MDD strategy is crucial for accurate ILD diagnoses
The field of interstitial lung diseases (ILD) is challenging, with more than 200 disorders as possible diagnoses for patients who present to clinicians with similar symptoms and chest x-ray findings. The multidisciplinary discussion (MDD) strategy is very important for attaining an accurate ILD diagnosis.
We have had routine, formal, multidisciplinary discussions at our center since 2008. My guesstimate is that at least a third of patients referred as having idiopathic pulmonary fibrosis or another form of ILD by pulmonologists had been given the wrong diagnosis. Frequently, this was because of incorrect impressions provided by local radiologists and/or pathologists along with the clinician’s own limited knowledge of ILD.
In my experience, some patients described their pulmonologists as becoming irate with them when they asked for a second opinion, and I have had to try to avoid confrontations with referring physicians when trying to explain why the referral diagnosis was inaccurate.
Challenges to instituting the multidisciplinary discussion approach include coverage by health plans for a second-opinion evaluation, the willingness of physicians (for example, pulmonologists) outside of academic referral centers to refer patients to a center capable of adequately conducting an MDD, and patients’ desire to undergo an evaluation at centers of excellence where an MDD can be performed.
One must have also adequate resources to perform a proper MDD. But even in centers that refer patients, pulmonologists should confer with their colleague radiologists – and pathologists when appropriate – to try to make the most accurate diagnosis. And they should continue to question their diagnosis at follow-up appointments, as new symptoms and findings may arise or additional crucial information can become available over time that can point to an alternative diagnosis.
Kenneth C. Meyer, MD, MS, served as medical director of the lung transplant program and head of ILD at the University of Wisconsin–Madison. He reported no relevant disclosures.
Second MDD may be helpful for CTD-related ILD
Accumulating evidence suggests that multidisciplinary committees play a central role in improving the diagnostic accuracy of complex medical conditions. Interstitial lung disease (ILD) encompasses a number of clinical entities and no single diagnostic test alone can discriminate among the various causes of ILD. Instead, these diagnoses are based on a constellation of signs and symptoms, and radiographic, pathologic, and laboratory studies.
In one of the largest studies to assess the impact of a multidisciplinary discussion (MDD) on the diagnosis of ILD, De Sadeleer and colleagues performed a retrospective, observational cohort study of 938 cases. After examining pre-MDD and post-MDD diagnoses over a 10-year period, the study found that in nearly half (42%) of patients with a pre-MDD diagnosis, the MDD altered the diagnosis. Furthermore, the MDD provided a definite diagnosis in 81% of all patients. Taken together, these findings suggest MDDs provide improved diagnostic discrimination for patients with ILD.
However, unanswered questions remain. First, it is unclear whether a single MDD is sufficient. The present study found that 20% of cases were unclassifiable after the MDD. A second MDD may be helpful, especially in patients with ILDs related to connective tissue disease (CTD). The rheumatic diseases most commonly associated with ILD (for example, systemic sclerosis, rheumatoid arthritis, myositis) often evolve at different rates, and not all of the signs and symptoms of these conditions may be present or apparent at the time of the ILD presentation. A second MDD discussion may be particularly helpful in patients presenting with a specific CTD-related autoantibody in the absence of clinical signs and symptoms of a CTD. Another unanswered question is whether MDDs actually improve clinically meaningful outcomes for patients, such as survival and quality of life. At our CTD-ILD Program at the University of California, Los Angeles, we have found that our MDD has augmented patient satisfaction with their care, and it has also improved our ability to identify patients who are eligible for specific clinical studies. Future research is needed to determine to assess the impact of MDD on a variety of patient-centered and practice/research-focused outcomes.
Elizabeth Volkmann, MD, is founder and codirector of the CTD-ILD Program at the University of California, Los Angeles. She disclosed serving as a consultant or as a member of an advisory board for Boehringer Ingelheim and Astellas Pharma. She has received grants from Boehringer Ingelheim, Merck Serono, and the Rheumatology Research Foundation.