Perspectives on the Multidisciplinary Approach to Diagnosing Idiopathic Pulmonary Fibrosis

An Interview with Justin Oldham, MD, MS

An interview was conducted with Justin Oldham, MD, MS, on the topic of multidisciplinary discussions in the diagnosis of idiopathic pulmonary fibrosis (IPF). Dr Oldham is currently the director of the Interstitial Lung Disease program at the University of California – Davis Medical Center. He also manages the UC-Davis ILD patient registry and supervises the clinical and translational ILD research program, which includes overseeing ongoing ILD clinical trials at the institution. His research interests include translating ILD genetics research into clinical application and identifying genetic subgroups within various ILDs that would benefit from currently available therapies. Below are the highlights from the interview, which reflect Dr Oldham’s experience and opinions.

General Information about Multidisciplinary Discussion in IPF

What is a multidisciplinary discussion in the context of IPF?

Dr. Oldham: A multidisciplinary discussion, we call it an MDD, is an interaction between a pulmonologist, chest radiologist, pulmonary pathologist (when necessary), and preferably a rheumatologist when one is available, all of whom have expertise in interstitial lung diseases (ILDs).

How commonly is MDD used in diagnosing ILDs?

Dr. Oldham: The use of MDD is a hallmark for ILD centers throughout the country. It is also a recommended component of evaluating ILDs per the American Thoracic Society and European Respiratory Society Joint Consensus Guidelines.¹

How does using MDD improve diagnosis for patients with IPF?

Dr. Oldham: MDD is critical for diagnosing cases of IPF and other ILDs where the high-resolution computed tomography, or HRCT scans and clinical workup do not provide a clear diagnosis. It is recommended by the American Thoracic Society and European Respiratory Society Joint Consensus Statements1, and it has been shown to increase the diagnostic confidence in ILD. A group at University of Michigan, led by Kevin Flaherty and Fernando Martinez, have done great work in this area. They showed that using an MDD increases confidence in the ultimate ILD diagnosis.^2,3 They also showed that there is a fair amount of disagreement, even between specialists with ILD expertise, prior to instituting an MDD.

Does MDD speed up the diagnostic process or is it primarily used to increase diagnostic accuracy?

Dr. Oldham: It depends on when the MDD is being done. Unfortunately, an MDD is not done until a patient is referred for ILD evaluation, so whoever is referring the patient needs to have ILD on their radar before an MDD can occur. A study published by Drs. Collard and Schwartz showed that a large percentage of patients experienced long delays and were evaluated by three or more physicians before receiving the correct ILD diagnosis.⁴ As such, it is difficult to say that MDD changes the time it takes to make a specific ILD diagnosis because ILD needs to be suspected first. That said—after the diagnosis is suspected and the patient has been referred, MDD does help speed up the specific diagnosis in cases where the clinical and radiologic findings do not indicate a clear-cut etiology. MDD gets all the relevant parties together in one room (or on one conference call) to nail down what the highest confident diagnosis would be.

Is MDD also used to monitor disease progression?

Dr. Oldham: We do not use MDD to monitor disease progression. However, we will revisit individual cases who have unexpected disease trajectories to ensure that we are still confident in our MDD diagnosis and that it does not change based on any new information.

Please describe the protocol for conducting an MDD.

Dr. Oldham: MDDs can take several different forms. Some centers perform MDDs weekly and use them to assess the majority of the cases at their center. I have heard of others holding them monthly and focusing the discussion on only the most difficult cases. At UC-Davis, we hold a monthly comprehensive MDD for all patients who underwent surgical lung biopsy, which has all the pertinent specialists there. One of our three ILD pulmonologists will briefly present the case, including pertinent clinical history, serologic data, and pulmonary function testing. Then our chest radiologist will review the HRCT, and the pulmonary pathologist will review the lung biopsy slides. The group will then assign a diagnosis based on the discussion that takes places among these specialists. This diagnosis will guide subsequent interventions when the patient returns to clinic a few weeks later.

