46. PulmPEEPs Interview with Dr. Jessica Zitter

We are thrilled to spend time with Dr. Jessica Zitter who is an expert in critical care and palliative care medicine, author, speaker, and documentary filmmaker. We are honored to have her join the show today to discuss her work on improving end-of-life and humanistic care as well as promoting conversations about death and dying.

Meet our Guest

Dr. Jessica Zitter received her medical degree from Case Western Reserve University Medical School, and her MPH from UC Berkeley. She completed her IM residency at BWH, her pulmonary and critical care training at UCSF, and is additionally board certified in Palliative Care. She is an author of multiple essays and articles that have appeared in publications ranging from the NY Times to the Journal of the American Medical Association, has authored a book, and is a documentarian whose work is Emmy and Oscar nominated.

Dr. Zitter’s first book, Extreme Measures: Finding A Better Path to the End of Life, offers an insider’s view of intensive care in America today and its impact on how we die. Her work is featured in the Oscar and Emmy-nominated short documentary Extremis, available to view on Netflix. She has also produced and directed the award-winning, 2020 short documentary Caregiver: A Love Story. Dr. Zitter is currently working on her third film, The Chaplain of Oakland, which explores the devastating problem of racial healthcare inequities at the end of life. She has also founded the production and education non-profit, Reel Medicine Media.

45. Meet the Glaucomfleckens

We are so excited to be joined today by the Glaucomfleckens. Listen today as we meet Kristin Flannary or Lady Glaucomflecken as well as Will Flanary better known as Dr. Glaucomflecken.

Learn about the history behind the Glaucomflecken name, the inspiration for Glaucomflecken videos as well as the advocacy work they are doing. Make sure to also check out their new podcast, Knock, Knock, Hi!

Meet our Guests

Kristin Flannary completed her undergraduate work at Texas Tech University and obtained a graduate degree from Dartmouth after training in cognitive neuroscience and social psychology. She has worked as an advocate and educator for survivors of critical illness, and for the families and partners of survivors. She co-founded Glaucomflecken with Will and has received multiple awards for her work in resuscitation science, including the AHA 2022 Resuscitation Champion award.

Will Flannary completed his undergraduate at Texas Tech University, and his MD at Dartmouth. He completed his residency in ophthalmology at the University of Iowa, and currently is an Ophthalmologist at EyeHealth Northwest in Portland, Oregon. During his education and training he was diagnosed with testicular cancer, a recurrence, and then suffered a cardiac arrest, for which Kristin, quickly acted. After surviving all of this with tenacity and a strong sense of humor, he has transformed his hilarity into multiple successful comedic initiatives about medical training

44. Decompensated Right Ventricular Failure in Pulmonary Arterial Hypertension

We are extremely excited to be hosting this episode in collaboration with CardioNerds! We have known Amit and Dan for many years, and they have been huge supporters of Pulm PEEPs, so it is an honor to address a topic we’re all interested in together.

We are joined by experts in the field today to discuss acute, decompensated right ventricle failure in patients with Pulmonary Arterial Hypertension (PAH). This topic can be quite intimidating, so we hope this will serve as a valuable guide for anyone who encounters a patient like this in the ICU.

Meet Our Guests

Leonid “Leon” Mirson is an internal medicine resident at the Johns Hopkins Hospital Osler Medical Residency and an Associate Editor here at Pulm PEEPs. He was born in Ukraine and moved to Philadelphia in early childhood with his family. He received his undergraduate degree from the University of Pittsburgh where he studied biomedical engineering and received his medical degree from the University of Pittsburgh School of Medicine. His current interests include pulmonary and critical care medicine with a focus on pulmonary hypertension as well as medical education. He is a rising PCCM fellow at the University of Pennsylvania.

Bhavya Varma completed her medical school at the University of Pittsburgh, her internal medicine residency at Johns Hopkins, and is a rising Cardiology fellow at NYU. She is interested in medical education and has done work with CardioNerds during her residency.

