614
Views
16
CrossRef citations to date
0
Altmetric
Review

Recent advances in connective tissue disease related interstitial lung disease

, &
Pages 591-603 | Received 09 Mar 2017, Accepted 24 May 2017, Published online: 02 Jun 2017

References

  • Fischer A, du Bois R. Interstitial lung disease in connective tissue disorders. Lancet. 2012;380:689–698.
  • Olson AL, Brown KK, Fischer A. Connective tissue disease-associated lung disease. Immunol Allergy Clin North Am. 2012;32:513–536.
  • Schurawitzki H, Stiglbauer R, Graninger W, et al. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology. 1990;176:755–759.
  • Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66:940–944.
  • Olson AL, Swigris JJ, Sprunger DB, et al. Rheumatoid arthritis-interstitial lung disease-associated mortality. Am J Respir Crit Care Med. 2011;183:372–378.
  • Marie I, Hachulla E, Chérin P, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002;47:614–622.
  • Gutsche M, Rosen GD, Swigris JJ. Connective tissue disease-associated interstitial lung disease: a review. Curr Respir Care Rep. 2012;1:224–232.
  • Fischer A, Antoniou KM, Brown KK, et al.; 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;46:976–987.
  • Ono H, Taniguchi Y, Shinoda K, et al. Evaluation of the usefulness of spectral analysis of inspiratory lung sounds recorded with phonopneumography in patients with interstitial pneumonia. J Nippon Med Sch. 2009;76:67–75.
  • Cottin V, Cordier J-F. Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? Eur Respir J. 2012;40:519–521.
  • Cottin V, Richeldi L. Neglected evidence in idiopathic pulmonary fibrosis and the importance of early diagnosis and treatment. Eur Respir Rev. 2014;23:106–110.
  • Epler GR, McLoud TC, Gaensler EA, et al. Normal chest roentgenograms in chronic diffuse infiltrative lung disease. N Engl J Med. 1978;298:934–939.
  • Epler GR, Carrington CB, Gaensler EA. Crackles (rales) in the interstitial pulmonary diseases. Chest. 1978;73:333–339.
  • Sellarés J, Hernández-González F, Lucena CM, et al. Auscultation of velcro crackles is associated with usual interstitial pneumonia. Medicine (Baltimore). 2016;95:e2573.
  • Bonella F, Costabel U. Biomarkers in connective tissue disease-associated interstitial lung disease. Semin Respir Crit Care Med. 2014;35:181–200.
  • Hant FN, Ludwicka-Bradley A, Wang H-J, et al.; Scleroderma Lung Study Research Group. Surfactant protein D and KL-6 as serum biomarkers of interstitial lung disease in patients with scleroderma. J Rheumatol. 2009;36:773–780.
  • Yamane K, Ihn H, Kubo M, et al. Serum levels of KL-6 as a useful marker for evaluating pulmonary fibrosis in patients with systemic sclerosis. J Rheumatol. 2000;27:930–934.
  • Bonella F, Volpe A, Caramaschi P, et al. Surfactant protein D and KL-6 serum levels in systemic sclerosis: correlation with lung and systemic involvement. Sarcoidosis Vasc Diffuse Lung Dis. 2011;28:27–33.
  • Kubo M, Ihn H, Yamane K, et al. Serum KL-6 in adult patients with polymyositis and dermatomyositis. Rheumatology (Oxford). 2000;39:632–636.
  • Fathi M, Barbasso Helmers S, Lundberg IE. KL-6: a serological biomarker for interstitial lung disease in patients with polymyositis and dermatomyositis. J Int Med. 2012;271:589–597.
  • Kinoshita F, Hamano H, Harada H, et al. Role of KL-6 in evaluating the disease severity of rheumatoid lung disease: comparison with HRCT. Respir Med. 2004;98:1131–1137.
  • Oguz EO, Kucuksahin O, Turgay M, et al. Association of serum KL-6 levels with interstitial lung disease in patients with connective tissue disease: a cross-sectional study. Clin Rheumatol. 2016;35:663–666.