We also perform a more limited MDD on a weekly basis between myself and our thoracic radiologist, Dr. Michael Kadoch, for cases where the patient did not undergo a lung biopsy. In those situations, the diagnosis tends to be more straightforward or the resultant diagnosis is unclassifiable, at which time a surgical lung biopsy is recommended for patients who can safely undergo this intervention. I also like to incorporate rheumatologists on a case-by-case basis for patients for whom a diagnosis of connective tissue disease-related ILD has been made or for whom the diagnosis is suspected.

Personally, I think that all cases of ILD should undergo an MDD when possible. A possible exception is for those with an established connective tissue disease (CTD), as various CTDs are strongly associated with ILD. However, MDD should certainly be considered for patients with presumed CTD-associated ILD that continue to progress despite optimal CTD therapy, as several therapies used to treat CTD can independently cause ILD.

In an MDD, who drives the discussion?

Dr. Oldham: At my institution, I lead the MDDs. Generally, it tends to be the pulmonologist or a group of pulmonologists who lead the MDD.

Is the MDD team predesignated or is it ad hoc?

Dr. Oldham: It tends to be a predesignated team. There are a handful of pulmonologists with ILD expertise at the various institutions. Some institutions have more experts than others. And then typically there are one or two chest radiologists and one pulmonary pathologist.

Are the MDDs universally conducted like a Grand Rounds or are some of them conducted in a closed session?

Dr. Oldham: All of the MDDs I have sat in on - at three different institutions - are run similarly, in that they are well attended by the relevant ILD experts but are open to trainees, nurses, respiratory therapists, and anybody who has an interest in ILD, such as research coordinators. Because of sensitive information regarding the patient’s case, only hospital employees can attend at my institution given HIPAA rules.

Does having open meetings improve knowledge and awareness about the disease among the population of people working in pulmonology?

Dr. Oldham: Absolutely. The pulmonologists in my group who attend seem to come out of it with a better understanding of the individual ILDs themselves. Another advantage of open MDDs is that they help increase awareness of new or updated data that we bring up during our discussions. General pulmonologists who do not necessarily have ILD expertise certainly gain some educational value from these MDDs.

When these cases are reviewed at the meetings, are other advanced care professionals (eg, occupational therapists, specialist nurses, anyone involved in rehabilitation or direct care of the patient) included in the discussion?

Dr. Oldham: At times, yes. Our clinical coordinator, who is a respiratory therapist, is there for every MDD, as are our research assistants, who know the patients quite well. These individuals will join the discussion on occasion to provide important information or context. Additionally, our MDD is announced throughout the division, so other pulmonologists and trainees often attend.

Are there some case characteristics, signs, or symptoms that would negate the need for an MDD?

Dr. Oldham: I think that all patients referred to an ILD center should have their cases reviewed by MDD. This again stems from work by the Michigan group showing that diagnostic disagreements exist even among ILD specialists.^2,3 MDD is our chance to put our heads together and offer the highest confidence diagnosis possible, particularly for patients without characteristic features of a particular ILD. For example, I often see patients who are not referred for ILD evaluation because they have relatively preserved lung volumes despite clear-cut basilar crackles and honeycombing on HRCT. MDD is important for these types of individuals because many will have combined pulmonary fibrosis and emphysema, which may have been overlooked without the MDD process.

Do the pulmonologists have final say if there are conflicting viewpoints among the radiologists, pathologists, or any other specialists?

Dr. Oldham: I do not like to think that any one person has final say. The pulmonologist’s job is to consider the information provided by the radiologist and pathologist, taking into account the clinical history as well. More often than not, all relevant parties agree on the final diagnosis.

Once a consensus has been reached in the MDD, does the group discuss treatment options?

