{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T00:07:55Z","timestamp":1792454875902,"version":"4.5.0"},"reference-count":151,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2016,2,1]],"date-time":"2016-02-01T00:00:00Z","timestamp":1454284800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","bprclinrheum.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Best Practice &amp; Research Clinical Rheumatology"],"published-print":{"date-parts":[[2016,2]]},"DOI":"10.1016\/j.berh.2016.03.002","type":"journal-article","created":{"date-parts":[[2016,4,12]],"date-time":"2016-04-12T21:52:02Z","timestamp":1460497922000},"page":"95-111","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":130,"title":["Mixed connective tissue disease"],"prefix":"10.1016","volume":"30","author":[{"given":"Ragnar","family":"Gunnarsson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siri Opsahl","family":"Hetlevik","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vibke","family":"Lilleby","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00d8yvind","family":"Molberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.berh.2016.03.002_bib1","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1016\/0002-9343(72)90064-2","article-title":"Mixed connective tissue disease \u2013 an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA)","volume":"52","author":"Sharp","year":"1972","journal-title":"Am J Med"},{"key":"10.1016\/j.berh.2016.03.002_bib2","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1016\/j.rdc.2005.04.007","article-title":"Does mixed connective tissue disease exist? Yes","volume":"31","author":"Aringer","year":"2005","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.berh.2016.03.002_bib3","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/j.rdc.2005.04.009","article-title":"Mixed connective tissue disease: still crazy after all these years","volume":"31","author":"Swanton","year":"2005","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.berh.2016.03.002_bib4","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1191\/0961203302lu220oa","article-title":"MCTD: a concept which stood the test of time","volume":"11","author":"Sharp","year":"2002","journal-title":"Lupus"},{"key":"10.1016\/j.berh.2016.03.002_bib5","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/0049-0172(80)90013-X","article-title":"Mixed connective tissue disease: a clinicopathologic study of 20 cases","volume":"10","author":"Bennett","year":"1980","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib6","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1097\/00005792-198007000-00001","article-title":"Mixed connective tissue disease: a subsequent evaluation of the original 25 patients","volume":"59","author":"Nimelstein","year":"1980","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.berh.2016.03.002_bib7","doi-asserted-by":"crossref","first-page":"1182","DOI":"10.1161\/01.CIR.68.6.1182","article-title":"Cardiovascular manifestations of mixed connective tissue disease in adults","volume":"68","author":"Alpert","year":"1983","journal-title":"Circulation"},{"key":"10.1016\/j.berh.2016.03.002_bib8","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1097\/00005792-198403000-00003","article-title":"A\u00a0prospective evaluation emphasizing pulmonary involvement in patients with mixed connective tissue disease","volume":"63","author":"Sullivan","year":"1984","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.berh.2016.03.002_bib9","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1093\/rheumatology\/31.12.811","article-title":"Clinical manifestations and anti-(U1)snRNP antibodies: a prospective study of 29 anti-RNP antibody positive patients","volume":"31","author":"Lundberg","year":"1992","journal-title":"Br J Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib10","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1002\/1529-0131(199905)42:5<899::AID-ANR8>3.0.CO;2-L","article-title":"Long-term outcome in mixed connective tissue disease: longitudinal clinical and serologic findings","volume":"42","author":"Burdt","year":"1999","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib11","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1016\/j.rdc.2005.04.005","article-title":"The prognosis of mixed connective tissue disease","volume":"31","author":"Lundberg","year":"2005","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.berh.2016.03.002_bib12","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1016\/j.rdc.2005.04.002","article-title":"Pediatric-onset mixed connective tissue disease","volume":"31","author":"Mier","year":"2005","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.berh.2016.03.002_bib13","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1136\/ard.2010.143792","article-title":"The prevalence and incidence of mixed connective tissue disease: a national multicentre survey of Norwegian patients","volume":"70","author":"Gunnarsson","year":"2011","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2016.03.002_bib14","first-page":"1088","article-title":"Clinical features of patients with juvenile onset mixed connective tissue disease: analysis of data collected in a nationwide collaborative study in Japan","volume":"23","author":"Kotajima","year":"1996","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib15","doi-asserted-by":"crossref","first-page":"1149","DOI":"10.1056\/NEJM197611182952101","article-title":"Association of antibodies to ribonucleoprotein and Sm antigens with mixed connective-tissue disease, systematic lupus erythematosus and other rheumatic diseases","volume":"295","author":"Sharp","year":"1976","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.berh.2016.03.002_bib16","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1016\/j.rdc.2005.04.006","article-title":"Raynaud's phenomenon in mixed connective tissue