Mardi Gomberg-Maitland is a Professor of Medicine at George Washington University. She serves as the Medical Director of the Pulmonary Hypertension Program at George Washington Hospital. She completed her medical degree at Albert Einstein College of Medicine, completed her residency at the Weill-Cornell Medical Center, and completed her fellowship in cardiovascular diseases at Mount Sinai Medical Center. Her research focus is on understanding the epidemiology of pulmonary hypertension and the development of novel therapeutics and biomarkers. Dr. Gomberg-Maitland is internationally known for her work, she has had extensive grant funding and has published over 150 articles, abstracts, reviews, and chapters.

Rachel Damico is a pulmonologist and an Associate Professor of Medicine at Johns Hopkins Hospital, where she is also the Associate Director of the physician-scientist training program. Dr. Damico received her medical degree and doctoral degree in Molecular and Cellular biology from the University of Pennsylvania. She completed her residency in the Osler Internal Medicine training program and continued on as a PCCM fellow at Johns Hopkins. She has quickly achieved an international reputation in the field of pulmonary vascular biology and both basic and translational research, as well as clinical excellence, in Pulmonary Arterial Hypertension.

Patient Presentation

A 21-year-old woman with a past medical history notable for congenital heart disease (primum ASD and sinus venosus with multiple surgeries) complicated by severe PAH on home oxygen, sildenafil, ambrisentan, and subcutaneous treprostinil is presenting with palpitations, chest pain, and syncope. She presented as a transfer from an outside ED where she arrived in an unknown tachyarrhythmia and had undergone DCCV due to tachycardia into the 200s and hypotension. On arrival at our hospital, she denied SOB but did endorse nausea, leg swelling, and poor medication adherence. Her initial vitals were notable for a BP of 80/50, HR 110, RR 25, and saturating 91% on 5L O2.  On exam, she was uncomfortable appearing but mentating well. She had cool extremities with 1-2+ LE edema. Her JVP was 15cm H2O. She has an RV Heave and 2/6 systolic murmur. Her lungs were clear bilaterally. Her labs were notable for Cr 2.0, an anion gap metabolic acidosis (HCO3 = 11), elevated lactate (4.1), elevated troponin to 14,  and a pro-BNP of ~5000.  Her CBC was unremarkable. Her EKG demonstrated 2:1 atrial flutter at a rate of 130.

Key Learning Points

Diagnosing RV failure in patients with PH:

RV dysfunction and RV failure are two separate entities. RV dysfunction can be measured on echocardiography, but RV failure can be thought of as a clinical syndrome where there is evidence of RV dysfunction and elevated right sided filling pressures.

RV failure is a spectrum and can present with a range of manifestations from evidence of R sided volume overload and markers of organ dysfunction, all the way to frank cardiogenic shock. Most patients with RV failure are not in overt shock.

One of the first signs of impending shock in patients with RV failure is the development of new or worsening hypoxemia. Patients with decompensated RV failure approaching shock often do not present with symptoms classic for LV low flow state. Instead, hypoxia 2/2 VQ mismatching may be the first sign and they can be otherwise well appearing. Particularly because patients with PH tend to be younger, they can often appear compensated until they rapidly decompensate.

Causes of decompensation for patients with RV dysfunction and PH:

Iatrogenesis (inadvertent cessation of pulmonary vasodilators by providers, surgery if providers are not familiar with risks of anesthesia), non-adherence to pulmonary vasodilators (either due to affordability issues or other reasons), infections, arrhythmias (particularly atrial arrhythmias), and progression of underlying disease.

Patients with atrial arrhythmias (atrial flutter or atrial fibrillation) and pulmonary hypertension do not tolerate the loss of the atrial kick well as it contributes a significant amount to their RV filling and impacts their cardiac output. It is often difficult to determine if the atrial arrhythmia is a cause or effect of decompensated RV failure, but its presence is associated with a worse prognosis. Efforts should be made to re-establish normal sinus rhythm in patients with decompensated RV failure and atrial arrhythmias. 

