Advertisement

Myositis-Related Interstitial Lung Diseases: Diagnostic Features, Treatment, and Complications

  • Courtney L. Shappley
  • Julie J. Paik
  • Lesley Ann SaketkooEmail author
Other CTD: Inflammatory Myopathies and Sjögren's (P Basharat, Section Editor)
First Online:
  • 1 Downloads
Part of the following topical collections:
  1. Topical Collection on Other CTD: Inflammatory Myopathies and Sjogren's

Abstract

Purpose of the review

Idiopathic inflammatory myopathies (IIMs) are a group of rare, heterogeneous connective tissue diseases marked by skeletal muscle inflammation. The four main forms of IIMs include dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM), and immune-mediated necrotizing myopathy (IMNM). Anti-synthetase syndrome (ASyS) is a very important classification of the IIM phenotypical spectrum and can be associated with rapidly progressive pulmonary disease. Each form of myositis, except inclusion body myositis, can be associated with multisystem organ involvement including pulmonary, cardiac, arthritis, and skin.

Recent findings

The lung in DM, PM and ASyS is the most common extra-muscular organ in IIM with a spectrum ranging from mild to fatal interstitial lung disease (ILD) with or without muscle involvement. Given that there is a subset of myositis patients with rapidly progressive ILD, it is important that rheumatologists in particular assess for lung involvement in all patients with myositis. In recent years, serologic testing for myositis autoantibodies has been more readily available allowing clinicians to order these tests commercially. The evaluation of a patient with myositis not only includes clinical phenotyping but also close observation to tendency for and rapidity of lung progression as well as serologic phenotyping with autoantibodies which can guide the treatment plan and prognosis of patients with ILD.

Summary

Herein, essential aspects of recognition, diagnosis, monitoring of progression, scope of treatment and IIM-ILD related complications are discussed.

Keywords

Idiopathic inflammatory myopathies Myosits Anti-synthetase Interstitial lung disease Restrictive lung disease Polymyositis Dermatomyositis Immune-mediated necrotizing myopathy 
This is a preview of subscription content, log in to check access.

Notes

Compliance with Ethical Standards

Conflict of Interest

Courtney Shappley, Julie J. Paik, and Lesley Ann Saketkoo each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

