in the suburbs with his wife. His house has central heating and air conditioning, they have no pets, and they have carpeted floors. He is a retired police detective.

His physical exam is notable for fine crackles at the bilateral bases on pulmonary auscultation, and he is breathing comfortably on nasal cannula although mildly tachypneic to 18 breaths per minute. He has no signs of volume overload, no peripheral clubbing, no rashes, and joint exam does not reveal swelling or synovitis.

Key Learning Points

Take away points from our guests:

— ILD is a symptom, not a diagnosis

— The first time a patient is evaluated for interstitial lung disease is the best chance for making the diagnosis so take the time to evaluate them thoughtfully

— Start the physical exam with the hands first. The hands can reveal a lot about the patient (clubbing, cyanosis, joint, skin, and nailbed findings) and it establishes a personal connection

— When doing the pulmonary exam, percuss first from top to bottom to learn the size of the lungs, and then listen from bottom to top

— When reading a CT scan the simplest approach is “Is it a UIP pattern or not?”. This can be your first diagnostic divide. UIP is consistent with IPF, and in select circumstances connective tissue disease, occupational lung disease, or advanced hypersensitivity pneumonitis. Non-UIP patterns have a broader differential

— A multi-disciplinary interstitial lung disease conference is the gold standard for establishing an ILD diagnosis

Gathering the history:

— Ask about onset: acute or chronic. “When was the last time your breathing was entirely normal?”

— Symptoms can be shortness of breath, a lingering cough, or often fatigue and decreased energy. Occurrence is important! Do symptoms occur only with exertion or at rest too?

— “Have you ever had chest imaging before?”

— Take a thorough exposure history, and the weird questions are all necessary! Ask about birds and feathers (pet birds, bird feeders, down pillows or blankets, hunting, taxidermy), mold or water damage, organic or inorganic compounds from work (landscaping, ship yards, coal mines)

American College of Chest Physicians – Interstitial Lung Disease Patient Questionnaire

Physical Exam:

— Assess stability first and foremost

— Lung findings: crackles, inspiratory squeaks (often in hypersensitivity pneumonitis)

— Look for evidence of alternative diagnoses: volume overload, liver disease, signs of infection

— Evaluate for signs of connective tissue disease: examine the skin around the forehead and mouth for signs of scleroderma, look for rashes, perform a thorough joint examination and look at their hands, and assess muscle strength

Imaging:

— Order a high-resolution CT scan without contrast. High resolution means thin slices that are 1-2 mm thick. Contrast should be avoided if possible because it makes looking for subtle reticulations or ground-glass opacities harder

— Inspiratory and expiratory films help evaluate for gas trapping. If present, this may indicate hypersensitivity pneumonitis

— Prone films allow you to distinguish reticular changes in the dependent portions of the lungs from atelectasis

Reading the CT scan:

— Look at the distribution first. Is it uniform from top to bottom or not? Is it subpleural, peripheral predominant, or central?

— Identify key features: reticulation, traction bronchiectasis, honeycombing, ground-glass opacities, cysts, and nodules

Laboratory evaluation:

— ANA, Scl-70, DS DNA, anti-RNP, anti-centromere, RF, CCP, SSA, SSB, RNA pol 3, HIV, myositis panel, Hypersensitivity pneumonitis panel

Pulmonary function tests:

— A restrictive ventialtory defect is the classic pattern and can tell you about severity. In ILD, TLC, VC, FRC, and RV are generally all reduced in proportion

— Identify if there is obstruction or not, because if present this may indicate coexisting emphysema or hypersensitivity pneumonitis

–The DLCO can be helpful for disease severity, and for raising concern about other co-existing diagnoses such as pulmonary vascular disease, or emphysema

— A DLCO < 50% predicted may predict that the patient will need oxygen with exertion and the patient should be walked

6 Minute Walk Test:

— This should be performed for all new patients because it is important for prognosis

— In the first year after diagnosis, a 6MWD should be performed every 3 – 4 months to assess disease trajectory. It can be done every 6 – 12 months after that.

Differential Diagnosis:

Silo ILDs into two large buckets

1) An exposure, trigger, or underlying cause is present: hypersensitivity pneumonitis, medication-induced, occupational lung disease, connective tissue disease, granulomatous disorder

2) Idiopathic interstitial pneumonia

References and links for further reading

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7. Top Consults: Severe Asthma Exacerbation

Posted on January 18, 2022 by Pulm PEEPs

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

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PulmPEEPs

Pulmonary and Critical Care content for learners and practitioners of all levels

Tag Archives: Top Consults

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9. Top Consults: Interstitial Lung Disease Diagnosis

7. Top Consults: Severe Asthma Exacerbation