A patient’s home PH medications should never be stopped for any reason upon admission unless on the basis of recommendations by a pulmonary hypertension provider as this is often a cause of decompensation inpatient

Interpreting findings on echocardiogram: 

Echo is a useful screening tool. When interpreting evidence of RV dysfunction, it is important to look at the global picture and not just one measurement.

RVSP, though commonly reported, may be of limited value when evaluating for decompensation. It’s a function of blood pressure, heart rate, and cardiac output. RVSP may even decline as shock worsens.

TAPSE is useful as a marker of RV dysfunction if it is reduced, but it is difficult to follow over time and only gives information about cardiac function around the annulus; it may be normal even when apical RV function is depressed. RV fractional area of change may be more useful for global RV function. It is important to pay attention to the RV size overall, the degree of TR, and the presence of effusion all of which are associated with RV dysfunction.

­Tips regarding the interpretation of invasive hemodynamics:

Cardiac output by thermodilution is the standard way to calculate PVR. Despite the degree of TR that is typically present, it is thought to be a better representation of cardiac output than the estimated Fick calculation.  

Our experts agree that routine monitoring of invasive hemodynamics for acute decompensated RV failure is likely not helpful and has significant risks. A good external volume exam or CVP off a central venous catheter + central venous saturation will likely be all you need to navigate a patient with shock secondary to RV failure. A right heart catheterization (should be only done under fluoroscopy for patients with large RVs) may be helpful if the etiology of shock is unclear. 

Management of decompensated RV failure in patients with pulmonary hypertension

Managing preload is of utmost importance, perhaps the most important tenant of management of decompensated RV failure.  The overwhelming majority of patients with PH and decompensation are volume overloaded, it is exceptionally rare that someone would be dry. Furthermore, the myth that the RV is “preload responsive” is only true in the setting of acute RV injury (eg. RV infarction) and not so in patients with acute on chronic RV dysfunction. It is important to optimize preload in someone in decompensated RV failure and it is safe to do this more rapidly than traditionally taught. Exact goals varied between our experts, but anywhere from 2-4L net negative per day is reasonable especially if the patient is hemodynamically tolerating the fluid removal. If the patient is not responding to diuretics, hemodialysis with ultrafiltration may be necessary to optimize the patient.

Afterload is the next tenant of management. Optimizing the following parameters will reduce the patient’s pulmonary vascular resistance and reduce afterload to the right ventricle.

— Avoiding hypoxic pulmonary vasoconstriction, liberalize the patient’s O2 goal 

— Avoid permissive hypercapnia and academia in this patient population

— Do not withhold a patient’s pulmonary vasodilator until discussion with the PH team. If stopped inadvertently, restart this medication immediately. For patients with malfunctioning pumps, there is a phone number on the back that you can call for rapid troubleshooting. Sildanefil can be given IV if a patient is NPO. 

— Inhaled nitric oxide can improve oxygenation and reduce afterload  

— Intubation and mechanical ventilation greatly increase PVR and are poorly tolerated. Exacting care must be taken to titrate PEEP and tidal volume, and avoid intubation when possible.

— Starting a new systemic pulmonary vasodilator in decompensated RV failure may be considered under close guidance from the pulmonary hypertension team

Management of atrial arrhythmias:

As above, patients with severe pulmonary hypertension do not tolerate loss of sinus rhythm well. If they are decompensated, every effort should be made to re-establish normal sinus rhythm. 

Management of RV perfusion:

Unlike the LV, the RV is perfused during BOTH systole and diastole. Maintaining effective coronary perfusion to the RV is essential in RV failure. For this reason, the systemic systolic pressure (as well as the mean arterial pressure) should be kept high enough to ensure that the RV is able to perfuse. There is no great body of evidence as to which pressor works best. Norepinephrine, vasopressin, and even phenylephrine are all reasonable choices to maintain appropriate perfusing blood pressure. 

