Others

Diagnostic Criteria

IIPs Diagnostic criteria

Patients with IPF and other IIPs will be classified according to the ATS/ERS/JRS/ALAT guidelines for diagnosis, management, and treatment of IPF in 2011 and 2015 and the ATS/ERS classification of IIPs in 2002 and 2013.

IPF

In cases with surgical lung biopsy, MDD is performed at each facility as much as possible. In accordance with the ATS/ERS/JRS/ALAT guidelines for IPF diagnosis in 2011, patients with Possible IPF, Probable IPF, and Definite IPF are diagnosed as having IPF.
In the case of no surgical lung biopsy, patients with a UIP honeycomb pattern in the lung are diagnosed as having IPF by CT diagnosis described in the ATS/ERS/JRS/ALAT guidelines for IPF diagnosis in 2011.
In the case of no surgical lung biopsy, patients with Possible UIP by CT findings are not diagnosed as IPF.

NSIP

In cases with surgical lung biopsy, MDD will be performed at each facility as much as possible.

RB-ILD

In symptomatic cases with surgical lung biopsy, MDD will be performed at each facility as much as possible.

DIP

In cases with surgical lung biopsy, MDD will be performed at each facility as much as possible.

COP

OP patients by a histopathological examination (surgical lung biopsy and transbronchial lung biopsy) are diagnosed with COP, excluding those with obvious causes such as infection, drugs, and radiation. MDD will be performed at each facility as much as possible.

AIP

In cases with diffuse alveolar disorder findings and no chronic fibrosing lesions in the background at the histopathological examination (surgical lung biopsy, transbronchial lung biopsy, or autopsy), MDD will be held as much as possible to diagnose AIP.

LIP

In cases with surgical lung biopsy, MDD will be performed at each facility as much as possible.

PPFE

In cases with surgical lung biopsy, MDD will be performed at each facility as much as possible.

Unclassifiable IIPs

The following cases are diagnosed as Unclassifiable IIPs.

  1. Causes of Unclassifiable IIPs include inadequate clinical, radiologic, or pathologic data.
  2. A major discordance between clinical, radiologic, and pathologic findings occurs, which happens in the following situations:
    1. Previous therapy resulting in substantial alteration of radiologic or histologic findings (e.g., biopsy of DIP after steroid therapy, which shows only residual nonspecific interstitial pneumonia).
    2. New entity, or unusual variant of recognized entity, not adequately characterized by the current ATS/ERS classification (e.g., variant of organizing pneumonia with supervening fibrosis).
    3. Multiple high-resolution CT and/or pathologic patterns that may be encountered in patients with IIPs

Central Committee

The central reviewers who are not affiliated with the physicians from the research facility will diagnose IIPs based on clinical, imaging, and histopathological findings.
Three central reviewers (one respiratory physician, one thoracic radiologist, and one pathologist) will constitute an MDD team, and two MDD teams will diagnose one case independently of each other.
If the diagnosis of the two MDD teams is different, the final diagnosis is decided by meeting with these teams. MDD of cases without pathological specimens is done by two specialists (except pathologists). The final diagnosis determined by the MDD team informs each facility of the result. Central review of diagnosis of IIPs is done twice by the MDD-at the time of registration and the last observation.
For acute exacerbation, clinical findings and imaging findings are used, and a central review is made by the pulmonologist and radiologist of the MDD team. In addition, the central reviewer cannot participate in a judgment targeting a case of its own facility.

Adverse Event Evaluation

In this study, adverse events (both severe and non-serious) of major anti-fibrotic drugs (nintedanib and pirfenidone) will be investigated, regardless of causality between drugs and adverse events. Medications for IIPs other than nintedanib and pirfenidone will only be studied for serious adverse events.

Ethical Approval

In principle, researchers shall obtain informed consent from all research subjects before conducting any epidemiological study, according to the local ethical guideline for clinical research by MHLW.

Conflict of Interest

JIPS registry is a doctor-initiated prospective observational study and is funded by Japan Boehringer Ingelheim Co., Ltd. To ensure that research results are not affected by the opinion of Japan Boehringer Ingelheim Co., JIPS registry is carried out to ensure transparency and reliability.
North East Japan Study Group, a specialized nonprofit corporation, will make decisions on the planning, implementation, and presentation of this research.

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