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

Idiopathic Interstitial Pneumonia Clinical Trial

— EVOLIPAF  

Official title:

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

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT04179058
• Other study ID #: 2019PI251
• Status: Not yet recruiting
• Start date: March 2020
• Est. completion date: September 2020

Study information

• Verified date: November 2019
• Source: Central Hospital, Nancy, France
• Contact: Roland JAUSSAUD, Pr
• Phone: 0383154067
• Email: r.jaussaud[@]chru-nancy.fr
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 300
• Est. completion date: September 2020
• Est. primary completion date: September 2020
• Accepts healthy volunteers: No
• Gender: All
• Age group: N/A and older

Eligibility

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)

Related Conditions & MeSH terms

• Connective Tissue Diseases
• Idiopathic Interstitial Pneumonia
• Idiopathic Interstitial Pneumonias
• Idiopathic Pulmonary Fibrosis
• Lung Diseases, Interstitial
• Pneumonia

Intervention

Other:
• Follow-up
Clinical data, radiological data and laboratory tests follow-up

Locations

Country	Name	City	State
France	Central Hospital	Lille
France	Central Hospital	Nancy

Sponsors (1)

Lead Sponsor	Collaborator
Central Hospital, Nancy, France

Country where clinical trial is conducted

France, 

References & Publications (9)

Fathi M, Dastmalchi M, Rasmussen E, Lundberg IE, Tornling G. Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis. Ann Rheum Dis. 2004 Mar;63(3):297-301. — View Citation

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. — View Citation

Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 2010 Aug;138(2):251-6. doi: 10.1378/chest.10-0194. — View Citation

Keane MP, Lynch JP 3rd. Pleuropulmonary manifestations of systemic lupus erythematosus. Thorax. 2000 Feb;55(2):159-66. Review. — View Citation

Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B, Jones KD, King TE Jr. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med. 2007 Oct 1;176(7):691-7. Epub 2007 Jun 7. — View Citation

Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007 Jul;66(7):940-4. Epub 2007 Feb 28. — View Citation

Tanaka N, Kim JS, Newell JD, Brown KK, Cool CD, Meehan R, Emoto T, Matsumoto T, Lynch DA. Rheumatoid arthritis-related lung diseases: CT findings. Radiology. 2004 Jul;232(1):81-91. Epub 2004 May 27. — View Citation

Tillie-Leblond I, Wislez M, Valeyre D, Crestani B, Rabbat A, Israel-Biet D, Humbert M, Couderc LJ, Wallaert B, Cadranel J. Interstitial lung disease and anti-Jo-1 antibodies: difference between acute and gradual onset. Thorax. 2008 Jan;63(1):53-9. Epub 2007 Jun 8. — View Citation

Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011 Nov;140(5):1292-1299. doi: 10.1378/chest.10-2662. Epub 2011 May 12. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	CTD incidence	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)	After 3 years of follow-up
Secondary	IPAF clinical domain criteria	"mechanic hands", Gottron's sign, distal digital tip ulceration, inflammatory arthritis or polyarticular joint stiffness > 60mn, telangiectasia, Raynaud's phenomenon, unexplained digital oedema	Baseline
Secondary	IPAF serological domain criteria	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	Baseline
Secondary	IPAF morphological domain criteria	NSIP, and/or OP, or LIP radiology pattern by HRCT	Baseline
Secondary	ILD severity	PFT (pulmonary function test): FVC, FEV1, DLCO (percentages of predicted values)	Baseline, 6 months of follow-up and at the last visit
Secondary	Survival rate		After 3 years and 5 years of follow-up