  • Takahashi H, Kuroki Y, Tanaka H, et al. Serum levels of surfactant proteins A and D are useful biomarkers for interstitial lung disease in patients with progressive systemic sclerosis. Am J Respir Crit Care Med. 2000;162:258–263.
  • Self SE. Autoantibody testing for autoimmune disease. Clin Chest Med. 2010;31:415–422.
  • Fertig N, Domsic RT, Rodriguez-Reyna T, et al. Anti-U11/U12 RNP antibodies in systemic sclerosis: a new serologic marker associated with pulmonary fibrosis. Arthritis Rheum. 2009;61:958–965.
  • Steen VD, Powell DL, Medsger TA Jr. Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum. 1988;31:196–203.
  • Hamaguchi Y, Fujimoto M, Matsushita T, et al. Common and distinct clinical features in adult patients with anti-aminoacyl-tRNA synthetase antibodies: heterogeneity within the syndrome. PLoS One. 2013;8:e60442.
  • Sato S, Murakami A, Kuwajima A, et al. Clinical utility of an enzyme-linked immunosorbent assay for detecting anti-melanoma differentiation-associated gene 5 autoantibodies. PLoS One. 2016;11:e0154285.
  • Moghadam-Kia S, Oddis CV, Sato S, et al. Anti-melanoma differentiation-associated gene 5 is associated with rapidly progressive lung disease and poor survival in US patients with amyopathic and myopathic dermatomyositis. Arthritis Care Res (Hoboken). 2016;68:689–694.
  • Chen Z, Cao M, Plana MN, et al. Utility of anti-melanoma differentiation-associated gene 5 antibody measurement in identifying patients with dermatomyositis and a high risk for developing rapidly progressive interstitial lung disease: a review of the literature and a meta-analysis. Arthritis Care Res (Hoboken). 2013;65:1316–1324.
  • Reynisdottir G, Karimi R, Joshua V, et al. Structural changes and antibody enrichment in the lungs are early features of anti-citrullinated protein antibody-positive rheumatoid arthritis. Arthritis Rhematol. 2014;66:31–39.
  • Davidson BK, Kelly CA, Griffiths ID. Ten year follow up of pulmonary function in patients with primary Sjögren’s syndrome. Ann Rheum Dis. 2000;59:709–712.
  • Aggarwal R, Lucas M, Fertig N, et al. Anti-U3 RNP autoantibodies in systemic sclerosis. Arthritis Rheum. 2009;60:1112–1118.
  • Mitri GM, Lucas M, Fertig N, et al. A comparison between anti-Th/To- and anticentromere antibody-positive systemic sclerosis patients with limited cutaneous involvement. Arthritis Rheum. 2003;48:203–209.
  • Zamora AC, Hoskote SS, Abascal-Bolado B, et al. Clinical features and outcomes of interstitial lung disease in anti-Jo-1 positive antisynthetase syndrome. Respir Med. 2016;118:39–45.
  • Sato S, Hirakata M, Kuwana M, et al. Clinical characteristics of Japanese patients with anti-PL-7 (anti-threonyl-tRNA synthetase) autoantibodies. Clin Exp Rheumatol. 2005;23:609–615.
  • Chartrand S, Swigris JJ, Peykova L, et al. A multidisciplinary evaluation helps identify the antisynthetase syndrome in patients presenting as idiopathic interstitial pneumonia. J Rheumatol. 2016;43:887–892.
  • Waseda Y, Johkoh T, Egashira R, et al. Antisynthetase syndrome: pulmonary computed tomography findings of adult patients with antibodies to aminoacyl-tRNA synthetases. Eur J Radiol. 2016;85:1421–1426.
  • Nakashima R, Imura Y, Hosono Y, et al. The multicenter study of a new assay for simultaneous detection of multiple anti-aminoacyl-tRNA synthetases in myositis and interstitial pneumonia. PLoS One. 2014;9:e85062.