  1. 1.
    Saketkoo LA, Ascherman DP, Cottin V, Christopher-Stine L, K. Danoff S, Oddis CV. Interstitial lung disease in idiopathic inflammatory myopathy. Curr Rheumatol Rev. 2010;6:108–19.PubMedPubMedCentralCrossRefGoogle Scholar
  2. 2.
    Chen I-J, Jan Wu Y-J, Lin C-W, Fan KW, Luo SF, Ho HH, et al. Interstitial lung disease in polymyositis and dermatomyositis. Clin Rheumatol. 2009;28:639–46.  https://doi.org/10.1007/s10067-009-1110-6.CrossRefPubMedGoogle Scholar
  3. 3.
    Hayashi S, Tanaka M, Kobayashi H, Nakazono T, Satoh T, Fukuno Y, et al. High-resolution computed tomography characterization of interstitial lung diseases in polymyositis/dermatomyositis. J Rheumatol. 2008;35:260–9.PubMedGoogle Scholar
  4. 4.
    Yu K-H, Wu Y-JJ, Kuo C-F, See LC, Shen YM, Chang HC, et al. Survival analysis of patients with dermatomyositis and polymyositis: analysis of 192 Chinese cases. Clin Rheumatol. 2011;30:1595–601.  https://doi.org/10.1007/s10067-011-1840-0.CrossRefPubMedGoogle Scholar
  5. 5.
    Ji S, Zeng F, Guo Q, Tan GZ, Tang HF, Luo YJ, et al. Predictive factors and unfavourable prognostic factors of interstitial lung disease in patients with polymyositis or dermatomyositis: a retrospective study. Chin Med J. 2010;123:517–22.PubMedGoogle Scholar
  6. 6.
    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;63:297–301.PubMedPubMedCentralCrossRefGoogle Scholar
  7. 7.
    Chua F, Higton AM, Colebatch AN, O'Reilly K, Grubnic S, Vlahos I, et al. Idiopathic inflammatory myositis-associated interstitial lung disease: ethnicity differences and lung function trends in a British cohort. Rheumatol Oxf Engl. 2012;51:1870–6.  https://doi.org/10.1093/rheumatology/kes167.CrossRefGoogle Scholar
  8. 8.
    Lee C-S, Chen T-L, Tzen C-Y, Lin FJ, Peng MJ, Wu CL, et al. Idiopathic inflammatory myopathy with diffuse alveolar damage. Clin Rheumatol. 2002;21:391–6.  https://doi.org/10.1007/s100670200104.CrossRefPubMedGoogle Scholar
  9. 9.
    Johnson C, Pinal-Fernandez I, Parikh R, Paik J, Albayda J, Mammen AL, et al. Assessment of mortality in autoimmune myositis with and without associated interstitial lung disease. Lung. 2016;194:733–7.  https://doi.org/10.1007/s00408-016-9896-x.CrossRefPubMedGoogle Scholar
  10. 10.
    Koreeda Y, Higashimoto I, Yamamoto M, Takahashi M, Kaji K, Fujimoto M, et al. Clinical and pathological findings of interstitial lung disease patients with anti-aminoacyl-tRNA synthetase autoantibodies. Intern Med Tokyo Jpn. 2010;49:361–9.CrossRefGoogle Scholar
  11. 11.
    Marie I, Josse S, Hatron PY, Dominique S, Hachulla E, Janvresse A, et al. Interstitial lung disease in anti-Jo-1 patients with antisynthetase syndrome. Arthritis Care Res. 2013;65:800–8.  https://doi.org/10.1002/acr.21895.CrossRefGoogle Scholar
  12. 12.
    Svensson J, Holmqvist M, Lundberg IE, Arkema EV. Infections and respiratory tract disease as risk factors for idiopathic inflammatory myopathies: a population-based case-control study. Ann Rheum Dis. 2017;76:1803–8.  https://doi.org/10.1136/annrheumdis-2017-211174.CrossRefPubMedGoogle Scholar
  13. 13.
    Tillie-Leblond I, Wislez M, Valeyre D, Crestani B, Rabbat A, Israel-Biet D, et al. Interstitial lung disease and anti-Jo-1 antibodies: difference between acute and gradual onset. Thorax. 2008;63:53–9.  https://doi.org/10.1136/thx.2006.069237.CrossRefPubMedGoogle Scholar
  14. 14.
    Pinal-Fernandez I, Casal-Dominguez M, Huapaya JA, Albayda J, Paik JJ, Johnson C, et al. A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies. Rheumatol Oxf Engl. 2017;56:999–1007.  https://doi.org/10.1093/rheumatology/kex021.CrossRefGoogle Scholar
  15. 15.
    Gono T, Kawaguchi Y, Kuwana M, Sugiura T, Furuya T, Takagi K, et al. Brief report: association of HLA-DRB1*0101/*0405 with susceptibility to anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis in the Japanese population. Arthritis Rheum. 2012;64:3736–40.  https://doi.org/10.1002/art.34657.CrossRefPubMedGoogle Scholar
  16. 16.
    Sato S, Hoshino K, Satoh T, Fujita T, Kawakami Y, Fujita 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;60:2193–200.  https://doi.org/10.1002/art.24621.CrossRefPubMedGoogle Scholar
  17. 17.
    Hall JC, Casciola-Rosen L, Samedy L-A, Werner J, Owoyemi K, Danoff SK, et al. Anti-melanoma differentiation-associated protein 5-associated dermatomyositis: expanding the clinical spectrum. Arthritis Care Res. 2013;65:1307–15.  https://doi.org/10.1002/acr.21992.CrossRefGoogle Scholar
  18. 18.
    Candore G, Lio D, Colonna Romano G, Caruso C. Pathogenesis of autoimmune diseases associated with 8.1 ancestral haplotype: effect of multiple gene interactions. Autoimmun Rev. 2002;1:29–35.PubMedCrossRefGoogle Scholar
  19. 19.
    Miller FW, Cooper RG, Vencovský J, Rider LG, Danko K, Wedderburn LR, et al. Genome-wide association study of dermatomyositis reveals genetic overlap with other autoimmune disorders. Arthritis Rheum. 2013;65:3239–47.  https://doi.org/10.1002/art.38137.CrossRefPubMedPubMedCentralGoogle Scholar
  20. 20.
    Miller FW, Chen W, O’Hanlon TP, et al. Genome-wide association study identifies HLA 8.1 ancestral haplotype alleles as major genetic risk factors for myositis phenotypes. Genes Immun. 2015;16:470–80.  https://doi.org/10.1038/gene.2015.28.CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Rothwell S, Cooper RG, Lundberg IE, Miller FW, Gregersen PK, Bowes J, et al. Dense genotyping of immune-related loci in idiopathic inflammatory myopathies confirms HLA alleles as the strongest genetic risk factor and suggests different genetic background for major clinical subgroups. Ann Rheum Dis. 2016;75:1558–66.  https://doi.org/10.1136/annrheumdis-2015-208119.CrossRefPubMedGoogle Scholar
