By: James Pitt  May. 30, 2018
Interstitial lung diseases (ILD) are more than 300 different conditions with similar symptoms. According to the European Respiratory Society, “Only about one in three cases of interstitial lung disease has a known cause.” Different ILDs present very different risks: “Survival rates at 5 years range from 20% for idiopathic pulmonary fibrosis [IPF] to almost 100% for cryptogenic organizing pneumonia.”
Diagnosis is difficult but important. Writing in Translation Respiratory Medicine, Dr. Keith C. Meyer of the University of Wisconsin Health notes that many ILDs respond well to anti-inflammatories. In other ILDs, diagnosis allows appropriate supportive care and detection and treatment of comorbidities, which can significantly benefit patients. Two therapies for IPF have become available since Dr. Meyer’s paper was published, nintedanib and pirfenidone. Both received FDA approval in October 2014. Secondary analyses found evidence that nintedanib reduces mortality, and that pirfenidone reduces respiratory hospitalizations and mortality.
IPF is typically diagnosed by identifying a pattern called “usual interstitial pneumonia.” Unfortunately, interobserver agreement on when a patient shows this pattern is low. One possible solution is genomic tests for RNA expression associated with IPF. A 2016 clinical utility analysis showed favorable results.
Following Dexur’s earlier analysis of ILD, analysts examined outcomes for ILD with major complications/ comorbidities (DRG 196) at four large hospitals. Florida Hospital Orlando had particularly good results, with the lowest mortality rate, 30-day all cause readmission rate, and average length of stay. Duke University Hospital (Durham, NC) had a higher mortality rate, but low 30-day readmissions and the average length of stay.
Intensive care admission practices varied widely. At Florida Hospital Orlando, 77% of Medicare-eligible DRG 196 inpatients had an ICU stay. At Johns Hopkins, only 42% did. There was no evidence of a link between ICU stay rates and mortality rate.
For interstitial lung disease overall and at DRG level, from 2013-2016, for patients with interstitial lung disease at Cleveland Clinic Main Campus (Cleveland, OH), Duke University Hospital (Durham, North Carolina), Florida Hospital Orlando (Orlando, Florida), Loyola University Medical Center (Maywood, Illinois), Massachusetts General Hospital (Massachusetts, Boston), Mclaren Regional Medical Center (Flint, Michigan), The Johns Hopkins Hospital (Baltimore, Maryland), UAB Hospital (Birmingham, Alabama), William P Clements Jr University Hospital (Dallas, Texas), Wilmington Hospital (Wilmington, Delaware), and Yale-New Haven Hospital (New Haven, Connecticut)