  • Sato S, Hirakata M, Kuwana M, et al. Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. Arthritis Rheum. 2005;52:1571–1576.
  • Sato S, Hoshino K, Satoh T, et al. RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: association with rapidly progressive interstitial lung disease. Arthritis Rheum. 2009;60:2193–2200.
  • Takada T, Aoki A, Asakawa K, et al. Serum cytokine profiles of patients with interstitial lung disease associated with anti-CADM-140/MDA5 antibody positive amyopathic deratomyositis. Respir Med. 2015;109:1174–1180.
  • Nagata K, Tomii K, Nanjo S, et al. Four cases of interstitial pneumonia associated with amyopathic dermatomyositis characterized by the anti-CADM-140 antibody. Nihon Kokyuki Gakkai Zasshi. 2011;49:30–36.
  • Suzuki A, Kondoh Y, Taniguchi H, et al. Lung histopathological pattern in a survivor with rapidly progressive interstitial lung disease and anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis. Respir Med Case Rep. 2016;19:5–8.
  • Avouac J, Gossec L, Dougados M. Diagnostic and predictive value of anti-cyclic citrullinated protein antibodies in rheumatoid arthritis: a systematic literature review. Ann Rheum Dis. 2006;65:845–851.
  • Klareskog L, Stolt P, Lundberg K, et al. A new model for an etiology of rheumatoid arthritis: smoking may trigger HLA-DR (shared epitope)-restricted immune reactions to autoantigens modified by citrullination. Arthritis Rheum. 2006;54:38–46.
  • Aubart F, Crestani B, Nicaise-Roland P, et al. High levels of anti-cyclic citrullinated peptide autoantibodies are associated with co-occurrence of pulmonary diseases with rheumatoid arthritis. J Rheumatol. 2011;38:979–982.
  • Fischer A, Solomon JJ, du Bois RM, et al. Lung disease with anti-CCP antibodies but not rheumatoid arthritis or connective tissue disease. Respir Med. 2012;106:1040–1047.
  • Yazisiz V, Arslan G, Ozbudak IH, et al. Lung involvement in patients with primary Sjögren’s syndrome: what are the predictors? Rheumatol Int. 2010;30:1317–1324.
  • Picano E, Matucci-Cerinic M. Unnecessary radiation exposure from medical imaging in the rheumatology patient. Rheumatology (Oxford). 2011;50:1537–1539.
  • Gargani L, Doveri M, D’Errico L, et al. Ultrasound lung comets in systemic sclerosis: a chest sonography hallmark of pulmonary interstitial fibrosis. Rheumatology (Oxford). 2009;48:1382–1387.
  • Gutierrez M, Salaffi F, Carotti M, et al. Utility of a simplified ultrasound assessment to assess interstitial pulmonary fibrosis in connective tissue disorders—preliminary results. Arthritis Res Ther. 2011;13:R134.
  • Tardella M, Gutierrez M, Salaffi F, et al. Ultrasound in the assessment of pulmonary fibrosis in connective tissue disorders: correlation with high-resolution computed tomography. J Rheumatol. 2012;39:1641–1647.
  • Barskova T, Gargani L, Guiducci S, et al. Lung ultrasound for the screening of interstitial lung disease in very early systemic sclerosis. Ann Rheum Dis. 2013;72:390–395.
  • Cogliati C, Antivalle M, Torzillo D, et al. Standard and pocket-size lung ultrasound devices can detect interstitial lung disease in rheumatoid arthritis patients. Rheumatology (Oxford). 2014;53:1497–1503.
  • Song G, Bae SC, Lee YH. Diagnostic accuracy of lung ultrasound for interstitial lung disease in patients with connective tissue diseases: a meta-analysis. Clin Exp Rheumatol. 2016;34:11–16.
  • Travis WD, Costabel U, Hansell DM, et al.; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733–748.