  22. 22.
    O’Hanlon TP, Carrick DM, Targoff IN, et al. Immunogenetic risk and protective factors for the idiopathic inflammatory myopathies: distinct HLA-A, -B, -Cw, -DRB1, and -DQA1 allelic profiles distinguish European American patients with different myositis autoantibodies. Medicine (Baltimore). 2006;85:111–27.  https://doi.org/10.1097/01.md.0000217525.82287.eb.CrossRefGoogle Scholar
  23. 23.
    Chinoy H, Salway F, Fertig N, Shephard N, Tait BD, Thomson W, et al. In adult onset myositis, the presence of interstitial lung disease and myositis specific/associated antibodies are governed by HLA class II haplotype, rather than by myositis subtype. Arthritis Res Ther. 2006;8:R13.  https://doi.org/10.1186/ar1862.CrossRefPubMedGoogle Scholar
  24. 24.
    Chen Z, Wang Y, Kuwana M, Xu X, Hu W, Feng X, et al. HLA-DRB1 alleles as genetic risk factors for the development of anti-MDA5 antibodies in patients with dermatomyositis. J Rheumatol. 2017;44:1389–93.  https://doi.org/10.3899/jrheum.170165.CrossRefPubMedGoogle Scholar
  25. 25.
    Hervier B, Devilliers H, Stanciu R, Meyer A, Uzunhan Y, Masseau A, et al. Hierarchical cluster and survival analyses of antisynthetase syndrome: phenotype and outcome are correlated with anti-tRNA synthetase antibody specificity. Autoimmun Rev. 2012;12:210–7.  https://doi.org/10.1016/j.autrev.2012.06.006.CrossRefPubMedGoogle Scholar
  26. 26.
    Hamaguchi Y, Fujimoto M, Matsushita T, Kaji K, Komura K, Hasegawa M, 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.  https://doi.org/10.1371/journal.pone.0060442.CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Marie I, Josse S, Decaux O, Dominique S, Diot E, Landron C, et al. Comparison of long-term outcome between anti-Jo1- and anti-PL7/PL12 positive patients with antisynthetase syndrome. Autoimmun Rev. 2012;11:739–45.  https://doi.org/10.1016/j.autrev.2012.01.006.CrossRefPubMedGoogle Scholar
  28. 28.
    Marie I, Hatron PY, Dominique S, Cherin P, Mouthon L, Menard JF, et al. Short-term and long-term outcome of anti-Jo1-positive patients with anti-Ro52 antibody. Semin Arthritis Rheum. 2012;41:890–9.  https://doi.org/10.1016/j.semarthrit.2011.09.008.CrossRefPubMedGoogle Scholar
  29. 29.
    Cavagna L, Nuño L, Scirè CA, Govoni M, Longo FJ, Franceschini F, et al. Clinical spectrum time course in anti Jo-1 positive antisynthetase syndrome: results from an international retrospective multicenter study. Medicine (Baltimore). 2015;94:e1144.  https://doi.org/10.1097/MD.0000000000001144.CrossRefGoogle Scholar
  30. 30.
    González-Gay MA, Montecucco C, Selva-O’Callaghan A, et al. Timing of onset affects arthritis presentation pattern in antisyntethase syndrome. Clin Exp Rheumatol. 2018;36:44–9.PubMedGoogle Scholar
  31. 31.
    Cavagna L, Nuño L, Scirè CA, et al. Serum Jo-1 autoantibody and isolated arthritis in the antisynthetase syndrome: review of the literature and report of the experience of AENEAS collaborative group. Clin Rev Allergy Immunol. 2017;52:71–80.  https://doi.org/10.1007/s12016-016-8528-9.CrossRefPubMedGoogle Scholar
  32. 32.
    Noguchi E, Uruha A, Suzuki S, Hamanaka K, Ohnuki Y, Tsugawa J, et al. Skeletal muscle involvement in antisynthetase syndrome. JAMA Neurol. 2017;74:992–9.  https://doi.org/10.1001/jamaneurol.2017.0934.CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Marie I, Josse S, Decaux O, Diot E, Landron C, Roblot P, et al. Clinical manifestations and outcome of anti-PL7 positive patients with antisynthetase syndrome. Eur J Intern Med. 2013;24:474–9.  https://doi.org/10.1016/j.ejim.2013.01.002.CrossRefPubMedGoogle Scholar
  34. 34.
    Chaisson NF, Paik J, Orbai A-M, Casciola-Rosen L, Fiorentino D, Danoff S, et al. A novel dermato-pulmonary syndrome associated with MDA-5 antibodies: report of 2 cases and review of the literature. Medicine (Baltimore). 2012;91:220–8.  https://doi.org/10.1097/MD.0b013e3182606f0b.CrossRefGoogle Scholar
  35. 35.
    Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad Dermatol. 2011;65:25–34.  https://doi.org/10.1016/j.jaad.2010.09.016.CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Cao H, Pan M, Kang Y, Xia Q, Li X, Zhao X, et al. Clinical manifestations of dermatomyositis and clinically amyopathic dermatomyositis patients with positive expression of anti-melanoma differentiation-associated gene 5 antibody. Arthritis Care Res. 2012;64:1602–10.  https://doi.org/10.1002/acr.21728.CrossRefGoogle Scholar
  37. 37.
    Koga T, Fujikawa K, Horai Y, Okada A, Kawashiri SY, Iwamoto N, et al. The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM. Rheumatol Oxf Engl. 2012;51:1278–84.  https://doi.org/10.1093/rheumatology/ker518.CrossRefGoogle Scholar
  38. 38.
    Chen F, Wang D, Shu X, Nakashima R, Wang G. Anti-MDA5 antibody is associated with A/SIP and decreased T cells in peripheral blood and predicts poor prognosis of ILD in Chinese patients with dermatomyositis. Rheumatol Int. 2012;32:3909–15.  https://doi.org/10.1007/s00296-011-2323-y.CrossRefPubMedPubMedCentralGoogle Scholar
  39. 39.