Inotropy:

Patients in shock and RV failure do not always require inotropes, but if they do it’s often a sign of a grim prognosis. Either dobutamine or milrinone is reasonable, but the negative effects of these drugs (arrhythmias, tachycardia, and systemic hypotension) may limit their uses. 

Mechanical circulatory support:

Limited options are available. Balloon pumps and Impella devices have limited roles except in expert centers, and ECMO remains the standard of care. ECMO (either V-V or V-A) may have utility as a bridge to recovery if a reversible cause is identified, or a bridge to transplant if the patient is on the transplant list. 

Goals of care:

The prognosis of a patient admitted to the ICU with acute on chronic decompensated RV failure is guarded, with very high mortality rates even if not in shock

It is important for the patient’s longitudinal pulmonary hypertension provider to discuss the prognosis and goals of care ahead of time but this is not always possible. If they are admitted, early discussions regarding code status and prognosis are essential. It may be helpful to bring in the patient’s longitudinal pulmonary hypertension doctor into these discussions if possible. 

Infographic:

References and further reading:

Ventetuolo CE, Klinger JR. Management of acute right ventricular failure in the intensive care unit. Ann Am Thorac Soc. 2014 Jun;11(5):811-22. doi: 10.1513/AnnalsATS.201312-446FR. PMID: 24828526; PMCID: PMC4225807.

Arrigo M, Huber LC, Winnik S, Mikulicic F, Guidetti F, Frank M, Flammer AJ, Ruschitzka F. Right Ventricular Failure: Pathophysiology, Diagnosis and Treatment. Card Fail Rev. 2019 Nov 4;5(3):140-146. doi: 10.15420/cfr.2019.15.2. PMID: 31768270; PMCID: PMC6848943.

Kholdani CA, Fares WH. Management of Right Heart Failure in the Intensive Care Unit. Clin Chest Med. 2015 Sep;36(3):511-20. doi: 10.1016/j.ccm.2015.05.015. Epub 2015 Jun 27. PMID: 26304287.

Houston BA, Brittain EL, Tedford RJ. Right Ventricular Failure. N Engl J Med. 2023 Mar 23;388(12):1111-1125. doi: 10.1056/NEJMra2207410. PMID: 36947468.

Radiology Rounds – 5/30/2023

A middle-aged man with metastatic esophageal cancer presents with 2 weeks of low-grade fevers and more recently progressive dyspnea. His exam is notable for late inspiratory crackles throughout the right lung.

A bronchoscopy was performed and an expanded infectious panel was sent and all were negative, as were serum infectious studies (e.g. histoplasmosis ag, urine legionella etc). A trans bronchial biopsy showed plugs of granulation tissue filling the alveolar spaces

He was treated with steroids but her hypoxemia progressed and she was ultimately intubated. What is your treatment of choice for refractory ICI pneumonitis?

Severe ICI is rare and the treatment for steroid refractory disease is still based on small series and case reports This patient was treated with high-dose steroids (2mg/kg), IVIG, MMF, and tocilizumab. With that, he improved after 10 days intubated and was weaned to room air.

43. ATS 2023 Symposium Preview – Cardiac Arrest: New Science and Changing Guidelines

We’re podcasting again today from #ATS2023! Yesterday, we heard all about the conference in general, with some great recaps and previews of the remaining sessions. Today, we are coming to you in collaboration with the Critical Care Assembly to preview one of the symposiums they are hosting. This session is about Cardiac Arrest: New Science and Changing Guidelines and is happening today (the day of podcast release, 5/23/23)! If you’re interested in hearing more after the episode then please plan to attend at 2:15 PM in Room 150 A-B.

For those of you who aren’t at the conference, or couldn’t make this session, we’ll talk about some of the key points and discussion topics. We will additionally be talking to the chairs of other symposiums over the next coming months so stay tuned for some more highlights from ATS 2023.

Meet Our Guest

Ari Moskowitz is an Assistant Professor of Medicine at Montefiore Medical Center / Albert Einstein College of Medicine. He is additionally the MICU Director at Montefiore Einstein and Director of Critical Care Quality Improvement. He is an NIH-funded researcher and has conducted research in cardiac arrest care, sepsis, and ARDS.