  • Satoh H, Kurishima K, Ishikawa H, et al. Increased levels of KL-6 and subsequent mortality in patients with interstitial lung diseases. J Intern Med. 2006;260:429–434.
  • Asano Y, Ihn H, Yamane K, et al. Clinical significance of surfactant protein D as a serum marker for evaluating pulmonary fibrosis in patients with systemic sclerosis. Arthritis Rheum. 2001;44:1363–1369.
  • De Lauretis A, Sestini P, Pantelidis P, et al. Serum interleukin 6 is predictive of early functional decline and mortality in interstitial lung disease associated with systemic sclerosis. J Rheumatol. 2013;40:435–446.
  • Winstone TA, Assayag D, Wilcox PG, et al. Predictors of mortality and progression in scleroderma-associated interstitial lung disease: a systematic review. Chest. 2014;146:422–436.
  • Assayag D, Lubin M, Lee JS, et al. Predictors of mortality in rheumatoid arthritis-related interstitial lung disease. Respirology. 2014;19:493–500.
  • Solomon JJ, Chung JH, Cosgrove GP, et al. Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J. 2016;47:588–596.
  • Walsh SL, Sverzellati N, Devaraj A, et al. Connective tissue disease related fibrotic lung disease: high resolution computed tomographic and pulmonary function indices as prognostic determinants. Thorax. 2014;69:216–222.
  • Kelly CA, Saravanan V, Nisar M, et al.; British Rheumatoid Interstitial Lung (BRILL) Network. Rheumatoid arthritis-related interstitial lung disease: associations, prognostic factors and physiological and radiological characteristics—a large multicenter UK study. Rheumatology (Oxford). 2014;53:1676–1682.
  • Park JH, Kim DS, Park IN, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med. 2007;175:705–711.
  • Moua T, Zamora Martinez AC, Baqir M, et al. Predictors of diagnosis and survival in idiopathic pulmonary fibrosis and connective tissue disease-related usual interstitial pneumonia. Respir Res. 2014;15:154.
  • Song JW, Do KH, Kim MY, et al. Pathologic and radiologic differences between idiopathic and collagen vascular disease-related usual interstitial pneumonia. Chest. 2009;136:23–30.
  • Fischer A, Richeldi L. Cross-disciplinary collaboration in connective tissue disease-related lung disease. Semin Respir Crit Care Med. 2014;35:159–165.
  • Ryerson CJ, Vittinghoff E, Ley B, et al. Predicting survival across chronic interstitial lung disease: the ILD-GAP model. Chest. 2014;145:723–728.
  • Ryerson CJ, O’Connor D, Dunne JV, et al. Predicting mortality in systemic sclerosis-associated interstitial lung disease using risk prediction models derived from idiopathic pulmonary fibrosis. Chest. 2015;148:1268–1275.
  • Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177:1248–1254.
  • Bartholmai BJ, Raghunath S, Karwoski RA, et al. Quantitative computed tomography imaging of interstitial lung diseases. J Thorac Imaging. 2013;28:298–307.
  • Maldonado F, Moua T, Rajagopalan S, et al. Automated quantification of radiological patterns predicts survival in idiopathic pulmonary fibrosis. Eur Respir J. 2014;43:204–212.
  • Iwasawa T, Asakura A, Sakai F, et al. Assessment of prognosis of patients with idiopathic pulmonary fibrosis by computer-aided analysis of CT images. J Thorac Imaging. 2009;24:216–222.
  • Best AC, Meng J, Lynch AM, et al. Idiopathic pulmonary fibrosis: physiologic tests, quantitative CT indexes, and CT visual scores as predictors of mortality. Radiology. 2008;246:935–940.
  • Jacob J, Bartholmai BJ, Rajagopalan S, et al. Evaluation of computer-based computer tomography stratification against outcome models in connective tissue disease-related interstitial lung disease: a patient outcome study. BMC Med. 2016;14:190.
