The Clinical Course of Interstitial Pneumonia With Autoimmune Features

NCT03950583 | Completed

• 1 other identifier: 6476
• Study Type: observational
• Target: 68
• Locations: 1 country
• Sites: 1

Brief Summary

Interstitial pneumonia with autoimmune features (IPAF) is a chronic interstitial lung disease (ILD) with some laboratoristic and/or clinical features of autoimmune disease, but without meeting criteria of connective tissue disease (CTD), and with no other causes of ILD. Despite recent efforts to standardize the diagnosis of IPAF, subjects with IPAF represent a very heterogenous group of patients, whose prognosis and clinical course are far from being clear. It also remains to be clarified what proportion of IPAF patients develop clear features of CTD over time. The aim of this observational trial is to define the clinical course of patients newly diagnosed with IPAF by prospectively following them for a period of 3 years, at 6-month intervals. The primary outcome will be 3-year lung transplant-free survival. The secondary outcome will be the proportion of patients who develop clinical and laboratoristic features clearly meeting criteria for a diagnosis of CTD at 3 years from the time of diagnosis of IPAF. Predictors of survival will also be studied.

Conditions

Interstitial Pneumonia

Keywords

interstitial lung disease; interstitial pneumonia; connective tissue disease; clinical course

Outcome Measures

Primary Outcomes (1)

• Survival

  Survival rate since the time of diagnosis

  3 years

Secondary Outcomes (3)

• Rate of development of autoimmune disease

  3 years

• Clinical progression

  3 years

• Acute exacerbation

  3 years

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with a new diagnosis of interstitial pneumonia with autoimmune features, according to ERS/ATS A,B,C criteria

You may qualify if:

• new diagnosis of IPAF based on the A,B,C) ERS/ATS criteria (Eur Respir J 2015;46:976-87)

You may not qualify if:

• Interstitial lung disease other than IPAF
• Not a new diagnosis of IPAF

Sponsors & Collaborators

• London Health Sciences Centre Research Institute OR Lawson Research Institute of St. Joseph's: lead
• Western University, Canada: collaborator

Study Sites (1)

London Health Science Centre

London, Ontario, N6A 5W9, Canada

Location

Related Publications (6)

• Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, Lee JS, Leslie KO, Lynch DA, Matteson EL, Mosca M, Noth I, Richeldi L, Strek ME, Swigris JJ, Wells AU, West SG, Collard HR, Cottin V; "ERS/ATS Task Force on Undifferentiated Forms of CTD-ILD". An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015 Oct;46(4):976-87. doi: 10.1183/13993003.00150-2015. Epub 2015 Jul 9.

  PMID: 26160873 RESULT

• Oldham JM, Adegunsoye A, Valenzi E, Lee C, Witt L, Chen L, Husain AN, Montner S, Chung JH, Cottin V, Fischer A, Noth I, Vij R, Strek ME. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016 Jun;47(6):1767-75. doi: 10.1183/13993003.01565-2015. Epub 2016 Apr 21.

  PMID: 27103387 RESULT

• Ahmad K, Barba T, Gamondes D, Ginoux M, Khouatra C, Spagnolo P, Strek M, Thivolet-Bejui F, Traclet J, Cottin V. Interstitial pneumonia with autoimmune features: Clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. Respir Med. 2017 Feb;123:56-62. doi: 10.1016/j.rmed.2016.10.017. Epub 2016 Oct 31.

  PMID: 28137497 RESULT

• Kelly BT, Moua T. Overlap of interstitial pneumonia with autoimmune features with undifferentiated connective tissue disease and contribution of UIP to mortality. Respirology. 2018 Jun;23(6):600-605. doi: 10.1111/resp.13254. Epub 2018 Jan 10.

  PMID: 29320807 RESULT

• Collins BF, Spiekerman CF, Shaw MA, Ho LA, Hayes J, Spada CA, Stamato CM, Raghu G. Idiopathic Interstitial Pneumonia Associated With Autoantibodies: A Large Case Series Followed Over 1 Year. Chest. 2017 Jul;152(1):103-112. doi: 10.1016/j.chest.2017.03.004. Epub 2017 Mar 12.

  PMID: 28300570 RESULT

• Ferri C, Manfredi A, Sebastiani M, Colaci M, Giuggioli D, Vacchi C, Della Casa G, Cerri S, Torricelli P, Luppi F. Interstitial pneumonia with autoimmune features and undifferentiated connective tissue disease: Our interdisciplinary rheumatology-pneumology experience, and review of the literature. Autoimmun Rev. 2016 Jan;15(1):61-70. doi: 10.1016/j.autrev.2015.09.003. Epub 2015 Sep 16.

  PMID: 26384526 RESULT

MeSH Terms

Conditions

Lung Diseases, Interstitial; Connective Tissue Diseases; Disease Progression

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Skin and Connective Tissue Diseases; Disease Attributes; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Study Officials

• Marco Mura, MD, PhD

  Lawson Research Institute

  PRINCIPAL INVESTIGATOR

• Pari Basharat, MD

  Lawson Research Institute

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: May 13, 2019
• First Posted: May 15, 2019
• Study Start: March 11, 2019
• Primary Completion: October 31, 2024
• Study Completion: October 31, 2024
• Last Updated: March 10, 2025

Record last verified: 2025-03

Data Sharing

• IPD Sharing: Will not share

Locations

CA (1)