disease","volume":"31","author":"Grader-Beck","year":"2005","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.berh.2016.03.002_bib17","first-page":"1511","article-title":"Clinical course of patients with anti-RNP antibodies. A prospective study of 32 patients","volume":"18","author":"Lundberg","year":"1991","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib18","first-page":"499","article-title":"Severe pulmonary involvement in mixed connective tissue disease","volume":"124","author":"Wiener-Kronish","year":"1981","journal-title":"Am Rev Respir Dis"},{"key":"10.1016\/j.berh.2016.03.002_bib19","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1016\/S0025-6196(12)64786-7","article-title":"Intrathoracic manifestations in mixed connective tissue disease","volume":"60","author":"Prakash","year":"1985","journal-title":"Mayo Clin Proc"},{"key":"10.1016\/j.berh.2016.03.002_bib20","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1016\/S0272-5231(05)70113-1","article-title":"Respiratory complications in mixed connective tissue disease","volume":"19","author":"Prakash","year":"1998","journal-title":"Clin Chest Med"},{"key":"10.1016\/j.berh.2016.03.002_bib21","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1097\/00005382-200104000-00005","article-title":"Pulmonary involvement in mixed connective tissue disease: high-resolution CT findings in 41 patients","volume":"16","author":"Kozuka","year":"2001","journal-title":"J\u00a0Thorac Imaging"},{"key":"10.1016\/j.berh.2016.03.002_bib22","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1097\/00004728-200205000-00006","article-title":"Pulmonary involvement in mixed connective tissue disease: comparison with other collagen vascular diseases using high resolution CT","volume":"26","author":"Saito","year":"2002","journal-title":"J\u00a0Comput Assist Tomogr"},{"key":"10.1016\/j.berh.2016.03.002_bib23","doi-asserted-by":"crossref","first-page":"656","DOI":"10.1093\/rheumatology\/keh575","article-title":"Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD)","volume":"44","author":"Bodolay","year":"2005","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.berh.2016.03.002_bib24","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1016\/j.rmed.2008.12.018","article-title":"Esophageal involvement and interstitial lung disease in mixed connective tissue disease","volume":"103","author":"Fagundes","year":"2009","journal-title":"Respir Med"},{"key":"10.1016\/j.berh.2016.03.002_bib25","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1191\/0961203305lu2205oa","article-title":"Cardiac involvement in autoimmune myositis and mixed connective tissue disease","volume":"14","author":"Lundberg","year":"2005","journal-title":"Lupus"},{"key":"10.1016\/j.berh.2016.03.002_bib26","doi-asserted-by":"crossref","first-page":"2125","DOI":"10.1002\/art.21131","article-title":"The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community-based rheumatologists (the UNCOVER study)","volume":"52","author":"Wigley","year":"2005","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib27","series-title":"Mixed connective tissue disease and anti-nuclear antibodies","first-page":"23","article-title":"Diagnostic criteria for classification of MCTD","author":"Sharp","year":"1987"},{"key":"10.1016\/j.berh.2016.03.002_bib28","series-title":"Mixed connective tissue disease and anti-nuclear antibodies","first-page":"41","article-title":"Preliminary diagnostic criteria for classification of mixed connective tissue disease","author":"Kasukawa","year":"1987"},{"key":"10.1016\/j.berh.2016.03.002_bib29","series-title":"Mixed connective tissue disease and anti-nuclear antibodies","first-page":"33","article-title":"Classification and diagnostic criteria for mixed connective tissue disease","author":"Alarc\u00f3n-Segovia","year":"1987"},{"key":"10.1016\/j.berh.2016.03.002_bib30","series-title":"Les maladies syst\u00e9miques","first-page":"545","article-title":"Syndrome de Sharp","author":"Kahn","year":"1991"},{"key":"10.1016\/j.berh.2016.03.002_bib31","first-page":"1968","article-title":"Pediatric rheumatology clinic populations in the United States: results of a 3 year survey. Pediatric Rheumatology Database Research Group","volume":"23","author":"Bowyer","year":"1996","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib32","series-title":"Textbook of pediatric rheumatology","first-page":"418","article-title":"Mixed connective tissue disease and undifferentiated connective tissue disease","author":"Pepmueller","year":"2016"},{"key":"10.1016\/j.berh.2016.03.002_bib33","doi-asserted-by":"crossref","first-page":"1966","DOI":"10.1136\/annrheumdis-2011-201253","article-title":"Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study","volume":"71","author":"Gunnarsson","year":"2012","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2016.03.002_bib34","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1016\/j.rdc.2005.04.004","article-title":"Autoantibodies in the pathogenesis of mixed connective tissue disease","volume":"31","author":"Greidinger","year":"2005","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.berh.2016.03.002_bib35","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/j.clim.2008.03.461","article-title":"Immune pathogenesis of mixed connective tissue disease: a short analytical review","volume":"128","author":"Hoffman","year":"2008","journal-title":"Clin Immunol"},{"key":"10.1016\/j.berh.2016.03.002_bib36","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1111\/j.1365-2249.1990.tb05290.x","article-title":"Recombinant 70-kD protein used for determination of autoantigenic epitopes recognized by anti-RNP sera","volume":"81","author":"Nyman","year":"1990","journal-title":"Clin Exp Immunol"},{"key":"10.1016\/j.berh.2016.03.002_bib37","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1093\/rheumatology\/keu310","article-title":"The HLA profiles of mixed connective tissue disease differ distinctly from the