  • Khanna D, Clements PJ, Furst DE, et al.; Scleroderma Lung Study Group. Correlation of the degree of dyspnea with health-related quality of life, functional abilities, and diffusing capacity for carbon monoxide in patients with systemic sclerosis and active alveolitis: results from the Scleroderma Lung Study. Arthritis Rheum. 2005;52:592–600.
  • Swigris JJ, Yorke J, Sprunger DB, et al. Assessing dyspnea and its impact on patients with connective tissue disease-related interstitial lung disease. Respir Med. 2010;104:1350–1355.
  • Mittoo S, Frankel S, Le Sage D, et al. Patient perspectives in OMERACT provide an anchor for future metric development and improved approaches to healthcare delivery in connective tissue disease related interstitial lung disease (CTD-ILD). Curr Respir Med Rev. 2015;11:175–183.
  • Fisher A, Chartrand S. Assessment and management of connective tissue disease-associated interstitial lung disease. Sarcoidosis Vasc Diffuse Lung Dis. 2015;32:3–21.
  • Chartrand S, Fischer A. Management of connective tissue disease-associated interstitial lung disease. Rheum Dis Clin North Am. 2015;41:279–294.
  • Hozumi H, Nakamura Y, Johkoh T, et al. Acute exacerbation in rheumatoid arthritis-associated interstitial lung disease: a retrospective case control study. BMJ Open. 2013;3:e003132.
  • Suda T, Kaida Y, Nakamura Y, et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med. 2009;103:846–853.
  • Tachikawa R, Tomii K, Ueda H, et al. Clinical features and outcome of acute exacerbation of interstitial pneumonia: collagen vascular diseases-related versus idiopathic. Respiration. 2012;83:20–27.
  • Mathai SC, Danoff SK. Management of interstitial lung disease associated with connective tissue disease. BMJ. 2016;352:h6819.
  • Iudici M, van der Goes MC, Valentini G, et al. Glucocorticoids in systemic sclerosis: weighing the benefits and risks – a systematic review. Clin Exp Rheumatol. 2013;31:157–165.
  • Ando K, Motojima S, Doi T, et al. Effect of glucocorticoid monotherapy on pulmonary function and survival in Japanese patients with scleroderma-related interstitial lung disease. Respir Investig. 2013;51:69–75.
  • Nannini C, West CP, Erwin PJ, et al. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther. 2008;10:R124.
  • Tashkin DP, Elashoff R, Clements PJ, et al.; Scleroderma Lung Study Research Group. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655–2666.
  • Tashkin DP, Elashoff R, Clements PJ, et al.; Scleroderma Lung Study Research Group. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med. 2007;176:1026–1034.
  • Hoyles RK, Ellis RW, Wellsbury J, et al. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids an intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum. 2006;54:3962–3970.
  • Fischer A, Brown KK, Du Bois RM, et al. Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease. J Rheumatol. 2013;40:640–646.
  • Tzouvelekis A, Galanopoulos N, Bouros E, et al. Effect and safety of mycophenolate mofetil or sodium in systemic sclerosis-associated interstitial lung disease: a meta-analysis. Pulm Med. 2012;2012:143637.
  • Tashkin DP, Roth MD, Clements PJ, et al.; Sclerodema Lung Study II Investigators. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomized controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4:708–719.
  • Kurita T, Yasuda S, Amengual O, et al. The efficacy of calcineurin inhibitors for the treatment of interstitial lung disease associated with polymyositis/dermatomyositis. Lupus. 2015;24:3–9.
  • Dumont FJ. FK506, an immunosuppressant targeting calcineurin function. Curr Med Chem. 2000;7:731–748.
  • Gruhn WB, Diaz-Buxo JA. Cyclosporine treatment of steroid resistant interstitial pneumonitis associated with dermatomyositis/polymyositis. J Rheumatol. 1987;14:1045–1047.
  • Nawata Y, Kurasawa Y, Takabayashi K, et al. Corticosteroid resistant interstitial pneumonitis in dermatomyositis/polymyositis: prediction and treatment with cyclosporine. J Rheumatol. 1999;26:1527–1533.