Meet our Collaborators

The American Thoracic Society Critical Care Assembly is the largest Assembly in the American Thoracic Society. Their members include a diverse group of intensivists and care providers for both adult and pediatric critically ill patients. The primary goal of the Critical Care Assembly is to “improve the care of the critically ill through education, research, and professional development.”

References and Further Reading

  1. Nielsen N, Wetterslev J, Cronberg T, Erlinge D, Gasche Y, Hassager C, Horn J, Hovdenes J, Kjaergaard J, Kuiper M, Pellis T, Stammet P, Wanscher M, Wise MP, Åneman A, Al-Subaie N, Boesgaard S, Bro-Jeppesen J, Brunetti I, Bugge JF, Hingston CD, Juffermans NP, Koopmans M, Køber L, Langørgen J, Lilja G, Møller JE, Rundgren M, Rylander C, Smid O, Werer C, Winkel P, Friberg H; TTM Trial Investigators. Targeted temperature management at 33°C versus 36°C after cardiac arrest. N Engl J Med. 2013 Dec 5;369(23):2197-206. doi: 10.1056/NEJMoa1310519. Epub 2013 Nov 17. PMID: 24237006.
  2. Dankiewicz J, Cronberg T, Lilja G, Jakobsen JC, Levin H, Ullén S, Rylander C, Wise MP, Oddo M, Cariou A, Bělohlávek J, Hovdenes J, Saxena M, Kirkegaard H, Young PJ, Pelosi P, Storm C, Taccone FS, Joannidis M, Callaway C, Eastwood GM, Morgan MPG, Nordberg P, Erlinge D, Nichol AD, Chew MS, Hollenberg J, Thomas M, Bewley J, Sweet K, Grejs AM, Christensen S, Haenggi M, Levis A, Lundin A, Düring J, Schmidbauer S, Keeble TR, Karamasis GV, Schrag C, Faessler E, Smid O, Otáhal M, Maggiorini M, Wendel Garcia PD, Jaubert P, Cole JM, Solar M, Borgquist O, Leithner C, Abed-Maillard S, Navarra L, Annborn M, Undén J, Brunetti I, Awad A, McGuigan P, Bjørkholt Olsen R, Cassina T, Vignon P, Langeland H, Lange T, Friberg H, Nielsen N; TTM2 Trial Investigators. Hypothermia versus Normothermia after Out-of-Hospital Cardiac Arrest. N Engl J Med. 2021 Jun 17;384(24):2283-2294. doi: 10.1056/NEJMoa2100591. PMID: 34133859.
  3. Vallentin MF, Granfeldt A, Meilandt C, Povlsen AL, Sindberg B, Holmberg MJ, Iversen BN, Mærkedahl R, Mortensen LR, Nyboe R, Vandborg MP, Tarpgaard M, Runge C, Christiansen CF, Dissing TH, Terkelsen CJ, Christensen S, Kirkegaard H, Andersen LW. Effect of Intravenous or Intraosseous Calcium vs Saline on Return of Spontaneous Circulation in Adults With Out-of-Hospital Cardiac Arrest: A Randomized Clinical Trial. JAMA. 2021 Dec 14;326(22):2268-2276. doi: 10.1001/jama.2021.20929. PMID: 34847226; PMCID: PMC8634154.
  4. Yannopoulos D, Bartos J, Raveendran G, Walser E, Connett J, Murray TA, Collins G, Zhang L, Kalra R, Kosmopoulos M, John R, Shaffer A, Frascone RJ, Wesley K, Conterato M, Biros M, Tolar J, Aufderheide TP. Advanced reperfusion strategies for patients with out-of-hospital cardiac arrest and refractory ventricular fibrillation (ARREST): a phase 2, single centre, open-label, randomised controlled trial. Lancet. 2020 Dec 5;396(10265):1807-1816. doi: 10.1016/S0140-6736(20)32338-2. Epub 2020 Nov 13. PMID: 33197396; PMCID: PMC7856571.
  5. Suverein MM, Delnoij TSR, Lorusso R, Brandon Bravo Bruinsma GJ, Otterspoor L, Elzo Kraemer CV, Vlaar APJ, van der Heijden JJ, Scholten E, den Uil C, Jansen T, van den Bogaard B, Kuijpers M, Lam KY, Montero Cabezas JM, Driessen AHG, Rittersma SZH, Heijnen BG, Dos Reis Miranda D, Bleeker G, de Metz J, Hermanides RS, Lopez Matta J, Eberl S, Donker DW, van Thiel RJ, Akin S, van Meer O, Henriques J, Bokhoven KC, Mandigers L, Bunge JJH, Bol ME, Winkens B, Essers B, Weerwind PW, Maessen JG, van de Poll MCG. Early Extracorporeal CPR for Refractory Out-of-Hospital Cardiac Arrest. N Engl J Med. 2023 Jan 26;388(4):299-309. doi: 10.1056/NEJMoa2204511. PMID: 36720132.