profiles of clinically related connective tissue diseases","volume":"54","author":"Flam","year":"2015","journal-title":"Rheumatology (Oxford, England)"},{"key":"10.1016\/j.berh.2016.03.002_bib38","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1002\/art.1780310119","article-title":"HLA and immunoglobulin allotypes in mixed connective tissue disease","volume":"31","author":"Black","year":"1988","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib39","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1136\/ard.51.1.52","article-title":"Differences in HLA antigens between patients with mixed connective tissue disease and systemic lupus erythematosus","volume":"51","author":"Ruuska","year":"1992","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2016.03.002_bib40","doi-asserted-by":"crossref","first-page":"938","DOI":"10.1002\/art.1780390610","article-title":"Clinical correlations with HLA type in Japanese patients with connective tissue disease and anti-U1 small nuclear RNP antibodies","volume":"39","author":"Kuwana","year":"1996","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib41","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1111\/tan.12698","article-title":"Association of HLA-DRB1 alleles with susceptibility to mixed connective tissue disease in Polish patients","volume":"87","author":"Paradowska-Gorycka","year":"2016","journal-title":"HLA"},{"key":"10.1016\/j.berh.2016.03.002_bib42","doi-asserted-by":"crossref","first-page":"1786","DOI":"10.1093\/rheumatology\/kem265","article-title":"HLA class II haplotype and autoantibody associations in children with juvenile dermatomyositis and juvenile dermatomyositis\u2013scleroderma overlap","volume":"46","author":"Wedderburn","year":"2007","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.berh.2016.03.002_bib43","doi-asserted-by":"crossref","first-page":"1837","DOI":"10.1002\/art.1780381218","article-title":"Analysis of anti-u1 RNA antibodies in patients with connective tissue disease","volume":"38","author":"Hoffman","year":"1995","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib44","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1136\/ard.46.6.457","article-title":"HLA-DR antigens in systemic lupus erythematosus: association with specificity of autoantibody responses to nuclear antigens","volume":"46","author":"Smolen","year":"1987","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2016.03.002_bib45","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1002\/art.1780360117","article-title":"Contrasting molecular patterns of MHC class ii alleles associated with the anti-Sm and anti-RNP precipitin autoantibodies in systemic lupus erythematosus","volume":"36","author":"Olsen","year":"1993","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib46","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1111\/j.0105-2896.2009.00863.x","article-title":"The U1-snRNP complex: structural properties relating to autoimmune pathogenesis in rheumatic diseases","volume":"233","author":"Kattah","year":"2010","journal-title":"Immunol Rev"},{"key":"10.1016\/j.berh.2016.03.002_bib47","doi-asserted-by":"crossref","first-page":"1001","DOI":"10.1056\/NEJMcp013013","article-title":"Clinical practice. Raynaud's phenomenon","volume":"347","author":"Wigley","year":"2002","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.berh.2016.03.002_bib48","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1002\/art.1780230208","article-title":"Diagnostic potential of in\u00a0vivo capillary microscopy in scleroderma and related disorders","volume":"23","author":"Maricq","year":"1980","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib49","first-page":"1","article-title":"Scleroderma pattern of nailfold capillary changes as predictive value for the development of a connective tissue disease: a follow-up study of 3,029 patients with primary Raynaud's phenomenon","author":"Pavlov-Dolijanovic","year":"2011","journal-title":"Rheumatol Int"},{"key":"10.1016\/j.berh.2016.03.002_bib50","doi-asserted-by":"crossref","first-page":"1091","DOI":"10.1093\/rheumatology\/keq430","article-title":"Association between nail-fold capillary findings and disease activity in dermatomyositis","volume":"50","author":"Mugii","year":"2011","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.berh.2016.03.002_bib51","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1002\/art.1780310116","article-title":"Nailfold capillary microscopy in polymyositis and dermatomyositis","volume":"31","author":"Ganczarczyk","year":"1988","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib52","series-title":"EULAR Berlin","article-title":"Clinical course and nailfold capillaroscopy patterns of 69 patients with mixed connective tissue disease (MCTD)","author":"Olesinska","year":"2012"},{"key":"10.1016\/j.berh.2016.03.002_bib53","doi-asserted-by":"crossref","first-page":"1134","DOI":"10.3899\/jrheum.121272","article-title":"Clinical course, prognosis, and causes of death in mixed connective tissue disease","volume":"40","author":"Hajas","year":"2013","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib54","doi-asserted-by":"crossref","first-page":"1037","DOI":"10.1016\/j.berh.2007.10.002","article-title":"Mixed connective tissue disease: what is behind the curtain?","volume":"21","author":"Aringer","year":"2007","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib55","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/0049-0172(86)90047-8","article-title":"Renal involvement in mixed connective tissue disease: a longitudinal clinicopathologic study","volume":"16","author":"Kitridou","year":"1986","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib56","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1016\/j.rdc.2005.04.003","article-title":"Muscle involvement in mixed connective tissue