Detection and Early Referral of Patients With Interstitial Lung Abnormalities

Abstract

on behalf of the ILA Study Group * BACKGROUND: Interstitial lung abnormalities (ILA) may represent undiagnosed early-stage or subclinical interstitial lung disease (ILD). ILAs are often observed incidentally in patients who subsequently develop clinically overt ILD. There is limited information on consensus definitions for, and the appropriate evaluation of, ILA. Early recognition of patients with ILD remains challenging, yet critically important. Expert consensus could inform early recognition and referral.RESEARCH QUESTION: Can consensus-based expert recommendations be identified to guide clinicians in the recognition, referral, and follow-up of patients with or at risk of developing early ILDs?STUDY DESIGN AND METHODS: Pulmonologists and radiologists with expertise in ILD participated in two iterative rounds of surveys. The surveys aimed to establish consensus regarding ILA reporting, identification of patients with ILA, and identification of populations that might benefit from screening for ILD. Recommended referral criteria and follow-up processes were also addressed. Threshold for consensus was defined a priori as $ 75% agreement or disagreement.RESULTS: Fifty-five experts were invited and 44 participated; consensus was reached on 39 of 85 questions. The following clinically important statements achieved consensus: honeycombing and traction bronchiectasis or bronchiolectasis indicate potentially progressive ILD; honeycombing detected during lung cancer screening should be reported as potentially significant (eg, with the Lung CT Screening Reporting and Data System Q10 "S-modifier" [which indicates clinically significant or potentially significant noncancer findings]), recommending referral to a pulmonologist in the radiology report; high-resolution CT imaging and full pulmonary function tests should be ordered if nondependent subpleural reticulation, traction bronchiectasis, honeycombing, centrilobular ground-glass nodules, or patchy ground-glass opacity are observed on CT imaging; patients with honeycombing or traction bronchiectasis should be referred to a pulmonologist irrespective of FVC and diffusion capacity values; and patients with systemic sclerosis should be screened with pulmonary function tests for early-stage ILD.INTERPRETATION: Guidance for identifying clinically relevant ILA, with subsequent referral and follow-up, was established. These results lay the foundation for developing practical guidance on managing patients with ILA.

Adrenomedullin Expression Is Associated With the Severity and Poor Prognosis of Interstitial Lung Disease in Dermatomyositis Patients

“…We found that PBMC adrenomedullin levels in DM patients with ILD associated with pulmonary function test results, higher adrenomedullin mRNA levels correlated with worse lung function in DM patients with ILD, which may help assess the severity of ILD ( 33 , 34 ). In addition, deterioration of lung function, especially forced vital capacity (FVC) and DLco, is known to indicate disease progression and to be corelated with prognosis in DM patients with ILD ( 34 ). Therefore, PBMC adrenomedullin levels can be indicative of disease severity and prognosis in these patients.…”

Resistin Expression Is Associated With Interstitial Lung Disease in Dermatomyositis

“…These metrics help to quantify the severity of ILD. Therefore, resistin mRNA levels are indicative of DM-ILD severity and disease activity ( 37 ).…”

Systemic sclerosis in Native Americans of the American Southwest

“…Right heart catheterization was undertaken only on patients with severe symptoms or abnormal echocardiographic signs that required confirmation. ILD was considered present according to a published algorithm based on radiographic abnormalities compatible with ILD on high‐resolution computed tomography or, if high‐resolution computed tomography was not available, by chest radiograph 24,25 . The presence of scleroderma renal crisis was obtained by chart review and patient history.…”