42. Live from ATS 2023

We are thrilled today here at Pulm PEEPs to be coming to you live from the American Thoracic Society 2023 Annual Meeting. We are joined by three fantastic speakers, and ATS leaders to discuss the highlights and events of the conference, and to share some great learning points along the way. The episode is being released immediately after recording today, Monday 5/22/23, so if you’re at the conference now make sure to listen for some extremely timely recommendations. If you’re not here in DC, we’ve highlighted some learning points that you can take away and some wisdom on how to maximize your conference experience!

We will be coming out with another episode tomorrow highlighting one of the ATS sessions, and we will continue to review symposiums from the conference throughout the year with ATS members and organizers.

Meet The Guests

Debra Boyer is a Pediatric Pulmonologist at Nationwide Children’s Hospital and has devoted much of her career to her patients and numerous trainees. Debra has been extensively involved with ATS as a prior chair of the Education Committee, and the Assembly on Pediatrics Planning Committee, and is currently on the Board of Directors and current Co-Chair of the ATS International Conference Committee.

Molly Hayes is an Assistant Professor of Medicine at Beth Israel Deaconess Medical Center and Harvard Medical School, the Director of the MICU at BIDMC, and the Director of External Education at the Carl J Shapiro Institute for Education and Research. She is also a course director for a yearly CME course on principles of critical care medicine run by BIDMC and HMS. Molly has been extensively involved with ATS as well and has served on the Education Committee, currently as a vice chair, chairs the Core Curriculum for the conference, is the chair for the Subcommittee on Education in Critical Care for the Critical Care Assembly’s Programming Committee, and has been very involved in the Section of Medical Education for the last several years.

Julianna Ferreria is an Associate Professor of Pulmonary and Critical Care Medicine at the University of Sao Paulo Medical School in Brazil, and her work focuses on mechanical ventilation. She has made a significant impact on global health initiatives and serves as the Co-Director of the MECOR program in Latin America for ATS. Julianna has also been extremely involved with ATS Critical Care Assembly Program Committee and was just awarded the Inaugural Philip Hopewell Prize for her commitment to research in low and middle-income countries.

41. Portopulmonary Hypertension and Hepatopulmonary Syndrome

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

Radiology Rounds – 5/9/2023

A woman in her 30s with no past medical history presents with acute dyspnea, fever and a non-productive cough. She has no sick contacts or recent travel. She is not on home medications.. She smokes 1/2 cigarettes per day and and occasionally vapes.

She is found to have diffuse bilateral patchy ground-glass opacities with some interlobular septal thickening. She has escalating oxygen requirements and was initially started on broad-spectrum empiric antimicrobial therapies.

Her initial serum infectious work-up and RVP are negative. She denies any new occupational or home exposures. She does not appear to respond to antibiotics after 72 hours.

You start empiric steroids (prednisone 60 mg daily) given concern for acute eosinophilic pneumonia. Within 48 hours she is weaned off HFNC and was discharged home on D7 on room air with close pulmonary follow-up.