Dr. Oldham: After we have an agreement in the room on the diagnosis, we will discuss treatment options as well. This part of the conversation usually starts with, “This patient has been diagnosed with IPF. Next visit, we need to discuss antifibrotic therapy with them.” If it is a connective tissue disease-related ILD, “Next visit, we need to discuss immunosuppression.” For hypersensitivity pneumonitis (HP), “Let’s discuss prednisone and antigen removal,” that sort of thing. So, it is not necessarily a specific treatment or recommendation, but it provides a plan that the pulmonologist can take with them to the next ILD clinic visit.

Do you also discuss symptom management for these patients?

Dr. Oldham: We generally address symptom management on a case-by-case basis. Some patients have a serious treatment-refractory cough, so if that is one of the defining features of their disease and it significantly interferes with their life, we do discuss this during MDD. Other issues, such as oxygen therapy and pulmonary rehab are discussed with the patients in clinic, not necessarily during an MDD.

Impact of MDD on Diagnosing IPF

Once the patient is referred to an ILD center and is suspected of having an ILD, does MDD help create a more straightforward path to a diagnosis of IPF (or other ILD)?

Dr. Oldham: After ILD is suspected and the patient is referred appropriately, I do think that MDD improves diagnostic confidence. Although there is no gold standard for diagnosis, data show that MDD improves diagnostic confidence, which means that the closest thing we have to a gold standard is a group of medical experts sitting in a room and ultimately agreeing on a diagnosis.³

What diseases are patients most often misdiagnosed with before reaching a consensus on a diagnosis of IPF?

Dr. Oldham: Prior to being diagnosed with an ILD, most patients generally present with nonspecific symptoms: dyspnea on exertion, dry cough. A lot of them are diagnosed with asthma or bronchitis, emphysema, COPD, heart disease, that sort of thing. Once ILD is suspected, the substantial overlap in presentation of various ILDs opens up a whole new set of possible diagnoses. Personally, I find that the highest misdiagnosis rate within ILD occurs among patients with HP. A lot of them are initially diagnosed as IPF, but when you perform a careful exposure history, and surgical lung biopsy when possible, a fair number of patients have their diagnosis changed from IPF to HP.

Do you think that the use of MDD has lowered the rate of misdiagnosis?

Dr. Oldham: MDD does not really lower the rate of misdiagnosis, only because MDDs are only conducted once you suspect ILD in the first place and refer the patient to an ILD center for evaluation. A better way to reduce the rate of misdiagnosis would be to implement an effective early ILD detection program among patients being followed by primary care. To my knowledge, such a program does not currently exist, unfortunately.

Are there any drawbacks to using a multidisciplinary approach?

Dr. Oldham: No. If anything, I think MDD helps to resolve conflicting diagnoses and recommendations among specialists. As mentioned earlier, the Michigan group showed that there is a fair amount of disagreement in diagnosis between the specialists, even among ILD specialists, when cases are reviewed in isolation.^2,3 Putting these specialists in a room together leads to ultimate agreement among these various perspectives, resulting in improved diagnostic confidence.

Does MDD place any additional burden on the patient?

Dr. Oldham: Patients may wait for an MDD for a week or two, sometimes even a month, which could be frustrating. But, in my experience, the peace of mind that an MDD diagnosis brings for patients greatly outweighs the inconvenience of the short wait time. I think patients sleep a little easier when I tell them that we discussed their case in front of all of the ILD experts in my institution to come up with this diagnosis. Knowing that the best minds were put to the case can be reassuring.

Is there anything that could be done to make the process even more accurate or complete?

Dr. Oldham: Having internationally agreed-upon consensus statements for all ILDs would help because it would remove some of the subjectivity in ILD diagnosis, especially with regard to HP. If we were able to standardize things on a global scale, that would certainly help.

Expanding the Use of MDD

How often is MDD used in the community?

