NEW YORK (Reuters Health) – New guidelines will help reduce the number of patients with suspected idiopathic pulmonary fibrosis (IPF) who must undergo a lung biopsy for definitive diagnosis.
“Since the publication of the most recent guidelines in 2011, we’ve learned a lot about the diagnosis of IPF,” Dr. David A. Lynch of National Jewish Health in Denver told Reuters Health in a telephone interview. “We saw an opportunity to widen the number of cases that could get a diagnosis of IPF without surgical lung biopsy.”
Dr. Lynch is current president of the Fleischner Society, the group of international experts on pulmonary health who authored a white paper on IPF diagnosis published online November 15 in The Lancet Respiratory Medicine.
Patients with IPF are typically 60 or older and male, with a history of cigarette smoking. IPF is becoming increasingly common, Dr. Lynch noted. While the five-year mortality rate is more than 50%, he added, “the exciting thing is that there are now two treatments which can slow the progression of this disease.”
Previously, the presence of honeycombing on a patient’s CT scan was needed to definitively diagnose IPF without a lung biopsy. The Fleischner white paper now includes a category of probable usual interstitial pneumonia (UIP, the histologic correlate of IPF), based on CT results, that allows for a confident diagnosis of IPF in an appropriate clinical context.
In addition, the new guidelines permit the diagnosis of IPF in cases with CT features that would previously have been regarded as inconsistent with IPF.
Dr. Lynch estimates that the new guidelines will reduce by 20% the number of IPF patients who require lung biopsy for diagnosis.
The white-paper authors also provide a clinical checklist for evaluating patients with suspected IPF, including structured questions. “We emphasize the importance of a multidisciplinary approach with dialogue between clinicians, radiologists and pathologists – because that’s been shown in several contexts to reduce the observer variation in making this diagnosis,” Dr. Lynch said.
Future research should address observer variation and the relationship between CT patterns and IPF progression and mortality, he added, while raising awareness of IPF is also important.
“In several studies there have been substantial delays in making this diagnosis, on the order of one to two years,” Dr. Lynch said. “To have increased awareness of the diagnosis and therefore earlier diagnosis would be very helpful.”
Dr. Demosthenes Bouros, of National and Kapodistrian University of Athens in Greece, and coauthor of an accompanying commentary, told Reuters Health by email, “One of the problems we face today is the correct and early diagnosis of idiopathic pulmonary disease, which is a chronic progressive fibrotic lung disease with dismal prognosis. To this end, the Fleischner Society diagnostic criteria help the practicing clinician to adopt pretest probability and use better categorization based on the mounting new information regarding the radiologic features.”
Dr. Bouros believes that the new diagnostic criteria can help clinicians avoid unnecessary lung biopsies in IPF patients, start antifibrotic treatment earlier, and facilitate investigation of IPF phenotypes.
SOURCES: http://bit.ly/2k7o5c7 and http://bit.ly/2AFsgTq
Lancet Respir Med 2017.
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