Connective tissue disease-associated interstitial lung disease (CTD-ILD) Connective tissue disease-associated interstitial lung disease, Progressive pulmonary fibrosis, Rheumatoid arthritis, Systemic sclerosis, Idiopathic inflammatory myopathies, Sjogren's disease, Systemic lupus erythematosus, Mixed connective tissue disease, ANCA-associated vasculitis
Conditions
Interventions
1) Single intravenous administration of [18F]FAPI-74
2) Acquisition of [18F]FAPI-PET/CT images
3) Acquisition of chest HRCT images on the same day as [18F]FAPI-PET/CT
Sponsors
Sugita Toshiki
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Patients who meet all of the following criteria will be eligible for inclusion in this study. 1) Patients aged 18 years or older on the date of informed consent. 2) Patients who have been diagnosed with connective tissue disease (CTD) by the date of informed consent, based on the criteria listed below: rheumatoid arthritis, systemic sclerosis, idiopathic inflammatory myopathy, Sjogren's disease, systemic lupus erythematosus, mixed connective tissue disease, microscopic polyangiitis, or granulomatosis with polyangiitis. - Rheumatoid arthritis: 2010 ACR/EULAR classification criteria - Systemic sclerosis: 2013 ACR/EULAR classification criteria - Idiopathic inflammatory myopathy: 2017 EULAR/ACR classification criteria or Bohan and Peter diagnostic criteria; for clinically amyopathic dermatomyositis (CADM), the Sontheimer criteria (2002) or Gerami criteria (2006) - Sjogren's disease: 2016 ACR/EULAR classification criteria - Systemic lupus erythematosus: 2019 EULAR/ACR classification criteria or 1997 revised ACR classification criteria - Mixed connective tissue disease: 2019 revised diagnostic criteria for MCTD or Kasukawa criteria - Microscopic polyangiitis and granulomatosis with polyangiitis: 2022 ACR/EULAR classification criteria or 2012 Chapel Hill Consensus Conference nomenclature 3) Patients who have been diagnosed with interstitial lung disease associated with CTD (CTD-ILD) by the date of informed consent and who show radiological features of pulmonary fibrosis. 4) Patients with %FVC >=45% and %DLCO (hemoglobin-corrected) >=30%, as assessed by pulmonary function testing performed during eligibility assessment after informed consent or within 4 weeks before informed consent. 5) Patients who are able to attend study visits according to the study schedule. 6) Patients who, after receiving sufficient explanation regarding participation in this study, have adequately understood the study and provided written informed consent of their own free will. If a patient has the capacity to provide consent but is unable to sign the consent form by themselves, consent may be documented by a proxy signature.
Exclusion criteria
Exclusion criteria: Patients who meet any of the following criteria will be excluded from this study. 1) Patients who have experienced an acute event within 12 weeks before the date of informed consent that may cause transient changes in pulmonary function or lung imaging findings, such as pulmonary infection, acute exacerbation, pulmonary edema/exacerbation of heart failure, or pulmonary embolism. 2) Patients who, within 12 weeks prior to the date of informed consent, have received glucocorticoids (>=20 mg/day prednisolone equivalent) for at least 1 week for the treatment of ILD; or have newly initiated or increased the dose of immunosuppressive agents (including mycophenolate mofetil, azathioprine, calcineurin inhibitors, rituximab, cyclophosphamide, or other agents) or antifibrotic agents (such as nintedanib). 3) Patients for whom treatment intensification for progressive ILD has already been planned as of the date of informed consent. 4) Patients who have active respiratory infection on the eligibility assessment date. 5) Patients with a history of lung resection on the eligibility assessment date. 6) Patients with active malignancy on the eligibility assessment date. 7) Patients for whom PET/CT is difficult to perform due to difficulty maintaining body position, dementia, or other reasons. 8) Patients who are pregnant or breastfeeding on the eligibility assessment date. 9) Patients whom the principal investigator or sub-investigator considers inappropriate for participation in this study. 10) Patients with a history of severe hypersensitivity or anaphylaxis to [18F]FAPI-74 or its excipients, particularly ethanol.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| [FALCON cross sectional study] %DLCO (hemoglobin-corrected) [FALCON prospective cohort study] Occurrence of PPF during the observation period | — |
Secondary
| Measure | Time frame |
|---|---|
| [FALCON cross sectional study] - %FVC - Serum KL-6 - Serum biomarkers other than KL-6 - Patient-reported outcome measures (K-BILD, mMRC) - Disease activity in patients with rheumatoid arthritis (DAS28-CRP, hand radiography) - Total disease extent on HRCT (%) [FALCON prospective cohort study] - Occurrence of progressor (defined as meeting PPF criteria or treatment intensification for ILD) during the observation period - Occurrence of events meeting at least one of the three PPF domains during the observation period - Annualized change in %FVC and FVC during the observation period - Annualized change in %DLCO (hemoglobin-corrected) and DLCO (hemoglobin-corrected) during the observation period - Percent change in serum KL-6 during the observation period - Change in patient-reported outcome measures (K-BILD, mMRC) during the observation period | — |
Contacts
Public ContactToshiki Sugita
University of Tsukuba Hospital
Outcome results
None listed