    Lega J-C, Fabien N, Reynaud Q, Durieu I, Durupt S, Dutertre M, et al. The clinical phenotype associated with myositis-specific and associated autoantibodies: a meta-analysis revisiting the so-called antisynthetase syndrome. Autoimmun Rev. 2014;13:883–91.  https://doi.org/10.1016/j.autrev.2014.03.004.CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Ferreira JP, Almeida I, Marinho A, Cerveira C, Vasconcelos C. Anti-ro52 antibodies and interstitial lung disease in connective tissue diseases excluding scleroderma. ISRN Rheumatol. 2012;2012:415272–4.  https://doi.org/10.5402/2012/415272.CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Rutjes SA, Vree Egberts WT, Jongen P, et al. Anti-Ro52 antibodies frequently co-occur with anti-Jo-1 antibodies in sera from patients with idiopathic inflammatory myopathy. Clin Exp Immunol. 1997;109:32–40.PubMedPubMedCentralCrossRefGoogle Scholar
  42. 42.
    La Corte R, Lo Mo Naco A, Locaputo A, et al. In patients with antisynthetase syndrome the occurrence of anti-Ro/SSA antibodies causes a more severe interstitial lung disease. Autoimmunity. 2006;39:249–53.  https://doi.org/10.1080/08916930600623791.CrossRefPubMedGoogle Scholar
  43. 43.
    Rigolet A, Musset L, Dubourg O, Maisonobe T, Grenier P, Charuel JL, et al. Inflammatory myopathies with anti-Ku antibodies: a prognosis dependent on associated lung disease. Medicine (Baltimore). 2012;91:95–102.  https://doi.org/10.1097/MD.0b013e31824d9cec.CrossRefGoogle Scholar
  44. 44.
    Fathi M, Vikgren J, Boijsen M, et al. Interstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and radiology. Arthritis Rheum. 2008;59(5):677–85.PubMedCrossRefGoogle Scholar
  45. 45.
    Clawson K, Oddis CV. Adult respiratory distress syndrome in polymyositis patients with the antiJo-1 antibody. Arthritis Rheum. 1995;38(10):1519–23.PubMedCrossRefGoogle Scholar
  46. 46.
    Guglielmi S, Merz TM, Gugger M, Suter C, Nicod LP. Acute respiratory distress syndrome secondary to antisynthetase syndrome is reversible with tacrolimus. Eur Respir J. 2008;31(1):213–7.PubMedCrossRefGoogle Scholar
  47. 47.
    Polosa R, Di Mauro C, Spampinato B, et al. A patient with antihistidyl-tRNA synthetase positive polymyositis presenting as acute respiratory distress syndrome. J Clin Rheumatol. 2008;14(4):219–21.PubMedCrossRefGoogle Scholar
  48. 48.
    Saketkoo LA, Mittoo S, Frankel S, LeSage D, Sarver C, Phillips K, et al. Reconciling healthcare professional and patient perspectives in the development of disease activity and response criteria in connective tissue disease-related interstitial lung diseases. J Rheumatol. 2014 Apr;41(4):792–8.PubMedPubMedCentralCrossRefGoogle Scholar
  49. 49.
    Richards TJ, Eggebeen A, Gibson K, Yousem S, Fuhrman C, Gochuico BR, et al. Characterization and peripheral blood biomarker assessment of anti-Jo-1 antibody-positive interstitial lung disease. Arthritis Rheum. 2009;60(7):2183–92.PubMedPubMedCentralCrossRefGoogle Scholar
  50. 50.
    Mittoo S, Frankel S, LeSage D, Strand V, Shah AA, Christopher-Stine L, 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(2):175–83.PubMedPubMedCentralCrossRefGoogle Scholar
  51. 51.
    Hirakata M, Nagai S. Interstitial lung disease in polymyositis and dermatomyositis. Opin Rheumatol. 2000;12:501–8.CrossRefGoogle Scholar
  52. 52.
    Morisset J, Johnson C, Rich E, Collard H, Lee J. Management of myositis-related interstitial lung disease. CHEST. 2016;150(5):1118–28.PubMedCrossRefGoogle Scholar
  53. 53.
    Miller SA, Glassberg MK, Ascherman DP. Pulmonary complications of inflammatory myopathy. Rheum Dis Clin N Am. 2015;41(2):249–62.CrossRefGoogle Scholar
  54. 54.
    Mimori T, Nakashima R, Hosono Y. Interstitial lung disease in myositis: clinical subsets, biomarkers, and treatment. Curr Rheumatol Rep. 2012;14(3):264–74.PubMedCrossRefGoogle Scholar
  55. 55.
    Torres C, Belmonte R, Carmona L, Gómez-Reino FJ, Galindo M, Ramos B, et al. Survival, mortality and causes of death in inflammatory myopathises. Autoimmunity. 2006;39(3):205–15.PubMedCrossRefGoogle Scholar
  56. 56.
    Danko K, Ponyi A, Constantin T, Borgulya G, Szegedi G. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases. Medicine. 2004;83(1):35–42.PubMedCrossRefGoogle Scholar
  57. 57.
    Fujisawa T, Hozumi H, Kono M, et al. Prognostic factors for myositis associated interstitial lung disease. PloS One. 2014;9(6):e98824.PubMedPubMedCentralCrossRefGoogle Scholar
  58. 58.
    Castelino FV, Goldberg H, Dellaripa PF. The impact of rheumatological evaluation in the management of patients with interstitial lung disease. Rheumatology (Oxford). 2011 Mar;50(3):489–93.CrossRefGoogle Scholar
  59. 59.
    Conchotome Muscle Biopsy. https://www.youtube.com/watch?v=Dma7uv0rRik. Last accessed 11 July 2018.
  60. 60.
    Hozumi H, Fujisawa T, Nakashima R, Johkoh T, et al. Comprehensive assessment of myositis-specific autoantibodies in polymyositis/dermatomyositis-associated interstitial lung disease. Respir Med. 2016;121:91–9.PubMedCrossRefGoogle Scholar
  61. 61.
    Hoshino K, Muro Y, Sugiura K, Tomita Y, Nakashima R, Mimori T. Anti-MDA5 and anti-TIF1-gamma antibodies have clinical significance for patients with dermatomyositis. Rheumatology (Oxford). 2010;49:1726–33.CrossRefGoogle Scholar
  62. 62.
    Cavagna L, et al. Serum Jo-1 Autoantibody and isolated arthritis in the antisynthetase syndrome: review of the literature and report of the experience of AENEAS collaborative group. Clinic Rev Allerg Immunol. 2017;52:71–80.CrossRefGoogle Scholar
  63. 63.