disease","volume":"31","author":"Hall","year":"2005","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.berh.2016.03.002_bib57","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/0049-0172(92)90006-Y","article-title":"A\u00a0clinical, serological, and histopathological study of myositis patients with and without anti-RNP antibodies","volume":"22","author":"Lundberg","year":"1992","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib58","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1136\/ard.60.2.116","article-title":"Autoantibody profiles in the sera of European patients with myositis","volume":"60","author":"Brouwer","year":"2001","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2016.03.002_bib59","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1016\/j.rdc.2005.04.011","article-title":"Other manifestations of mixed connective tissue disease","volume":"31","author":"Pope","year":"2005","journal-title":"Rheum Dis Clin North Am"},{"key":"10.1016\/j.berh.2016.03.002_bib60","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1148\/124.3.613","article-title":"Mixed connective tissue disease: the spectrum of radiographic manifestations","volume":"124","author":"Udoff","year":"1977","journal-title":"Radiology"},{"key":"10.1016\/j.berh.2016.03.002_bib61","doi-asserted-by":"crossref","first-page":"1453","DOI":"10.2214\/ajr.183.5.1831453","article-title":"MRI of large intraosseous lesions in patients with inflammatory arthritis","volume":"183","author":"Tehranzadeh","year":"2004","journal-title":"AJR Am J Roentgenol"},{"key":"10.1016\/j.berh.2016.03.002_bib62","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1080\/02841850902787677","article-title":"Chest abnormalities in juvenile-onset mixed connective tissue disease: assessment with high-resolution computed tomography and pulmonary function tests","volume":"50","author":"Aalokken","year":"2009","journal-title":"Acta Radiol"},{"key":"10.1016\/j.berh.2016.03.002_bib63","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1097\/00005382-199203000-00007","article-title":"Lungs in mixed connective tissue disease","volume":"7","author":"Prakash","year":"1992","journal-title":"J\u00a0Thorac Imaging"},{"key":"10.1016\/j.berh.2016.03.002_bib64","first-page":"234","article-title":"Pulmonary involvement in long-term mixed connective tissue disease: functional trends and image findings after 10 years","volume":"33","author":"Kawano-Dourado","year":"2015","journal-title":"Clin Exp Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib65","doi-asserted-by":"crossref","first-page":"2412","DOI":"10.1002\/art.38029","article-title":"Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: a systematic review and meta-analysis","volume":"65","author":"Lef\u00e8vre","year":"2013","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib66","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","volume":"37","author":"Galie","year":"2016","journal-title":"Eur Heart J"},{"key":"10.1016\/j.berh.2016.03.002_bib67","doi-asserted-by":"crossref","first-page":"D34","DOI":"10.1016\/j.jacc.2013.10.029","article-title":"Updated clinical classification of pulmonary hypertension","volume":"62","author":"Simonneau","year":"2013","journal-title":"J\u00a0Am Coll Cardiol"},{"key":"10.1016\/j.berh.2016.03.002_bib68","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1164\/rccm.200806-953OC","article-title":"Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era","volume":"179","author":"Condliffe","year":"2009","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.berh.2016.03.002_bib69","doi-asserted-by":"crossref","first-page":"1846","DOI":"10.1093\/rheumatology\/kes140","article-title":"Clinical characteristics and survival of Japanese patients with connective tissue disease and pulmonary arterial hypertension: a single-centre cohort","volume":"51","author":"Shirai","year":"2012","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.berh.2016.03.002_bib70","doi-asserted-by":"crossref","first-page":"1494","DOI":"10.1378\/chest.13-3014","article-title":"Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry","volume":"146","author":"Chung","year":"2014","journal-title":"Chest"},{"key":"10.1016\/j.berh.2016.03.002_bib71","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1097\/00000372-199706000-00002","article-title":"Mixed connective tissue disease. A clinical, histologic, and immunofluorescence study of eight cases","volume":"19","author":"Magro","year":"1997","journal-title":"Am J Dermatopathol"},{"key":"10.1016\/j.berh.2016.03.002_bib72","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1097\/00004836-200101000-00008","article-title":"Esophageal investigations in connective tissue disease: which tests are most appropriate?","volume":"32","author":"Ling","year":"2001","journal-title":"J\u00a0Clin Gastroenterol"},{"key":"10.1016\/j.berh.2016.03.002_bib73","first-page":"515","article-title":"Esophageal motility disorders in the rheumatic diseases: a review of 150 patients","volume":"12","author":"Lapadula","year":"1994","journal-title":"Clin Exp Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib74","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1034\/j.1600-0676.2000.020005366.x","article-title":"The liver in collagen diseases: pathologic study of 160 cases with particular reference to hepatic arteritis, primary biliary cirrhosis, autoimmune hepatitis and nodular regenerative hyperplasia of the liver","volume":"20","author":"Matsumoto","year":"2000","journal-title":"Liver"},{"key":"10.1016\/j.berh.2016.03.002_bib75","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1007\/s10067-006-0218-1","article-title":"Severe acute hepatitis related to hydroxychloroquine in a woman with mixed connective tissue disease","volume":"26","author":"Galvan","year":"2007","journal-title":"Clin Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib76","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1507\/endocrj.46.173","article-title":"A\u00a0case of