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Management of Advanced Pulmonary Sarcoidosis
The term “advanced sarcoidosis” is used for forms of sarcoidosis with a significant risk of loss of organ function or death. Advanced sarcoidosis often involves the lung and is described as “advanced pulmonary sarcoidosis” (APS), which includes advanced pulmonary fibrosis, associated complications such as bronchiectasis and infections, and pulmonary hypertension.
Although APS affects a small proportion of patients with sarcoidosis, it is the leading cause of poor outcomes, including death. Here we review the major patterns
of APS with a focus on the current management as well as potential approaches for improved outcomes for this most serious sarcoidosis phenotype.
Target Audience
Pulmonologists, critical care specialists, translational researchers, and clinicians
Learning Objectives
At the conclusion of this activity, learners should be able to:
- Identify the various forms of advanced pulmonary sarcoidosis.
- Describe the mechanisms leading to the development of the various forms of advanced pulmonary sarcoidosis.
- Discuss the general approach to the management of patients with various forms of advanced pulmonary sarcoidosis.
Accreditation Statement
The American Thoracic Society is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.
Disclosure Declaration
The current practice of the American Journal of Respiratory and Critical Care Medicine (AJRCCM) is to publish high quality, peer-reviewed and evidence-based original research, Concise Clinical Reviews, Guidelines and Consensus Statements. Articles published in AJRCCM include evidence-based summaries of optimal practice (Concise Clinical Reviews), evidence-based guidelines, workshop summaries and original contributions that will influence clinical practice. The publication of these articles by itself is only one piece of a multi-step process for the translation of evidence-based improvements in care to clinical practice. Articles selected for CME credit are designed to be the next step in the process of translating clinically relevant, evidence-based recommendations into clinical practice. This will be accomplished through a series of questions specifically designed by the author(s) to test that readers have the tools needed to translate recommendations for diagnostic and therapeutic clinical care into clinical practice. Members of the AJRCCM editorial board will review these questions and assess the quality of the questions based on:
- Clarity,
- Educational content, and
- The quality of the evidence supporting the response to the question. Posttest questions will assess if practitioners have understood the most important recommendations available for the diagnosis and treatment of pulmonary diseases, critical illness, and sleep disorders and are able to implement them into clinical practice.
Article Authorship Disclosures (as submitted to the ATS prior to article publication date)
Rohit Gupta, M.B.B.S. (Lewis Katz School of Medicine at Temple University, Philadelphia, PA, USA) reported no relevant financial relationships.
Marc A. Judson, M.D. (Albany Medical College, Albany, NY, USA) reported receiving consulting fees from Riovant Sciences and Star Therapeutics; and receiving grants or contracts paid to their institution from Mallinckrodt and the Foundation for Sarcoidosis Research.
Robert P. Baughman, M.D. (University of Cincinnati Medical Center, Cincinnati, OH, USA) reported receiving consulting fees from Mallinckrodt and Bellephron; receiving payments or honoraria from Mallinckrodt, Boehringer Ingelheim, and United Therapeutics; serving on a data safety monitoring board or advisory board for United Therapeutics; and receiving grants or contracts paid to their institution from Bayer, Genentech, aTyr, Mallinckrodt, Novartis, Celgene, Actelion, and Bellephron.
Off-Label Usage Disclosure
Methotrexate, Azathioprine, Leflunomide, Mycophenolate, TNF inhibitors (Infliximab, Adalimumab, Etanercept, and Golimumab), biosimilars, Rituximab, JAK inhibitors, and Tocilizumab as antiinflammatory agents in sarcoidosis; Pirfenidone and Nintedanib as antifibrotics in sarcoidosis; Epoprostenol/Treprostinil, Inhaled Iloprost, Bosentan, Ambrisentan, Sildenafil, Tadalafil, and Riociguat as pulmonary vasodilators (SAPH); and Roflumilast for sarcoidosis-associated bronchiectasis.
Disclosures of AJRCCM CME Planners
The current practice of the American Journal of Respiratory and Critical Care Medicine (AJRCCM) is to publish high quality, peer-reviewed and evidence-based original research, Concise Clinical Reviews, Guidelines and Consensus Statements. Articles published in AJRCCM include evidence-based summaries of optimal practice (Concise Clinical Reviews), evidence-based guidelines, workshop summaries and original contributions that will influence clinical practice. The publication of these articles by itself is only one piece of a multi-step process for the translation of evidence-based improvements in care to clinical practice. Articles selected for CME credit are designed to be the next step in the process of translating clinically relevant, evidence-based recommendations into clinical practice. This will be accomplished through a series of questions specifically designed by the author(s) to test that readers have the tools needed to translate recommendations for diagnostic and therapeutic clinical care into clinical practice. Members of the AJRCCM editorial board will review these questions and assess the quality of the questions based on (1) clarity, (2) educational content, and (3) the quality of the evidence supporting the response to the question. Posttest questions will assess if practitioners have understood the most important recommendations available for the diagnosis and treatment of pulmonary diseases, critical illness, and sleep disorders and are able to implement them into clinical practice.
AJRCCM CME Planners
Harold Collard, M.D.
Associate Editor, AJRCCM
Dr. Collard reported that he is on the advisory committee of Bayer, Biogen, Boehringer Ingelheim, Fibrogen, Genentech, Gilead, Intermune, and Promedior; and is a consultant at Five Prime.
Gerard J. Criner, M.D.
Associate Editor, AJRCCM
Dr. Criner reported that he has research awards from Aeris Therapeutics, AstraZeneca, Boehringer Ingelheim, Forest Laboratories, GlaxoSmithKline, Ikaria, Medimmune, Novartis, Pearl, PneumRx, Pulmonx, Respironics, Spectral Diagnostics, and Theratechnologies Inc.; and has received lecture fees from Almirall, AstraZeneca, Boehringer Ingelheim, Celerion, CSA Medical Inc., Johnson & Johnson, and Respivert.
Jadwiga A. Wedzicha, M.D.
Editor-in-Chief, AJRCCM
Dr. Wedzicha reported that she is on the advisory committee of Napp, Pfizer, and Takeda; has research awards from Chiesi, Takeda, and Vifor Pharma; and has received lecture fees from Almirall, Bayer, Boehringer Ingelheim, Novartis, and Takeda.
Instructions to Receive Credit
To receive credit for this journal article:
- Read the journal article. Keep track of how long it takes you to read it.
- Once you open the article, the Post-Test becomes available. After reading the article, answer the post-test questions. You must answer all questions correctly to earn credit. You may take the test as many times as you like.
- Once you pass the Post-Test, the Evaluation becomes available. Answer all the evaluation questions.
- Once you complete the evaluation, select the amount of credit to receive based on the time it took you to read the article.
- You can view, save and print your Certificate by pressing the Certificate button.
- To review the credits you've earned in this system and reprint certificates, go to the My Learning drop-down list. Then select Transcript.
Available Credit
- 1.00 AMA PRA Category 1 Credit(s)™The American Thoracic Society designates this for a maximum of 1.00 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
- 1.00 Participation