  • Nagasaka K, Harigai M, Tateishi M, et al. Efficacy of combination treatment with cyclosporine A and corticosteroids for acute interstitial pneumonitis associated with dermatomyositic. Mod Rheumatol. 2003;13:231–238.
  • Watanabe N, Sakamoto K, Taniguchi H, et al. Efficacy of combined therapy with cyclosporine and low-low dose prednisolone in interstitial pneumonia associated with connective tissue disease. Respiration. 2014;87:46–477.
  • Kurita T, Yasuda S, Oba K, et al. The efficacy of tacrolimus in patients with interstitial lung diseases complicated with polymyositis or dermatomyositis. Rheumatology (Oxford). 2015;54:39–44.
  • Witt LJ, Demchuk C, Curran JJ, et al. Benefit of adjunctive tacrolimus in connective tissue disease-interstitial lung disease. Pulm Pharmacol Ther. 2016;36:46–52.
  • Ge Y, Zhou H, Shi J, et al. The efficacy of tacrolimus in patients with refractory dermatomyositis/polymyositis: a systematic review. Clin Rheumatol. 2015;34:2097–2103.
  • Lee JS, Fischer A. Current and emerging treatment options for interstitial lung disease in patients with rheumatic disease. Expert Rev Clin Immunol. 2016;12:509–520.
  • Dheda K, Lalloo UG, Cassim B, et al. Experience with azathioprine in systemic sclerosis associated with interstitial lung disease. Clin Rheumatol. 2004;23:306–309.
  • Nadashkevich O, Davis P, Fritzler M, et al. A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol. 2006;25:205–212.
  • Poormoghim H, Rezaei N, Sheidaie Z, et al. Systemic sclerosis: comparison of efficacy of oral cyclophosphamide and azathioprine on skin score and pulmonary involvement-a retrospective study. Rheumatol Int. 2014;34:1691–1699.
  • Oldham JM, Lee C, Valenzi E, et al. Azathioprine response in patients with fibrotic connective tissue disease-associated interstitial lung disease. Respir Med. 2016;121:117–122.
  • Gürcan HM, Keskin DB, Stern JN, et al. A review of the current use of rituximab in autoimmune diseases. Int Immunopharmacol. 2009;9:10–25.
  • Keir GJ, Maher TM, Hansell DM, et al. Severe interstitial lung disease in connective tissue disease: rituximab as rescue therapy. Eur Respir J. 2012;40:641–648.
  • Sharp C, McCabe M, Dodds N, et al. Rituximab in autoimmune connective tissue disease-associated interstitial lung disease. Rheumatology (Oxford). 2016;55:1318–1324.
  • Daoussis D, Liossis SN, Tsamandas AC, et al. Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis. Clin Exp Rheumatol. 2012;30:S17–S22.
  • Matteson EL, Bongartz T, Ryu JH, et al. Open-label, pilot study of the safety and clinical effects of rituximab in patients with rheumatoid arthritis-associated interstitial pneumonia. Open J Rheumatol Autoimmune Dis. 2012;2:53–58.
  • Lim SW, Gillis D, Smith W, et al. Rituximab use in systemic lupus erythematosus pneumonitis and a review of current reports. Intern Med J. 2006;36:260–262.
  • Jordan S, Distler JH, Maurer B, et al.; EUSTAR Rituximab study group. Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group. Ann Rheum Dis. 2015;74:1188–1194.
  • Oddis CV, Reed AM, Aggarwal R, et al.; RIM Study Group. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum. 2013;65:314–324.
  • Hallowell RW, Amariei D, Danoff SK. Intravenous immunoglobulin as potential adjunct therapy for interstitial lung disease. Ann Am Thorac Soc. 2016;13:1682–1688.
  • Dalakas MC, Illa I, Dambrosia JM, et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med. 1993;329:1993–2000.