Dr. Oldham: I cannot speak for institutions outside my own, but among the community doctors that I have met with and talked to, it seems that MDD is not very common in the community, though through no fault of their own, as many do not have on-site chest radiologists or pulmonary pathologists. I know a lot of community doctors would love to have MDDs if they had access to the appropriate resources.

Do you think community healthcare professionals want to start MDDs at their own centers or otherwise get access to this tool?

Dr. Oldham: I think a fair number of community HCPs are interested in having access to those of us with ILD expertise, whether that is through a formal program or informal relationships. Several community institutions near my center here in California have sought to establish ties with our radiology department so that they can have access to a thoracic radiologist for their ILD cases.

Are there guidelines or recommendations to help institutions that want to start an MDD?

Dr. Oldham: The key components are a pulmonologist, a thoracic radiologist, and a pulmonary pathologist. If you have those components at your institution, the pulmonologist just needs to spearhead the project and recruit the appropriate personnel. If those resources are not available at an institution, it will be difficult to have an in-person MDD at that institution. However, based on my experience with community physicians in our area, I strongly suggest that ILD centers foster strong ties to community medical centers because that relationship gives community pulmonologists access to resources they may not otherwise have.

Concluding Remarks

Is there anything else you would like to add to this discussion of MDD?

Dr. Oldham: The MDD is the cornerstone of proper ILD evaluation and a hallmark of ILD centers throughout the country. It is important for as many patients to be referred to an ILD center as possible. This recommendation stems from the improved diagnostic confidence that MDD provides, along with improved outcomes that are associated with ILD center referral.⁵ While the reasons that ILD center referral is associated with better outcomes have not yet been delineated, we spend a lot of time focusing on implementing MDD for patients, along with focusing on comorbidity management, assessing for supplemental oxygen needs, prescribing pulmonary rehabilitation, and providing access to clinical trials - all these things play a role in improving outcomes for patients who are treated at ILD centers. I recognize that not all patients can be referred due to logistics, which leads back to why I advocate for strong ties between academic institutions and the community. These relationships can provide some of the resources that we have at ILD centers for those community physicians.

REFERENCES

1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
2. Martinez FJ, Chisholm A, Collard HR, et al. The diagnosis of idiopathic pulmonary fibrosis: current and future approaches. Lancet Respir Med. 2017;5(1):61-71.
3. Flaherty KR, King TE, Jr., Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? American journal of respiratory and critical care medicine. 2004;170(8):904-910.
4. Collard HR, Tino G, Noble PW, et al. Patient experiences with pulmonary fibrosis. Respiratory medicine. 2007;101(6):1350-1354.
5. Lamas DJ, Kawut SM, Bagiella E, Philip N, Arcasoy SM, Lederer DJ. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. American journal of respiratory and critical care medicine. 2011;184(7):842-847.

Justin Michael Oldham, M.D., M.S.
Assistant Professor
Director, Interstitial Lung Disease Program
University of California-Davis

Justin Oldham, MD, MS, is on faculty at the University of California-Davis in the department of Pulmonary and Critical Care Medicine, where he serves as the director of the Interstitial Lung Disease program. He also oversees the ILD patient registry and supervises the conduct of ILD clinical trials at his institution. Dr Oldham received his MD from University of Colorado School of Medicine in 2006, which he followed with a residency in Internal Medicine at UC Davis School of Medicine. He pursued a fellowship in Pulmonary and Critical Care Medicine at the University of Chicago, during which he also found time to do a Master’s in Biostatistics and Epidemiology. He is board certified in Internal Medicine as well as in the sub-specialty of Pulmonary and Critical Care Medicine.

Dr Oldham is interested in translating ILD genetics research into clinical application, with a focus on using pharmacogenetics to identify subgroups of patients that are most likely to benefit from particular ILD therapies. Some of his recent publications includes one that identified IPF subgroups genetically predisposed to a favorable response to anti-oxidant therapy, as well as a number of papers that describe characteristics of patients with IPF and their implications on survival and/or prognosis.