Graves' disease associated with autoimmune hepatitis and mixed connective tissue disease","volume":"46","author":"Inoue","year":"1999","journal-title":"Endocr J"},{"key":"10.1016\/j.berh.2016.03.002_bib77","first-page":"1278","article-title":"Primary biliary cirrhosis \u2013 a revised clinical spectrum","volume":"1","author":"James","year":"1981","journal-title":"Lancet"},{"key":"10.1016\/j.berh.2016.03.002_bib78","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1007\/BF02936959","article-title":"Mixed connective tissue disease associated with idiopathic portal hypertension and chronic thyroiditis","volume":"32","author":"Hirasaki","year":"1997","journal-title":"J\u00a0Gastroenterol"},{"key":"10.1016\/j.berh.2016.03.002_bib79","doi-asserted-by":"crossref","first-page":"1359","DOI":"10.2169\/internalmedicine.31.1359","article-title":"Mixed connective tissue disease associated with protein losing enteropathy: successful treatment with intravenous cyclophosphamide therapy","volume":"31","author":"Furuya","year":"1992","journal-title":"Intern Med"},{"key":"10.1016\/j.berh.2016.03.002_bib80","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1097\/RHU.0b013e318227e836","article-title":"Mixed connective tissue disease with protein-losing enteropathy: discussion of the treatment strategy","volume":"17","author":"Chao","year":"2011","journal-title":"J\u00a0Clin Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib81","doi-asserted-by":"crossref","first-page":"455","DOI":"10.3899\/jrheum.121087","article-title":"Gastric antral vascular ectasia and its clinical correlates in patients with early diffuse systemic sclerosis in the SCOT trial","volume":"40","author":"Hung","year":"2013","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib82","doi-asserted-by":"crossref","first-page":"584","DOI":"10.1016\/S0016-5107(94)70258-6","article-title":"The watermelon stomach: long-term outcome in patients treated with Nd:YAG laser therapy","volume":"40","author":"Liberski","year":"1994","journal-title":"Gastrointest Endosc"},{"key":"10.1016\/j.berh.2016.03.002_bib83","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1177\/0961203312441511","article-title":"Neuropsychological assessment in mixed connective tissue disease: comparison with systemic lupus erythematosus","volume":"21","author":"Nowicka-Sauer","year":"2012","journal-title":"Lupus"},{"key":"10.1016\/j.berh.2016.03.002_bib84","doi-asserted-by":"crossref","first-page":"1930","DOI":"10.3899\/jrheum.081314","article-title":"Sensorineural hearing loss in patients with mixed connective tissue disease: immunological markers and cytokine levels","volume":"36","author":"Hajas","year":"2009","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib85","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1159\/000166947","article-title":"Renal involvement in mixed connective tissue disease. Report of 5 cases","volume":"5","author":"Kobayashi","year":"1985","journal-title":"Am J Nephrol"},{"key":"10.1016\/j.berh.2016.03.002_bib86","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1097\/01.ASN.0000108969.21691.5D","article-title":"The classification of glomerulonephritis in systemic lupus erythematosus revisited","volume":"15","author":"Weening","year":"2004","journal-title":"J\u00a0Am Soc Nephrol"},{"key":"10.1016\/j.berh.2016.03.002_bib87","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1002\/art.1780380216","article-title":"Hla type as a predictor of mixed connective tissue disease differentiation ten-year clinical and immunogenetic followup of 46 patients","volume":"38","author":"Gendi","year":"1995","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib88","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1093\/rheumatology\/33.12.1117","article-title":"Long-term follow-up of 46 patients with anti-(U1)snRNP antibodies","volume":"33","author":"van den Hoogen","year":"1994","journal-title":"Br J Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib89","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1016\/j.semarthrit.2011.07.010","article-title":"\u201cTo be or not to be,\u201d ten years after: evidence for mixed connective tissue disease as a distinct entity","volume":"41","author":"Cappelli","year":"2012","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib90","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1002\/art.1780310302","article-title":"The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis","volume":"31","author":"Arnett","year":"1988","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib91","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1097\/00005792-197707000-00001","article-title":"Computer-assisted analysis of 153 patients with polymyositis and dermatomyositis","volume":"56","author":"Bohan","year":"1977","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.berh.2016.03.002_bib92","doi-asserted-by":"crossref","first-page":"1725","DOI":"10.1002\/art.1780400928","article-title":"Updating the American college of rheumatology revised criteria for the classification of systemic lupus erythematosus","volume":"40","author":"Hochberg","year":"1997","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib93","doi-asserted-by":"crossref","first-page":"2677","DOI":"10.1002\/art.34473","article-title":"Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus","volume":"64","author":"Petri","year":"2012","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib94","doi-asserted-by":"crossref","first-page":"1271","DOI":"10.1002\/art.1780251101","article-title":"The 1982 revised criteria for the classification of systemic lupus erythematosus","volume":"25","author":"Tan","year":"1982","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib95","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1002\/art.1780230510","article-title":"Subcommittee for Scleroderma Criteria of the American Rheumatism Association D, Therapeutic Criteria