    Gonzalez-Gay MA, et al. Timing of onset affects arthritis presentation pattern in antisynthetase syndrome. Clin Exp Rheumatol. 2018;36:44–9.PubMedGoogle Scholar
  64. 64.
    Fernandez-Pinal I, Casal-Dominguez M, Huapaya JA, Albayda J, Paik JJ, Johnson C, et al. A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies. Rheumatology (Oxford). 2017;56(6):999–1007.CrossRefGoogle Scholar
  65. 65.
    Sugiyama Y, Yoshimi R, Tamura M, Takeno M, Kunishita Y, Kishimoto D, et al. The predictive prognostic factors for polymyositis/dermatomyositis associated interstitial lung disease. Arthritis Res Ther. 2018;20(1):7.PubMedPubMedCentralCrossRefGoogle Scholar
  66. 66.
    Mejia M, Herrera-Bringas D, Perez-Roman DI, Rivero H, Mateos-Toledo H, Castorena-Garcia P, et al. Interstitial lung disease and myositis-specific and associated autoantibodies: clinical manifestations, survival and the performance of the new ATS/ERS criteria for interstitial pneumonia with autoimmune features (IPAF). Respir Med. 2017;123:79–86.PubMedCrossRefGoogle Scholar
  67. 67.
    Vancsa A, Csipo I, Nemeth J, Devenyi K, Gergely L, Danko K. Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients. Rheumatol Int. 2009;29(9):989–94.PubMedCrossRefGoogle Scholar
  68. 68.
    Obert J, Freynet O, Nunes H, Brillet PY, Miyara M, Dhote R, et al. Outcomes and prognostic factors in a French cohort of patients with myositis-associated interstitial lung disease. Rheumatol Int. 2016;36(12):1727–35.PubMedCrossRefGoogle Scholar
  69. 69.
    Chen F, Li S, Wang T, Shi J, Wang G. Clinical heterogeneity of interstitial lung disease in polymyositis and dermatomyositis patients with or without specific autoantibodies. Am J Med Sci. 2018;355(1):48–53.PubMedCrossRefGoogle Scholar
  70. 70.
    Visscher DW, Myers JL. Histologic spectrum of idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006;3(4):322–9.PubMedCrossRefGoogle Scholar
  71. 71.
    Flaherty KR, Colby TV, Travis WD, Toews GB, Mumford J, Murray S, et al. Fibroblastic foci in usual interstitial pneumonia: idiopathic versus collagen vascular disease. Am J Respir Crit Care Med. 2003;167:1410–5.PubMedCrossRefGoogle Scholar
  72. 72.
    Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P, et al. Histopathological subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med. 2002;165:1581–6.PubMedCrossRefGoogle Scholar
  73. 73.
    Marie I, Hatron PY, Dominique S, et al. Short-term and long-term outcomes of interstitial lung disease in polymyositis and dermatomyositis: a series of 107 patients. Arthritis Rheum. 2011;63(11):3439–46.PubMedCrossRefGoogle Scholar
  74. 74.
    Lee JW, Lee KS, Lee HY, Chung MP, et al. Cryptogenic organizing pneumonia: serial high-resolution CT findings in 22 patients. Am J Roentgenol. 2010;195(4):916–22.CrossRefGoogle Scholar
  75. 75.
    Bonnefoy O, Ferretti G, Calaque O, Coulomb M, Begueret H, Beylot-Barry M, et al. Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis. Eur J Radiol. 2004;49(3):235–44.PubMedCrossRefGoogle Scholar
  76. 76.
    Mukae H, Ishimoto H, Sakamoto N, Hara S, Kakugawa T, Nakayama S, et al. Clinical differences between interstitial lung disease associated with clinically amyopathic dermatomyositis and classic dermatomyositis. Chest. 2009;136(5):1341–7.PubMedCrossRefGoogle Scholar
  77. 77.
    Ye S, Chen XX, Lu XY, Wu MF, Deng Y, Huang WQ, et al. Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study. Clin Rheumatol. 2007;26(10):1647–54.PubMedCrossRefGoogle Scholar
  78. 78.
    Woodhead F, Wells AU, Desai SR. Pulmonary complications of connective tissue diseases. Clin Chest Med. 2008;29(1):149–64.PubMedCrossRefGoogle Scholar
  79. 79.
    Wells AU, Desai SR, Rubens MB, Goh NSL, Cramer D, Nicholson AG, et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med. 2003;167:962–9.PubMedCrossRefGoogle Scholar
  80. 80.
    Marie I, Hachulla E, Chérin P, Dominique S, Hatron PY, Hellot MF, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002;47(6):614–22.PubMedCrossRefGoogle Scholar
  81. 81.
    Suda T, Fujisawa T, Enomoto N, Nakamura Y, Inui N, Naito T, et al. Interstitial lung diseases associated with amyopathic dermatomyositis. Euro Resp J. 2006;28(5):1005–12.CrossRefGoogle Scholar
  82. 82.
    Sriprasart T, Aragaki A, Baughman R, Wikenheiser-Brokamp K, Khanna G, Tanase D, et al. A single US center Experience of transbronchial lung cryobiopsy for diagnosing interstitial lung disease with a 2-scope technique. J Bronchology Interv Pulmonol. 2017;24(2):131–5.PubMedPubMedCentralCrossRefGoogle Scholar
  83. 83.
    Deconinck B, Verschakenlen J, Coolen J, et al. Diagnostic workup for diffuse parenchymal lung disease: schematic flowchart, literature review and pitfalls. Lung. 2013;191:19–25.PubMedCrossRefGoogle Scholar
  84. 84.
    Kreider ME, Hansen-Flaschen J, Ahmad NN, et al. Complications of video-assisted thorascopic lung biopsy in patients with interstitial lung disease. Ann Thorac Surg. 2007;83(3):1120–45.CrossRefGoogle Scholar
  85. 85.
    Park JH, Kim DK, Kim DS, et al. Mortality and risk factors for surgical lung biopsy in patients with idiopathic interstitial pneumonia. Eur J Cardiothorac Surg. 2007;31(6):1115–9.PubMedCrossRefGoogle Scholar
  86. 86.
    Hyldgaard C, Bendstrup E, Wells AU, et al. Unclassifiable interstitial lung diseases: clinical characteristics and survival. Respirology. 2017;22:494–500.PubMedCrossRefGoogle Scholar
  87. 87.
    Poletti V, Patelli M, Poggi S, et al. Transbronchial lung biopsy and bronchoalveolar lavage in diagnosis of diffuse infiltrative lung diseases. Respiration. 1988;54:66–72.PubMedCrossRefGoogle Scholar
