Abstract
Some patients with idiopathic pulmonary fibrosis (IPF) do not have honeycombing on high-resolution computed tomography (HRCT) at their initial evaluation. The clinical course and sequential changes in HRCT findings in these patients are not fully understood. We reviewed the cases of 43 patients with IPF without honeycombing on initial HRCT from institutions throughout Japan. All patients were diagnosed with IPF based on a surgical lung biopsy. Multidisciplinary discussions were held five times between 2011 and 2014, to exclude alternative etiologies. We evaluated the sequential changes in HRCT findings in 30 patients with IPF. We classified these 30 patients into three groups based on their HRCT patterns and clarified the clinical characteristics and prognosis among the groups. The patterns of all 30 patients on initial HRCT corresponded to a possible usual interstitial pneumonia (UIP) pattern which was described in the 2011 International Statement. On long-term follow-up (71.0±38.7 standard deviation [SD] months), honeycombing was seen in 16 patients (53%, the HoneyCo group); traction bronchiectasis or cysts without honeycombing was observed in 12 patients (40%, the NoHoneyCo group), and two patients showed no interval change (7%, the NoChange group) on HRCT. The mean survival periods of the HoneyCo and NoHoneyCo groups were 67.1 and 61.2 months, respectively (p = 0.76). There are some patients with IPF whose conditions chronically progress without honeycombing on HRCT. The appearance of honeycombing on HRCT during the follow-up might not be related to prognosis.
Highlights
In 2000, the American Thoracic Society (ATS) and European Respiratory Society (ERS) in collaboration with the American College of Chest Physicians published an international consensus statement on the diagnosis and management of idiopathic pulmonary fibrosis (IPF) [1], and since accumulated data and observations of the radiological patterns of IPF have contributed to new guidelines
But the imaging features otherwise meet the criteria for the usual interstitial pneumonia (UIP) pattern, the imaging features are regarded as representing a ‘possible UIP pattern,’ and the surgical lung biopsy pattern must be a UIP pattern or a probable UIP pattern to make a definitive diagnosis of IPF [8]
All 30 patients (22 men and eight women) were diagnosed with a possible UIP pattern on the initial high-resolution computed tomography (HRCT) scan. They were diagnosed with a UIP pattern or a probable UIP pattern based on the initial histopathological evaluation
Summary
In 2000, the American Thoracic Society (ATS) and European Respiratory Society (ERS) in collaboration with the American College of Chest Physicians published an international consensus statement on the diagnosis and management of idiopathic pulmonary fibrosis (IPF) [1], and since accumulated data and observations of the radiological patterns of IPF have contributed to new guidelines. According to the 2011 ATS/ERS/Japanese Respiratory Society (JRS)/Latin American Thoracic Association (ALAT) guideline, the diagnosis of IPF should be based on the findings obtained using the combination of high-resolution computed tomography (HRCT) and surgical lung biopsy with a formal multidisciplinary discussion among the treating pulmonologist, radiologist, and pathologist [8]. If the patient’s HRCT findings meet the criteria for the usual interstitial pneumonia (UIP) pattern— in which honeycombing is critical for making the diagnosis of the UIP pattern — a surgical lung biopsy is unnecessary. This IPF guideline placed great importance on honeycombing on HRCT.
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