C. Preliminary criteria for the classification of systemic sclerosis (scleroderma)","volume":"23","author":"Masi","year":"1980","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib96","doi-asserted-by":"crossref","first-page":"2737","DOI":"10.1002\/art.38098","article-title":"2013 Classification criteria for systemic sclerosis: an American College of Rheumatology\/European League against Rheumatism collaborative initiative","volume":"65","author":"van den Hoogen","year":"2013","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib97","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1016\/j.berh.2013.03.001","article-title":"How to perform and interpret capillaroscopy","volume":"27","author":"Cutolo","year":"2013","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib98","doi-asserted-by":"crossref","first-page":"1166","DOI":"10.1016\/j.apmr.2011.01.022","article-title":"Effectiveness of interventions for secondary Raynaud's phenomenon: a systematic review","volume":"92","author":"Huisstede","year":"2011","journal-title":"Arch Phys Med Rehabil"},{"key":"10.1016\/j.berh.2016.03.002_bib99","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1093\/rheumatology\/keh390","article-title":"Calcium channel blockers for primary Raynaud's phenomenon: a meta-analysis","volume":"44","author":"Thompson","year":"2005","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.berh.2016.03.002_bib100","article-title":"Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis","author":"Pope","year":"1998","journal-title":"Cochrane Database Syst Rev"},{"key":"10.1016\/j.berh.2016.03.002_bib101","doi-asserted-by":"crossref","first-page":"1475","DOI":"10.1136\/ard.2009.116475","article-title":"Effect of sildenafil on digital ulcers in systemic sclerosis: analysis from a single centre pilot study","volume":"69","author":"Brueckner","year":"2010","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2016.03.002_bib102","doi-asserted-by":"crossref","first-page":"e102","DOI":"10.1016\/j.jaad.2011.04.029","article-title":"Combination therapy with an endothelin-1 receptor antagonist (bosentan) and a phosphodiesterase V inhibitor (sildenafil) for the management of severe digital ulcerations in systemic sclerosis","volume":"65","author":"Moinzadeh","year":"2011","journal-title":"J\u00a0Am Acad Dermatol"},{"key":"10.1016\/j.berh.2016.03.002_bib103","doi-asserted-by":"crossref","first-page":"1454","DOI":"10.1016\/j.jhsa.2009.05.003","article-title":"Long-term results of periarterial sympathectomy","volume":"34","author":"Hartzell","year":"2009","journal-title":"J\u00a0Hand Surg Am"},{"key":"10.1016\/j.berh.2016.03.002_bib104","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/j.semarthrit.2009.08.002","article-title":"Systemic sclerosis: bilateral improvement of Raynaud's phenomenon with unilateral digital sympathectomy","volume":"40","author":"Wasserman","year":"2010","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib105","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1016\/j.semarthrit.2011.07.006","article-title":"Botulinum toxin A treatment of Raynaud's phenomenon: a review","volume":"41","author":"Iorio","year":"2012","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib106","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/j.hcl.2014.09.003","article-title":"The role of botulinum toxin in vasospastic disorders of the hand","volume":"31","author":"Neumeister","year":"2015","journal-title":"Hand Clin"},{"key":"10.1016\/j.berh.2016.03.002_bib107","doi-asserted-by":"crossref","first-page":"2005","DOI":"10.2165\/10489160-000000000-00000","article-title":"Bosentan: a review of its use in the management of digital ulcers associated with systemic sclerosis","volume":"69","author":"Dhillon","year":"2009","journal-title":"Drugs"},{"key":"10.1016\/j.berh.2016.03.002_bib108","doi-asserted-by":"crossref","first-page":"3985","DOI":"10.1002\/art.20676","article-title":"Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist","volume":"50","author":"Korn","year":"2004","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib109","doi-asserted-by":"crossref","first-page":"1550","DOI":"10.3899\/jrheum.080992","article-title":"Bosentan for digital ulcers in patients with systemic sclerosis: a prospective 3-year followup study","volume":"36","author":"Tsifetaki","year":"2009","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib110","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1016\/j.healun.2015.10.002","article-title":"Anticoagulation in patients with pulmonary arterial hypertension: an update on current knowledge","volume":"35","author":"Roldan","year":"2016","journal-title":"J\u00a0Heart Lung Transpl"},{"key":"10.1016\/j.berh.2016.03.002_bib111","doi-asserted-by":"crossref","DOI":"10.1002\/14651858.CD010695.pub2","article-title":"Anticoagulation therapy versus placebo for pulmonary hypertension","volume":"6","author":"Ezedunukwe","year":"2014","journal-title":"Cochrane Database Syst Rev"},{"key":"10.1016\/j.berh.2016.03.002_bib112","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1111\/imj.12111","article-title":"Should patients with systemic sclerosis-related pulmonary arterial hypertension be anticoagulated?","volume":"43","author":"Nikpour","year":"2013","journal-title":"Intern Med J"},{"key":"10.1016\/j.berh.2016.03.002_bib113","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1183\/09031936.00039212","article-title":"Major bleeding with vitamin K antagonist anticoagulants in pulmonary hypertension","volume":"41","author":"Henkens","year":"2013","journal-title":"Eur Respir J"},{"key":"10.1016\/j.berh.2016.03.002_bib114","doi-asserted-by":"crossref","first-page":"896","DOI":"10.1056\/NEJMoa012212","article-title":"Bosentan therapy for pulmonary arterial hypertension","volume":"346","author":"Rubin","year":"2002","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.berh.2016.03.002_bib115","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1016\/j.jacc.2005.04.050","article-title":"Ambrisentan