She was amenable to tobacco cessation therapies and was followed closely outpatient with tapering of her steroids over 4 weeks.

40. Pulm PEEPs and ATS RCMB Assembly: Short Telomeres and Interstitial Lung Disease

This week on Pulm PEEPs we are thrilled to share a collaboration with the American Thoracic Society Assembly on Respiratory Cell & Molecular Biology. We are joined by two expert members of the ATS RCMB Assembly who have done basic and translational research in respiratory biology and lung disease. We explore the topic of Short Telomeres and their role in lung disease. With the annual ATS Conference just around the corner, this is a great intro episode for everyone from aspiring researchers and clinical pulmonologists.

Meet The Guests

Mark Snyder is an Assistant Professor of Medicine at the University of Pittsburgh Medical Center, and a member of the Graduate Program in Microbiology and Immunology there. He does research on the role of the adaptive immune system’s role in chronic rejection after lung transplantation and has received both a Parker B Francis Foundation award and an NIH K23 grant for this work.

Jonathan Alder is an Assistant Professor of Medicine at the University of Pittsburgh. His research focuses on telomeres and their role in human health and disease. He is an accomplished researcher, was a Parker B Francis fellow, and now has an NIH RO1 studying Telomere-mediated Lung disease.

Further Reading and References

Alder JK, Armanios M. Telomere-mediated lung disease. Physiol Rev. 2022 Oct 1;102(4):1703-1720. doi: 10.1152/physrev.00046.2021. Epub 2022 May 9. PMID: 35532056; PMCID: PMC9306791.

Alder JK, Chen JJ, Lancaster L, Danoff S, Su SC, Cogan JD, Vulto I, Xie M, Qi X, Tuder RM, Phillips JA 3rd, Lansdorp PM, Loyd JE, Armanios MY. Short telomeres are a risk factor for idiopathic pulmonary fibrosis. Proc Natl Acad Sci U S A. 2008 Sep 2;105(35):13051-6. doi: 10.1073/pnas.0804280105. Epub 2008 Aug 27. PMID: 18753630; PMCID: PMC2529100.

Armanios MY, Chen JJ, Cogan JD, Alder JK, Ingersoll RG, Markin C, Lawson WE, Xie M, Vulto I, Phillips JA 3rd, Lansdorp PM, Greider CW, Loyd JE. Telomerase mutations in families with idiopathic pulmonary fibrosis. N Engl J Med. 2007 Mar 29;356(13):1317-26. doi: 10.1056/NEJMoa066157. PMID: 17392301.

Radiology Rounds – 4/25/2023

Today’s #RadiologyRounds is a reminder that pulmonary care extends beyond the lungs! Although we’ll have a CT chest too

A 70-year-old woman with a diagnosis of seronegative RA presents with progressive dyspnea. PFTs show a moderate restrictive defect and moderately reduced DLCO. Here is the CT scan:

The CT was interpreted as indeterminate for UIP / fibrosing NSIP. The basilar and subpleural fibrosis and honeycombing are consistent with UIP, but with reticular nodular infiltrates and pleural effusions that are less consistent. In reviewing her history: RF, CCP, and anti-SCL70 were all negative. On hand exam, she had MCP enlargement, some PIP synovitis, and slight R hand ulnar deviation. She did not have clear telangiectasias. She had a history of GERD. She denied Raynauds. She had significant skin thickening over her legs and limited ankle mobility. X-rays were obtained:

This showed severe dystrophic calcifications and no bony erosions. This degree of extensive dystrophic calcifications is consistent with scleroderma and the patient was felt to have limited scleroderma, and possibly an overlap syndrome

The patient was started on MMF as the initial agent based on the Scleroderma Lung Study II (SLSII): https://pubmed.ncbi.nlm.nih.gov/27469583/ Glucocorticoids were avoided given the risk of scleroderma renal crisis with steroids. An anti-fibrotic was also considered but not started as 1st line.