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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| IPAF patients | IPAF definition according to 2015 ERS/ATS criteria |
| |
| non-IPAF patients |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Follow-up | Other | Clinical data, radiological data and laboratory tests follow-up |
|
| Measure | Description | Time Frame |
|---|---|---|
| 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 |
| Measure | Description | Time Frame |
|---|---|---|
| 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 |
| IPAF serological domain criteria |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with idiopathic interstitial pneumonia classifiable or not as IPAF
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Roland JAUSSAUD, Pr | Contact | 0383154067 | r.jaussaud@chru-nancy.fr | |
| Paul DECKER, MR | Contact | p.decker@chru-nancy.fr |
| Name | Affiliation | Role |
|---|---|---|
| Roland JAUSSAUD, Pr | Central Hospital, Nancy, France | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Central Hospital | Lille | France | ||||
| Central Hospital |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 17329309 | Background | Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007 Jul;66(7):940-4. doi: 10.1136/ard.2006.066068. Epub 2007 Feb 28. | |
| 14962966 | Background | 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. doi: 10.1136/ard.2003.006122. |
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| ID | Term |
|---|---|
| D054988 | Idiopathic Interstitial Pneumonias |
| D003240 | Connective Tissue Diseases |
| D017563 | Lung Diseases, Interstitial |
| ID | Term |
|---|---|
| D054990 | Idiopathic Pulmonary Fibrosis |
| D011658 | Pulmonary Fibrosis |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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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 |
| IPAF morphological domain criteria | NSIP, and/or OP, or LIP radiology pattern by HRCT | Baseline |
| ILD severity | PFT (pulmonary function test): FVC, FEV1, DLCO (percentages of predicted values) | Baseline, 6 months of follow-up and at the last visit |
| Survival rate | After 3 years and 5 years of follow-up |
| Nancy |
| France |
|
| 15166329 | Background | 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. doi: 10.1148/radiol.2321030174. Epub 2004 May 27. |
| 10639536 | Background | Keane MP, Lynch JP 3rd. Pleuropulmonary manifestations of systemic lupus erythematosus. Thorax. 2000 Feb;55(2):159-66. doi: 10.1136/thorax.55.2.159. No abstract available. |
| 17557770 | Background | 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. doi: 10.1136/thx.2006.069237. Epub 2007 Jun 8. |
| 17556720 | Background | 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. doi: 10.1164/rccm.200702-220OC. Epub 2007 Jun 7. |
| 21565966 | Background | 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. |
| 20682528 | Background | 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. |
| 26160873 | Background | 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. |
| D017437 | Skin and Connective Tissue Diseases |