  88. 88.
    Pajares V, Puzo C, Castillo D, et al. Diagnostic yield of transbronchial cryobiopsy in interstitial lung disease: a randomized trial. Respirology. 2014;19(6):900–6.PubMedCrossRefGoogle Scholar
  89. 89.
    Kropski J, Pritchett J, Mason W, et al. Bronchoscopic cryobiopsy for the diagnosis of diffuse parenchymal lung disease. Plos One. 2014;8:e78674.CrossRefGoogle Scholar
  90. 90.
    Braun NM, Arora NS, Rochester DF. Respiratory muscle and pulmonary function in polymyositis and other proximal myopathies. Thorax. 1983;38(8):616–23.PubMedPubMedCentralCrossRefGoogle Scholar
  91. 91.
    Chen J, Nguyen N, Soong M, Baydur A. Postural change of FVC in patients with neuromuscular disease: relation to initiating non-invasive ventilation. J Pulmon Resp Med. 2013;3:147.CrossRefGoogle Scholar
  92. 92.
    Baydur A, Behrakis PK, Zin WA, Jaeger MJ, Weiner JM, et al. Effect of posture on ventilation and breathing pattern during room air breathing at rest. Lung 1987 (3); 165:341–351.Google Scholar
  93. 93.
    Hill NS, Liesching T, Kwok H. Indications for non-invasive ventilation: update in intensive care medicine. Mechanical Ventilation. 2005.Google Scholar
  94. 94.
    Kawasumi H, Gono T, Kawaguchi Y, et al. IL-6, IL-8, and IL-10 are associated with hyperferritinemia in rapidly progressive interstitial lung disease as a complication of dermatomyositis. Biomed Res Int. 2014;2014:815245.PubMedPubMedCentralCrossRefGoogle Scholar
  95. 95.
    Gono T, Kawaguchi Y, Satoh T, et al. Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis. Rheumatology (Oxford). 2010;49(9):1213–719.CrossRefGoogle Scholar
  96. 96.
    Tanizawa K, Handa T, Nakashima R, Kubo T, Hosono Y, Aihara K, et al. The prognostic value of HRCT in myositis-associated interstitial lung disease. Resp Med. 2013;107(5):745–52.CrossRefGoogle Scholar
  97. 97.
    Gono T, Kawaguchi Y, Hara M, et al. Increased ferritin predicts development and severity of acute interstitial lung disease as a complication of dermatomyositis. Rheumatology (Oxford). 2010;49(7):1354–60.CrossRefGoogle Scholar
  98. 98.
    Kubo M, Ihn H, Kikuchi K, et al. Serum KL-6 in adult patients with polymyositis and dermatomyositis. Rheumatology. 2000;39(6):632–6.PubMedCrossRefGoogle Scholar
  99. 99.
    Fathi M, Barbasso HS, Lundberg I. KL-6: a serological biomarker for interstitial lung disease in patients with polymyositis and dermatomyositis. J Intern Med. 2012;271(6):589–97.PubMedCrossRefGoogle Scholar
  100. 100.
    Nakayama M, Satoh H, Ishikawa H, Fujiwara M, Kamma H, Ohtsuka M, et al. Cytokeratin 19 fragment in patients with nonmalignant respiratory diseases. Chest. 2003;123(6):2001–6.PubMedCrossRefGoogle Scholar
  101. 101.
    Satoh H, Kurishima K, Ishikawa H, Ohtsuka M. Increased levels of KL-6 and subsequent mortality in patients with interstitial lung diseases. J Intern Med. 2006;260(5):429–34.PubMedCrossRefGoogle Scholar
  102. 102.
    Travis WD, Costabel U, Hansell DM, et al. 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(6):733–48.PubMedPubMedCentralCrossRefGoogle Scholar
  103. 103.
    Redondo-Benito A, Curran A, Villar-Gomez A, Trallero-Araguas E, Fernandez-Codina A, Pinal-Fernandez I, et al. Opportunistic infections in patients with idiopathic inflammatory myopathies. Int J Rheum Dis. 2018;21(2):487–96.PubMedCrossRefGoogle Scholar
  104. 104.
    Chen IJ, Tsai WP, Wu YJ, et al. Infections in polymyositis and dermatomyositis: analysis of 192 cases. Rheumatology (Oxford). 2010;49(12):2429–37.CrossRefGoogle Scholar
  105. 105.
    Marie I, Hachulla E, Gherin P, Hellot M-F, Herson S, Levesque H, et al. Opportunistic infections in polymyositis and dermatomyositis. Arthritis Rheum. 2005;53(2):155–65.PubMedCrossRefGoogle Scholar
  106. 106.
    Ng KP, Ramos F, Sultan SM, Isenberg DA. Concomitant diseases in a cohort of patients with idiopathic myositis during long-term follow-up. Clin Rheumatol. 2009;28(8):947–53.PubMedCrossRefGoogle Scholar
  107. 107.
    Murray S, Schmajuk G, Trupin L, Lawson E, Cascino M, Barton J, Margaretten M, Katz P, Yelin E, Yazdany J. A population-based study of infection-related hospital mortality in patients with dermatomyositis/polymyositis. 2015; 67(5):673–680.Google Scholar
  108. 108.
    Ward MM, Donald F. Pneumocystis carinii pneumonia in patients with connective tissue diseases: the role of hospital experience in diagnosis and mortality. Arthritis Rheum. 1999;42(9):780–9.PubMedCrossRefGoogle Scholar
  109. 109.
    Godeau B, Cxoutant-Perrone V, Le Thi HD, et al. Pneumocystis carinii pneumonia in the course of connective tissue disease: report of 34 cases. J Rheumatol. 1994;21(2):246–51.PubMedGoogle Scholar
  110. 110.
    Kovacs JA, Hiemenz JW, Macher AM, et al. Pneumocystis carinii pneumonia: a comparison between patients with the acquired immunodeficiency syndrome and patients with other immunodeficiencies. Ann Intern Med. 1984;100(5):663–71.PubMedCrossRefGoogle Scholar
  111. 111.
    Mori S, Sugimoto M. Pneumocystis jirovecii infection: an emerging threat to patients with rheumatoid arthritis. Rheumatology (Oxford). 2012;51(12):2120–30.CrossRefGoogle Scholar
  112. 112.
    Marie I, Menard JF, Hachulla E, et al. Infectious complications in polymyositis and dermatomyositis: a series of 279 patients. Semin Arthritis Rheum. 2011;41:48–60.PubMedCrossRefGoogle Scholar
  113. 113.
