Interstitial Pneumonia With Autoimmune Features: Evaluation of Connective Tissue Disease Incidence During Follow-up

• Conditions: Idiopathic Interstitial Pneumonia
• Interventions: Other: Follow-up

• Registration Number: NCT04179058
• Lead Sponsor: Central Hospital, Nancy, France

Brief Summary

Interstitial lung diseases (ILD) represent a frequent complication of connective tissue diseases (CTDs), especially systemic sclerosis, idiopathic inflammatory myopathies and rheumatoid arthritis. ILD can either occur during CTD course or be the first manifestation of CTDs. Therefore screening patients with ILD for CTD is crucial. In some cases, ILD are associated with clinical and/or serological autoimmune features but not classifiable for CTDs. Evolution of these forms to defined CTDs has never been study. Recently, the European Respiratory Society/American Thoracic Society experts proposed a new term, "interstitial pneumonia with autoimmune features" or IPAF, to describe these patients according to updated classification criteria. Aims of this study were to compare CTD occurence during follow-up between IPAF and non-IPAF patients in a idiopathic interstitial pneumonia cohort and to identify risk factors of CTD progression in IPAF patients at diagnosis.

Detailed Description

Not available

Study Timeline

• Status Verified: 2019-11
• Study First Submitted: 2019-11-18
• Study First Submitted QC: 2019-11-25
• Last Update Submitted: 2019-11-25
• Study First Posted: 2019-11-26
• Last Update Posted: 2019-11-26
• Study Start: 2020-03
• Primary Completion: 2020-09
• Study Completion: 2020-09

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 300

Inclusion Criteria

• Patients with a new diagnosis of ILD confirmed by two chest-HRCT 3 months apart
• Patients with a minimal follow-up duration of 3 years after ILD diagnosis

Exclusion Criteria

• Patients with a defined CTD at ILD diagnosis
• Patients with an other ILD etiology identified at diagnosis (i.e. sarcoidosis, hypersensitivity pneumonitis)

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
IPAF patients	Follow-up	IPAF definition according to 2015 ERS/ATS criteria
non-IPAF patients	Follow-up	-

Primary Outcome Measures

Name	Time	Method
CTD incidence	After 3 years of follow-up	CTD incidence according to classification criteria: rheumatoid arthritis (2010 ACR/EULAR criteria), systemic erythematosus lupus (2019 ACR/EULAR criteria), Sjögren syndrome (2016 ACR/EULAR criteria), systemic sclerosis (2013 ACR/EULAR criteria), idiopathic inflammatory myopathies (2017 ACR/EULAR criteria) and mixed connective tissue disease (modified Sharp criteria or Alarcon-Segovia criteria or Kasukawa criteria)

Secondary Outcome Measures

Name	Time	Method
IPAF serological domain criteria	Baseline	ANA titre and pattern, RF, anti-CCP, anti-dsDNA, anti-Ro, anti-La, anti-ribonucleoprotein, anti-Smith, anti-Scl70, anti-tRNA synthetase, anti-PM-Scl, anti-MDA5
IPAF clinical domain criteria	Baseline	"mechanic hands", Gottron's sign, distal digital tip ulceration, inflammatory arthritis or polyarticular joint stiffness \> 60mn, telangiectasia, Raynaud's phenomenon, unexplained digital oedema
IPAF morphological domain criteria	Baseline	NSIP, and/or OP, or LIP radiology pattern by HRCT
ILD severity	Baseline, 6 months of follow-up and at the last visit	PFT (pulmonary function test): FVC, FEV1, DLCO (percentages of predicted values)
Survival rate	After 3 years and 5 years of follow-up

Trial Locations

Locations (1)

Central Hospital; 🇫🇷 Nancy, France