Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis

Abstract

BackgroundAcute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is associated with high mortality. However, few studies have so far reviewed analyses of autopsy findings in patients with AE-IPF.MethodsWe retrospectively reviewed 52 consecutive patients with AE-IPF who underwent autopsies at five university hospitals and one municipal hospital between 1999 and 2013. The following variables were abstracted from the medical records: demographic and clinical data, autopsy findings and complications during the clinical course until death.ResultsThe median age at autopsy was 71 years (range 47–86 years), and the subjects included 38 (73.1%) males. High-dose corticosteroid therapy was initiated in 45 (86.5%) patients after AE-IPF. The underling fibrotic lesion was classified as having the usual interstitial pneumonia (UIP) pattern in all cases. Furthermore, 41 (78.8%) patients had diffuse alveolar damage (DAD), 15 (28.8%) exhibited pulmonary hemorrhage, nine (17.3%) developed pulmonary thromboembolism and six (11.5%) were diagnosed with lung carcinoma. In addition, six (11.5%) patients developed pneumothorax prior to death and 26 (53.1%) developed diabetes that required insulin treatment after the administration of high-dose corticosteroid therapy. In addition, 15 (28.8%) patients presented with bronchopneumonia during their clinical course and/or until death, including fungal (seven, 13.5%), cytomegalovirus (six, 11.5%) and bacterial (five, 9.6%) infections.ConclusionsThe pathological findings in patients with AE-IPF represent not only DAD, but also a variety of pathological conditions. Therefore, making a diagnosis of AE-IPF is often difficult, and the use of cautious diagnostic approaches is required for appropriate treatment.

Prognostic evaluation by oxygenation with positive end‐expiratory pressure in acute exacerbation of idiopathic pulmonary fibrosis: A retrospective cohort study

“…12 PEEP is effective in the exudative phase of DAD because it prevents compression atelectasis secondary to severe pulmonary edema. These differences may be influenced by the stage of DAD, which is the most commonly described finding in AE-IPF.…”

Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report

“…The causes of ALI are extensive and include infection, aspiration, drugs, massive transfusion and surgery (20). A sizable body of literature now suggests many of these same conditions may lead to events that are indistinguishable from idiopathic acute exacerbations of IPF (6,10,13,(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34).…”

“…Assessment of post-mortem samples from patients who died from acute exacerbation of IPF has also identified evidence of occult respiratory infection in some (26,28) but not all (37) cohorts. Epidemiological support for an infectious etiology comes from studies demonstrating that acute exacerbation of IPF was significantly more common in the winter and spring months (5,29), and in patients who are taking immunosuppressive medications (5,6,38).…”

Acute exacerbation of IPF has systemic consequences with multiple organ injury, with SRA+ and TNF-α+ cells in the systemic circulation playing central roles in multiple organ injury

“…This finding is shared with the acute respiratory distress syndrome (ARDS) [10] which is considered to be a systemic multi-organ disease [11–13]. Nonetheless, the existing pathologic evidence base in IPF and CTD-associated ILD do not include an assessment of the peripheral blood or systemic organs in patients who have died of an acute exacerbation [5, 8, 9, 14]. …”