    Tsai SY, Lin CL, Wong YC, et al. Increased risk of herpes zoster following dermatomyositis and polymyositis: a nationwide population-based cohort study. Medicine (Baltimore). 2015;94(37):e1138.CrossRefGoogle Scholar
  114. 114.
    Takizawa Y, Inoduma S, Tanaka Y, et al. Clinical characteristics of cytomegalovirus infection in rheumatic diseases: multicenter survey in a large patient population. Rheumatology (Oxford). 2008;47(9):1373–8.CrossRefGoogle Scholar
  115. 115.
    Barcat D, Mercie P, Constans J, Traissac T, le Clouerec G, Texier-Maugein J, et al. Disseminated mycobacterium avium complex infection associated with bifocal synovitis in a patient with dermatomyositis. Clin Infect Dis. 1998;26:1004–5.PubMedCrossRefGoogle Scholar
  116. 116.
    Selva-O’Callaghan A, Sanches-Sitjes L, Munoz-Gall X, et al. Respiratory failure due to muscle weakness in inflammatory myopathies: maintenance therapy with home mechanical ventilation. Rheumatology (Oxford). 2000;39(8):914–6.CrossRefGoogle Scholar
  117. 117.
    Selva-O’Callaghan A, Labrador-Horrillo M, Muñoz-Gall X, Martínez-Gomez X, Majó-Masferrer J, Solans-Laque R, et al. Polymyositis/dermatomyositis-associated lung disease: analysis of a series of 81 patients. Lupus. 2005;14(7):534–42.PubMedCrossRefGoogle Scholar
  118. 118.
    Le Goff B, Cherin P, Cantagrel A, et al. Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Rheum. 2009;61(1):108–18.PubMedCrossRefGoogle Scholar
  119. 119.
    Imokawa S, Colby T, Leslie K, et al. Methotrexate pneumonitis: review of the literature and histopathological findings in nine patients. Eur Respir J. 2000;15(2):373.PubMedCrossRefGoogle Scholar
  120. 120.
    Hadjinicolaou AV, Nisar MK, Bhagat S, et al. Non-infectious pulmonary complications of newer biological agents for rheumatic diseases-a systematic literature review. Rheumatology (Oxford). 2011;50(12):2297–305.CrossRefGoogle Scholar
  121. 121.
    Hervier B, Myer A, Dieval C, et al. Pulmonary hypertension in antisynthetase syndrome: prevalence, aetiology and survival. Eur Respir J. 2013;42(5):1271–82.PubMedCrossRefGoogle Scholar
  122. 122.
    Denbow C, Lie J, Tancredi R, et al. Cardiac involvement in polymyositis: a clinicopathologic study of 20 autopsied patients. Arthritis Rheum. 1979;22(10):1088–92.PubMedCrossRefGoogle Scholar
  123. 123.
    Avouac J, Huscher D, Furst DE, Opitz CF, Distler O, Allonore Y, et al. Expert consensus for performing right heart catheterization for suspected pulmonary arterial hypertension in systemic sclerosis: a Delphi consensus study with cluster analysis. Ann Rheum Dis. 2014;73(1):191–7.PubMedCrossRefGoogle Scholar
  124. 124.
    Saketkoo LA, Espinoza LR. Experience of mycophenolate mofetil in 10 patients with autoimmune-related interstitial lung disease demonstrates promising effects. Am J Med Sci. 2009;337:329–35.  https://doi.org/10.1097/MAJ.0b013e31818d094b.CrossRefPubMedGoogle Scholar
  125. 125.
    Hervier B, Masseau A, Mussini J-M, Audrain M, Hamidou MA. Long-term efficacy of mycophenolate mofetil in a case of refractory antisynthetase syndrome. Jt Bone Spine Rev Rhum. 2009;76:575–6.  https://doi.org/10.1016/j.jbspin.2009.02.004.CrossRefGoogle Scholar
  126. 126.
    Swigris JJ, Olson AL, Fischer A, et al. Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease. Chest. 2006;130:30–6.  https://doi.org/10.1378/chest.130.1.30.CrossRefPubMedGoogle Scholar
  127. 127.
    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;40:640–6.  https://doi.org/10.3899/jrheum.121043.CrossRefPubMedPubMedCentralGoogle Scholar
  128. 128.
    Mira-Avendano IC, Parambil JG, Yadav R, Arrossi V, Xu M, Chapman JT, et al. A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis/dermatomyositis. Respir Med. 2013;107:890–6.  https://doi.org/10.1016/j.rmed.2013.02.015.CrossRefPubMedGoogle Scholar
  129. 129.
    Casal-Dominguez M, Pinal-Fernandez I, Huapaya J, et al. Efficacy and adverse effects of methotrexate compared with azathioprine in the antisynthetase syndrome. In: Abstract Poster. San Diego: 2017.Google Scholar
  130. 130.
    Kurita T, Yasuda S, Oba K, Odani T, Kono M, Otomo K, et al. The efficacy of tacrolimus in patients with interstitial lung diseases complicated with polymyositis or dermatomyositis. Rheumatol Oxf Engl. 2015;54:39–44.  https://doi.org/10.1093/rheumatology/keu166.CrossRefGoogle Scholar
  131. 131.
    Labirua-Iturburu A, Selva-O’Callaghan A, Martínez-Gómez X, et al. Calcineurin inhibitors in a cohort of patients with antisynthetase-associated interstitial lung disease. Clin Exp Rheumatol. 2013;31:436–9.PubMedGoogle Scholar
  132. 132.
    Cavagna L, Caporali R, Abdì-Alì L, et al. Cyclosporine in anti-Jo1-positive patients with corticosteroid-refractory interstitial lung disease. J Rheumatol. 2013;40:484–92.  https://doi.org/10.3899/jrheum.121026.CrossRefPubMedGoogle Scholar
  133. 133.
    Ge Y, Peng Q, Zhang S, Zhou H, Lu X, Wang G. Cyclophosphamide treatment for idiopathic inflammatory myopathies and related interstitial lung disease: a systematic review. Clin Rheumatol. 2015;34:99–105.  https://doi.org/10.1007/s10067-014-2803-z.CrossRefPubMedGoogle Scholar
  134. 134.
    Ingegnoli F, Lubatti C, Ingegnoli A, Boracchi P, Zeni S, Meroni PL. Interstitial lung disease outcomes by high-resolution computed tomography (HRCT) in anti-Jo1 antibody-positive polymyositis patients: a single centre study and review of the literature. Autoimmun Rev. 2012;11:335–40.  https://doi.org/10.1016/j.autrev.2011.09.007.CrossRefPubMedGoogle Scholar