therapy for pulmonary arterial hypertension","volume":"46","author":"Galie","year":"2005","journal-title":"J\u00a0Am Coll Cardiol"},{"key":"10.1016\/j.berh.2016.03.002_bib116","doi-asserted-by":"crossref","first-page":"2148","DOI":"10.1056\/NEJMoa050010","article-title":"Sildenafil citrate therapy for pulmonary arterial hypertension","volume":"353","author":"Gali\u00e8","year":"2005","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.berh.2016.03.002_bib117","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1016\/j.ejim.2011.02.005","article-title":"Long-term effects of intermittent iloprost infusion on pulmonary arterial pressure in connective tissue disease","volume":"22","author":"Caravita","year":"2011","journal-title":"Eur J Intern Med"},{"key":"10.1016\/j.berh.2016.03.002_bib118","doi-asserted-by":"crossref","first-page":"1723","DOI":"10.1164\/rccm.201101-0093OC","article-title":"Vardenafil in pulmonary arterial hypertension","volume":"183","author":"Jing","year":"2011","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.berh.2016.03.002_bib119","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1586\/ers.11.38","article-title":"Tadalafil for the treatment of pulmonary arterial hypertension","volume":"5","author":"Klinger","year":"2011","journal-title":"Expert Rev Respir Med"},{"key":"10.1016\/j.berh.2016.03.002_bib120","doi-asserted-by":"crossref","first-page":"D60","DOI":"10.1016\/j.jacc.2013.10.031","article-title":"Updated treatment algorithm of pulmonary arterial hypertension","volume":"62","author":"Gali\u00e8","year":"2013","journal-title":"J\u00a0Am Coll Cardiol"},{"key":"10.1016\/j.berh.2016.03.002_bib121","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1055\/s-0033-1356460","article-title":"Management of severe pulmonary arterial hypertension","volume":"34","author":"Granton","year":"2013","journal-title":"Semin Respir Crit Care Med"},{"key":"10.1016\/j.berh.2016.03.002_bib122","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1055\/s-0033-1356495","article-title":"Current challenges in pulmonary hypertension","volume":"34","author":"Humbert","year":"2013","journal-title":"Semin Respir Crit Care Med"},{"key":"10.1016\/j.berh.2016.03.002_bib123","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1056\/NEJMoa1213917","article-title":"Macitentan and morbidity and mortality in pulmonary arterial hypertension","volume":"369","author":"Pulido","year":"2013","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.berh.2016.03.002_bib124","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1164\/rccm.201507-1398OC","article-title":"Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension","volume":"192","author":"Hassoun","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.berh.2016.03.002_bib125","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1056\/NEJMoa1503184","article-title":"Selexipag for the treatment of pulmonary arterial hypertension","volume":"373","author":"Sitbon","year":"2015","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.berh.2016.03.002_bib126","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1183\/09031936.00137511","article-title":"Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension","volume":"40","author":"Simonneau","year":"2012","journal-title":"Eur Respir J"},{"key":"10.1016\/j.berh.2016.03.002_bib127","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1586\/17476348.2016.1121103","article-title":"Selexipag for the treatment of pulmonary arterial hypertension","volume":"10","author":"Sharma","year":"2016","journal-title":"Expert Rev Respir Med"},{"key":"10.1016\/j.berh.2016.03.002_bib128","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1002\/art.23303","article-title":"Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases","volume":"58","author":"Jais","year":"2008","journal-title":"Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib129","doi-asserted-by":"crossref","first-page":"1628","DOI":"10.1093\/rheumatology\/kem208","article-title":"Comment on: a case of Raynaud's phenomenon in mixed connective tissue disease responding to rituximab therapy response","volume":"46","author":"Dunkley","year":"2007","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.berh.2016.03.002_bib130","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1093\/rheumatology\/kem003","article-title":"A\u00a0case of Raynaud's phenomenon in mixed connective tissue disease responding to rituximab therapy","volume":"46","author":"Haroon","year":"2007","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.berh.2016.03.002_bib131","doi-asserted-by":"crossref","first-page":"1734","DOI":"10.1055\/s-0029-1234008","volume":"134","author":"Rudolph","year":"2009","journal-title":"Dtsch Med Wochenschr"},{"key":"10.1016\/j.berh.2016.03.002_bib132","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.1177\/0961203310369145","article-title":"Severe ischemia following treatment with rituximab in a patient with mixed connective tissue disease: an unusual complication","volume":"19","author":"Vela","year":"2010","journal-title":"Lupus"},{"key":"10.1016\/j.berh.2016.03.002_bib133","first-page":"S1","article-title":"Evaluating the therapeutic effects of B cell depletion therapy with rituximab in a longitudinal cohort of mixed connective tissue disease patients","author":"Gunnarsson","year":"2012","journal-title":"Arthritis Rheum"},{"issue":"suppl 10","key":"10.1016\/j.berh.2016.03.002_bib134","article-title":"Rituximab in refractory mixed connective tissue disease: an observational study","volume":"67","author":"Pascual","year":"2015","journal-title":"Arthritis Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib135","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1007\/s10067-010-1630-0","article-title":"B\u00a0cell depletion for autoimmune diseases in paediatric