  • Miyasaka N, Hara M, Koike T, et al.; GB-0998 Study Group. Effects of intravenous immunoglobulin therapy in Japanese patients with polymyositis and dermatomyositis resistant to corticosteroids: a randomized double-blind placebo-controlled trial. Mod Rheumatol. 2012;22:382–393.
  • Suzuki Y, Hayakawa H, Miwa S, et al. Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis. Lung. 2009;187:201–206.
  • Enomoto N, Chida K, Suda T, et al. An exploratory trial of intravenous immunoglobulin therapy for idiopathic pulmonary fibrosis: a preliminary multicenter report. Clin Respir J. 2016;10:746–755.
  • Taniguchi H, Ebina M, Kondoh Y, et al.; Pirfenidone Clinical Study Group in Japan. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010;35:821–829.
  • Noble PW, Albera C, Bradford WZ, et al.; CAPACITY Study Group. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomized trials. Lancet. 2011;377:1760–1769.
  • King TE Jr, Bradford WZ, Castro-Bernardini S, et al.; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2083–2092.
  • Richeldi L, Costabel U, Selman M, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011;365:1079–1087.
  • Richeldi L, du Bois RM, Raghu G, et al.; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2071–2082.
  • Miura Y, Saito T, Fujita K, et al. Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31:235–238.
  • Khanna D, Albera C, Fischer A, et al. An open-label, phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial. J Rheumatol. 2016;43:1672–1679.
  • Gratwohl A, Baldomero H, Aljurf M, et al.; Worldwide Network of Blood and Marrow Transplantation. Hematopoietic stem cell transplantation: a global perspective. Jama. 2010;303:1617–1624.
  • van Laar JM, Naraghi K, Tyndall A. Haematopoietic stem cell transplantation for poor-prognosis systemic sclerosis. Rheumatology (Oxford). 2015;54:2126–2133.
  • Binks M, Passweg JR, Furst D, et al. Phase I/II trial of autologous stem cell transplantation in systemic sclerosis: procedure related mortality and impact on skin disease. Ann Rheum Dis. 2001;60:577–584.
  • Farge D, Passweg J, van Laar JM, et al.; EBMT/EULAR Registry. Autologous stem cell transplantation in the treatment of systemic sclerosis: report from the EBMT/EULAR Registry. Ann Rheum Dis. 2004;63:974–981.
  • Nash RA, McSweeney PA, Crofford LJ, et al. High-dose immunosuppressive therapy and autologous hematopoietic cell transplantation for severe systemic sclerosis; long-term follow-up of the US multicenter pilot study. Blood. 2007;110:1388–1396.
  • Burt RK, Shah SJ, Dill K, et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomized phase 2 trial. Lancet. 2011;378:498–506.
  • van Laar JM, Farge D, Sont JK, et al.; EBMT/EULAR Scleroderma Study Group. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. Jama. 2014;311:2490–2498.
  • Yazdani A, Singer LG, Strand V, et al. Survival and quality of life in rheumatoid arthritis-associated interstitial lung disease after lung transplantation. J Heart Lung Transplant. 2014;33:514–520.
  • Yusen RD, Edwards LB, Kucheryavaya AY, et al.; International Society for Heart and Lung Transplantation. The registry of the International Society for Heart and Lung Transplantation: thirty-first adult lung and heart-lung transplant report-2014; focus theme: retransplantation. J Heart Lung Transplant. 2014;33:1009–1024.
  • Saggar R, Khanna D, Furst DE, et al. Systemic sclerosis and bilateral lung transplantation: a single centre experience. Eur Respir J. 2010;36:893–900.
  • Takagishi T, Ostrowski R, Alex C, et al. Survival and extrapulmonary course of connective tissue disease after lung transplantation. J Clin Rheumatol. 2012;18:283–289.
  • Weill D, Benden C, Corris PA, et al. A consensus document for the selection of lung transplant candidates: 2014 – an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34:1–15.