  135. 135.
    Aggarwal R, Bandos A, Reed AM, Ascherman DP, Barohn RJ, Feldman BM, et al. Predictors of clinical improvement in rituximab-treated refractory adult and juvenile dermatomyositis and adult polymyositis. Arthritis Rheumatol Hoboken NJ. 2014;66:740–9.  https://doi.org/10.1002/art.38270.CrossRefGoogle Scholar
  136. 136.
    Oddis CV, Reed AM, Aggarwal R, Rider LG, Ascherman DP, Levesque MC, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum. 2013;65:314–24.  https://doi.org/10.1002/art.37754.CrossRefPubMedPubMedCentralGoogle Scholar
  137. 137.
    Sem M, Molberg O, Lund MB, Gran JT. Rituximab treatment of the anti-synthetase syndrome: a retrospective case series. Rheumatol Oxf Engl. 2009;48:968–71.  https://doi.org/10.1093/rheumatology/kep157.CrossRefGoogle Scholar
  138. 138.
    Allenbach Y, Guiguet M, Rigolet A, Marie I, Hachulla E, Drouot L, et al. Efficacy of rituximab in refractory inflammatory myopathies associated with anti-synthetase auto-antibodies: an open-label, phase II trial. PloS One. 2015;10:e0133702.  https://doi.org/10.1371/journal.pone.0133702.CrossRefPubMedPubMedCentralGoogle Scholar
  139. 139.
    Hallowell RW, Amariei D, Danoff SK. Intravenous immunoglobulin as potential adjunct therapy for interstitial lung disease. Ann Am Thorac Soc. 2016;13:1682–8.  https://doi.org/10.1513/AnnalsATS.201603-179PS.CrossRefPubMedGoogle Scholar
  140. 140.
    Suzuki Y, Hayakawa H, Miwa S, Shirai M, Fujii M, Gemma H, et al. Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis. Lung. 2009;187:201–6.  https://doi.org/10.1007/s00408-009-9146-6.CrossRefPubMedGoogle Scholar
  141. 141.
    Fernández-Díaz C, Loricera J, Castañeda S, et al. Abatacept in patients with rheumatoid arthritis and interstitial lung disease: a national multicenter study of 63 patients. Semin Arthritis Rheum Published Online First: 6 January 2018.  https://doi.org/10.1016/j.semarthrit.2017.12.012.
  142. 142.
    Hornung T, Janzen V, Heidgen F-J, Wolf D, Bieber T, Wenzel J. Remission of recalcitrant dermatomyositis treated with ruxolitinib. N Engl J Med. 2014;371:2537–8.  https://doi.org/10.1056/NEJMc1412997.CrossRefPubMedGoogle Scholar
  143. 143.
    Ladislau L, Suárez-Calvet X, Toquet S, Landon-Cardinal O, Amelin D, Depp M, et al. JAK inhibitor improves type I interferon induced damage: proof of concept in dermatomyositis. Brain J Neurol. 2018;141:1609–21.  https://doi.org/10.1093/brain/awy105.CrossRefGoogle Scholar
  144. 144.
    Kurtzman DJB, Wright NA, Lin J, et al. Tofacitinib citrate for refractory cutaneous dermatomyositis: an alternative treatment. JAMA Dermatol Published Online First: 27 April 2016.  https://doi.org/10.1001/jamadermatol.2016.0866.
  145. 145.
    Paik JJ, Christopher-Stine L. A case of refractory dermatomyositis responsive to tofacitinib. Semin Arthritis Rheum. 2017;46:e19.  https://doi.org/10.1016/j.semarthrit.2016.08.009.CrossRefPubMedGoogle Scholar
  146. 146.
    Kurasawa K, Arai S, Namiki Y, et al. Tofacitinib for refractory interstitial lung diseases in anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis. Rheumatol Oxf Engl Published Online First: 27 July 2018.  https://doi.org/10.1093/rheumatology/key188.
  147. 147.
    Dowman LM, McDonald CF, Hill CJ, Lee AL, Barker K, Boote C, et al. The evidence of benefits of exercise training in interstitial lung disease: a randomised controlled trial. Thorax. 2017;72(7):610–9.PubMedCrossRefGoogle Scholar
  148. 148.
    Alexanderson H. Physical exercise as a treatment for adult and juvenile myositis. J Intern Med. 2016 Jul;280(1):75–96.PubMedCrossRefGoogle Scholar
  149. 149.
    Alexanderson H, Munters LA, Dastmalchi M, Loell I, Heimbürger M, Opava CH, et al. Resistive home exercise in patients with recent-onset polymyositis and dermatomyositis -- a randomized controlled single-blinded study with a 2-year followup. J Rheumatol. 2014 Jun;41(6):1124–32.PubMedCrossRefGoogle Scholar
  150. 150.
    International Society for Heart and Lung Transplantation. JHLT 2017; 36(10):1037–1079.Google Scholar
  151. 151.
    Ameye H, Ruttens D, Benveniste O, Verieden GM, Wuyts WA. Is lung transplantation a valuable therapeutic option for patients with pulmonary polymyositis? Experiences from the Leuven transplant cohort. Transplant Proceedings. 2014;46:3147–53.CrossRefGoogle Scholar
  152. 152.
    Orens JB, et al. International guidelines for the selection of lung transplant candidates: 2006 update—a consensus report from the pulmonary scientific council of the international society for heart and lung transplantation. J Heart Lung Transplant. 2006;25(7):745–55.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Courtney L. Shappley
    • 1
    • 2
  • Julie J. Paik
    • 3
  • Lesley Ann Saketkoo
    • 2
    • 4
    • 5
    Email author
  1. 1.Department of Pulmonary and Critical Care Medicine, and Multi-Organ Transplant InstituteOchsner Clinic FoundationNew OrleansUSA
  2. 2.Tulane University School of Medicine, Section of Pulmonary Medicine; Section of RheumatologyNew OrleansUSA
  3. 3.Division of RheumatologyJohns Hopkins University School of MedicineBaltimoreUSA
  4. 4.University Medical Center Comprehensive Pulmonary Hypertension CenterNew OrleansUSA
  5. 5.New Orleans Scleroderma and Sarcoidosis Patient Care and Research CenterTulane University School of MedicineNew OrleansUSA

Personalised recommendations

Cite article

Buy options