patients","volume":"30","author":"Jansson","year":"2011","journal-title":"Clin Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib136","first-page":"273","article-title":"Lupus erythematosus manifestations exacerbated by etanercept therapy in a patient with mixed connective tissue disease","volume":"23","author":"Richez","year":"2005","journal-title":"Clin Exp Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib137","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1016\/j.semarthrit.2007.08.003","article-title":"Drug-induced lupus due to anti-tumor necrosis factor alpha agents","volume":"37","author":"Costa","year":"2008","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.berh.2016.03.002_bib138","first-page":"2725","article-title":"Tumor necrosis factor-alpha antagonists induce lupus-like syndrome in patients with scleroderma overlap\/mixed connective tissue disease","volume":"30","author":"Christopher-Stine","year":"2003","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib139","first-page":"587","article-title":"Mixed connective tissue disease \u2013 a subset with sequential clinical and laboratory features","volume":"8","author":"Grant","year":"1981","journal-title":"J\u00a0Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib140","doi-asserted-by":"crossref","first-page":"359","DOI":"10.3109\/07853899708999362","article-title":"Course of mixed connective tissue disease in children","volume":"29","author":"Michels","year":"1997","journal-title":"Ann Med"},{"key":"10.1016\/j.berh.2016.03.002_bib141","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1093\/rheumatology\/29.5.345","article-title":"Patients with arthritis and anti-U1-RNP antibodies: a 10-year follow-up","volume":"29","author":"Piirainen","year":"1990","journal-title":"Br J Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib142","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1016\/S0022-3476(77)80555-6","article-title":"Mixed connective tissue disease in childhood. A clinical and serologic survey","volume":"90","author":"Singsen","year":"1977","journal-title":"J\u00a0Pediatr"},{"key":"10.1016\/j.berh.2016.03.002_bib143","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1093\/rheumatology\/kes430","article-title":"Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature","volume":"52","author":"Gunnarsson","year":"2013","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.berh.2016.03.002_bib144","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1378\/chest.12-2417","article-title":"Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry","volume":"144","author":"Barst","year":"2013","journal-title":"Chest"},{"key":"10.1016\/j.berh.2016.03.002_bib145","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1183\/09031936.00092311","article-title":"The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension","volume":"39","author":"Nickel","year":"2012","journal-title":"Eur Respir J"},{"key":"10.1016\/j.berh.2016.03.002_bib146","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1378\/chest.15-0300","article-title":"FIve-year outcomes of patients enrolled in the reveal registry","volume":"148","author":"Farber","year":"2015","journal-title":"Chest"},{"key":"10.1016\/j.berh.2016.03.002_bib147","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1002\/art.39432","article-title":"Characteristics and survival of patients with anti-U1RNP antibodies in connective tissue disease associated pulmonary arterial hypertension","volume":"68","author":"Sobanski","year":"2016","journal-title":"Arthritis Rheumatol"},{"key":"10.1016\/j.berh.2016.03.002_bib148","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1093\/rheumatology\/kev300","article-title":"Associations between anti-Ro52 antibodies and lung fibrosis in mixed connective tissue disease","volume":"55","author":"Gunnarsson","year":"2016","journal-title":"Rheumatology (Oxford)"},{"key":"10.1016\/j.berh.2016.03.002_bib149","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1002\/acr.20693","article-title":"Practical approach to screening for scleroderma-associated pulmonary arterial hypertension","volume":"64","author":"Fischer","year":"2012","journal-title":"Arthritis Care Res Hob"},{"key":"10.1016\/j.berh.2016.03.002_bib150","doi-asserted-by":"crossref","first-page":"1340","DOI":"10.1136\/annrheumdis-2013-203301","article-title":"Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study","volume":"73","author":"Coghlan","year":"2014","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.berh.2016.03.002_bib151","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/S0022-3476(06)80112-5","article-title":"Juvenile-onset mixed connective tissue disease: longitudinal follow-up","volume":"122","author":"Tiddens","year":"1993","journal-title":"J\u00a0Pediatr"}],"container-title":["Best Practice &amp; Research Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521694216000140?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1521694216000140?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,9,13]],"date-time":"2018-09-13T11:53:01Z","timestamp":1536839581000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1521694216000140"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,2]]},"references-count":151,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2016,2]]}},"alternative-id":["S1521694216000140"],"URL":"https:\/\/doi.org\/10.1016\/j.berh.2016.03.002","relation":{},"ISSN":["1521-6942"],"issn-type":[{"value":"1521-6942","type":"print"}],"subject":[],"published":{"date-parts":[[2016,2]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Mixed connective tissue disease","name":"articletitle","label":"Article Title"},{"value":"Best Practice & Research Clinical Rheumatology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.berh.2016.03.002","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2016 Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}]}}