  • Crockett AJ, Cranston JM, Antic N. Domiciliary for interstitial lung disease. Cochrane Database Syst Rev. 2001;(3):CD002883. DOI: 10.1002/14651858.CD002883.
  • Sharp C, Adamali H, Millar AB. Ambulatory and short-burst oxygen for interstitial lung disease. Cochrane Database Syst Rev. 2016;7:CD011716.
  • Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014;(10):CD006322. DOI: 10.1002/14651858.CD006322.pub3.
  • Baughman RP, Meyer KC, Nathanson I, et al. Monitoring of nonsteroidal immunosuppressive drugs in patients with lung disease and lung transplant recipients: American College of Chest Physicians evidence-based clinical practice guidelines. Chest. 2012;142:e1S–e111S.
  • Fischer A, Chartrand S. Assessment and management of connective tissue disease-associated interstitial lung disease. Sarcoidosis Vasc Diffuse Lung Dis. 2015;32:2–21.
  • Vananuvat P, Suwannalai P, Sungkanuparph S, et al. Primary prophylaxis for Pneumocystis jirovecii pneumonia in patients with connective tissue diseases. Semin Arthritis Rheum. 2011;41:497–502.
  • Caplan-Shaw CE, Arcasoy SM, Shane E, et al. Osteoporosis in diffuse parenchymal lung disease. Chest. 2006;129:140–146.
  • Van Staa TP, Leufkens HG, Abenhaim L, et al. Use of oral corticosteroids and risk of fractures, June, 2000. J Bone Miner Res. 2005;20:1487–1494.
  • Christmann RB, Wells AU, Capelozzi VL, et al. Gastroesophageal reflux incites interstitial lung disease in systemic sclerosis: clinical, radiologic, histopathologic, and treatment evidence. Semin Arthritis Rheum. 2010;40:241–249.
  • Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37:67–119.
  • Takahashi K, Taniguchi H, Ando M, et al. Mean pulmonary arterial pressure as a prognostic indicator in connective tissue disease associated with interstitial lung disease: a retrospective cohort study. BMC Pulm Med. 2016;16:55.
  • Trad S, Amoura Z, Beigelman C, et al. Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease. Arthritis Rheum. 2008;54:184–191.
  • Launay D, Humbert M, Berezne A, et al. Clinical characteristics and survival in systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease. Chest. 2011;140:1016–1024.
  • Volkmann ER, Saggar R, Khanna D, et al. Improved transplant-free survival in patients with systemic sclerosis-associated pulmonaru hypertension and interstitial lung disease. Arthritis Rheumatol. 2014;66:1900–1908.
  • Kinder BW, Collard HR, Koth L, et al. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med. 2007;176:691–697.
  • Fischer A, West SG, Swigris JJ, et al. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 2010;138:251–256.
  • Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011;140:1292–1299.
  • Kim H-C, Ji W, Kim MY, et al. Interstitial pneumonia related to undifferentiated connective tissue disease: pathologic pattern and prognosis. Chest. 2015;147:165–172.
  • Kondoh Y, Johkoh T, Fukuoka J, et al. Broader criteria of undifferentiated connective tissue disease in idiopathic interstitial pneumonias. Respir Med. 2015;109:389–396.
  • Alhamad EH, Al-Kassimi FA, Alboukai AA, et al. Comparison of three groups of patients with usual interstitial pneumonia. Respir Med. 2012;106:1575–1585.
  • Omote N, Taniguchi H, Kondoh Y, et al. Lung-dominant connective tissue disease: clinical, radiologic, and histologic features. Chest. 2015;148:1438–1446.
  • Oldham JM, Adegunsoye A, Valenzi E, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47:1767–1775.
  • Ahmad K, Barba T, Gamondes D, et al. Interstitial pneumonia with autoimmune features: clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. Respir Med. 2017;123:56–62.
  • Chartrand S, Swigris JJ, Stanchev L, et al. Clinical features and natural history of interstitial pneumonia with autoimmune features: a single center experience. Respir Med. 2016;119:150–154.

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.