A comprehensive assessment of environmental exposures and the medical history guides multidisciplinary discussion in interstitial lung disease

Abstract

Background: Multidisciplinary discussion (MDD) is widely recommended for patients with interstitial lung disease (ILD), but published primary data from MDD has been scarce, and factors influencing MDD other than chest computed tomography (CT) and lung histopathology interpretations have not been well-described.

Methods: Single institution MDD of 179 patients with ILD.

Results: MDD consensus clinical diagnoses included autoimmune-related ILD, chronic hypersensitivity pneumonitis, smoking-related ILD, idiopathic pulmonary fibrosis, medication-induced ILD, occupation-related ILD, unclassifiable ILD, and a few less common pulmonary disorders. In 168 of 179 patients, one or more environmental exposures or pertinent features of the medical history were identified, including recreational/avocational, residential, and occupational exposures, systemic autoimmune disease, malignancy, medication use, and family history. The MDD process demonstrated the importance of comprehensively assessing these exposures and features, beyond merely noting their presence, for rendering consensus clinical diagnoses. Precise, well-defined chest CT and lung histopathology interpretations were rendered at MDD, including usual interstitial pneumonia, nonspecific interstitial pneumonia, and organizing pneumonia, but these interpretations were associated with a variety of MDD consensus clinical diagnoses, demonstrating their nonspecific nature in many instances. In 77 patients in which MDD consensus diagnosis differed from referring diagnosis, assessment of environmental exposures and medical history was found retrospectively to be the most impactful factor.

Conclusions: A comprehensive assessment of environmental exposures and pertinent features of the medical history guided MDD. In addition to rendering consensus clinical diagnoses, MDD presented clinicians with opportunities to initiate environmental remediation, behavior modification, or medication alteration likely to benefit individual patients with ILD.

Keywords: Autoimmune; Hypersensitivity; Idiopathic pulmonary fibrosis; Interstitial lung disease; Multidisciplinary discussion; Occupation; Smoking.

Figure 1.

Representative images demonstrating the association of environmental exposures and medical history assessment with radiologic and histopathologic interpretations. (A) Axial chest CT image demonstrating peripheral reticulation, traction bronchiectasis (more apparent on coronal reformatting) and honeycombing in a basilar-predominant distribution; MDD chest CT interpretation was typical UIP. The pleural-based opacities seen in the periphery of the right hemithorax are consistent with pleural fat on mediastinal windowing, a finding often observed with pulmonary fibrosis. Medical history was pertinent for clinical and laboratory findings consistent with antisynthetase syndrome, including the serologic presence of myositis-specific antibodies anti-NXP-2 and anti-MDA5. MDD consensus clinical diagnosis was autoimmune-related ILD. (B) Coronal CT image demonstrating widespread traction bronchiectasis and lower lobe volume loss consistent with severe pulmonary fibrosis, but lacking the typical radiologic features and distribution of UIP, NSIP, or OP; MDD chest CT interpretation was unclassifiable fibrosis. Exposure history indicated many years as a machinist in the steel industry, with longterm exposure to stone grinding wheels and hard-metals. MDD consensus clinical diagnosis was occupation-related ILD. (C) Low magnification SLB histopathology image demonstrating widespread thickening of interalveolar septae in a geographically homogenous pattern, and with high magnification (inset) demonstrating both cellular and fibrotic components; MDD histopathologic interpretation was NSIP. Exposure history indicated long-term avian exposures, and medical history indicated intermittent fever, myalgias, dyspnea, and serologic testing consistent with avian protein exposure, all consistent with HP, despite the lack of bronchiolocentric or granulomatous inflammation on SLB. MDD consensus clinical diagnosis was chronic HP. (D) Low magnification SLB histopathology image demonstrating pulmonary fibrosis, collapse of secondary pulmonary lobules, honeycombing, and fibroblastic foci (inset); MDD histopathologic interpretation was UIP. Medical history indicated treatment with infliximab, and her clinical course was most consistent with subacute and chronic lung injury as a result of infliximab therapy. MDD consensus clinical diagnosis was medication-induced ILD. Gray scale bars in lower left corners of panels C and D represent 3 mm and 2 mm, respectively.

Figure 2.

Venn diagram of 77 patients in which MDD consensus clinical diagnosis differed from referring clinical diagnosis, and demonstrating the relative contribution of individual factors (assessment of environmental exposures and pertinent features of the medical history, chest CT interpretation, and SLB interpretation) that were most impactful on MDD consensus clinical diagnoses. Of note, the overall size of the three circles reflects the numbers of patients enclosed within the circles. Assessment of environmental exposures and medical history had the greatest impact on MDD consensus clinical diagnoses in these 77 patients.