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Update on Treatment of Antisynthetase Syndrome: A Brief Review

  • Other CTD: Inflammatory Myopathies and Sjogren’s (P Basharat and JFL Albayda, Section Editors)
  • Published:
Current Treatment Options in Rheumatology Aims and scope Submit manuscript

Abstract

Purpose of the review

Antisynthetase syndrome (ASSD) is a rare, heterogeneous, systemic disease characterized by the clinical triad of arthritis, myositis, and interstitial lung disease (ILD) together with other accompanying findings, which often has a progressive and life-threatening evolution. The rarity of this condition and the lack of classification criteria make it hard to conduct studies on a homogenous cohort, resulting in the lack of standardized treatment regimens for this condition.

Recent findings

To date, the evidence regarding the treatment of ASSD mostly derives from observational studies on polymyositis and dermatomyositis cohorts.

Summary

Corticosteroids, calcineurin inhibitors, cyclophosphamide, and rituximab are the most widely used treatments for ILD and myositis, while few data are available for mycophenolate mofetil, methotrexate, and azathioprine. Data on arthritis are scarce, suggesting efficacy of corticosteroids, calcineurin inhibitors, and rituximab. A homogenous classification of these patients is necessary in order to produce good quality data on ASSD treatment.

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References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Cavagna L, Nuño L, Scirè CA, Govoni M, Longo FJL, Franceschini F, 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.

    CAS  PubMed  Google Scholar 

  2. Cavagna L, Caporali R, Abdì-Alì L, Dore R, Meloni F, Montecucco C. Cyclosporine in anti-Jo1-positive patients with corticosteroid-refractory interstitial lung disease. J Rheumatol. 2013;40:484–92.

    CAS  PubMed  Google Scholar 

  3. Cavagna L, Nuño L, Scirè CA, Govoni M, Longo FJL, 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.

    CAS  Google Scholar 

  4. Monti S, Montecucco C, Cavagna L. Clinical spectrum of anti-Jo-1-associated disease. Curr Opin Rheumatol. 2017;29:612–7.

    CAS  PubMed  Google Scholar 

  5. Hirakata M, Suwa A, Takada T, Sato S, Nagai S, Genth E, et al. Clinical and immunogenetic features of patients with autoantibodies to asparaginyl-transfer RNA synthetase. Arthritis Rheum. 2007;56:1295–303.

    CAS  PubMed  Google Scholar 

  6. Sato S, Kuwana M, Hirakata M. Clinical characteristics of Japanese patients with anti-OJ (anti-isoleucyl-tRNA synthetase) autoantibodies. Rheumatol Oxf Engl. 2007;46:842–5.

    CAS  Google Scholar 

  7. Sato S, Hirakata M, Kuwana M, Nakamura K, Suwa A, Inada S, et al. Clinical characteristics of Japanese patients with anti-PL-7 (anti-threonyl-tRNA synthetase) autoantibodies. Clin Exp Rheumatol. 2005;23:609–15.

    CAS  PubMed  Google Scholar 

  8. Betteridge Z, Gunawardena H, North J, Slinn J, McHugh N. Anti-synthetase syndrome: a new autoantibody to phenylalanyl transfer RNA synthetase (anti-Zo) associated with polymyositis and interstitial pneumonia. Rheumatol Oxf Engl. 2007;46:1005–8.

    CAS  Google Scholar 

  9. Kondratiuk I, Khoruzenko A, Cherednyk O, Filonenko V, Kornelyuk A. Monoclonal antibodies against Tyrosyl-tRNA Synthetase and its isolated cytokine-like domain. Monoclon Antibodies Immunodiagn Immunother. 2013;32:200–4.

    CAS  Google Scholar 

  10. Love LA, Leff RL, Fraser DD, Targoff IN, Dalakas M, Plotz PH, et al. A New Approach to the Classification of Idiopathic Inflammatory Myopathy: Myositis-Specific Autoantibodies Define Useful Homogeneous Patient Groups. Medicine (Baltimore). 1991;70:360.

    CAS  Google Scholar 

  11. •• Bartoloni E, Gonzalez-Gay MA, Scire C, Castaneda S, Gerli R, Lopez-Longo FJ, et al. Clinical follow-up predictors of disease pattern change in anti-Jo1 positive anti-synthetase syndrome: results from a multicenter, international and retrospective study. Autoimmun Rev. 2017;16:253–7This large observational study showed that ASSD may change over time, with the occurrence of new triad manifestation during the follow-up, especially when accompanying features are present.

    CAS  PubMed  Google Scholar 

  12. Trallero-Araguas E, Grau-Junyent JM, Labirua-Iturburu A, Garcia-Hernandez FJ, Monteagudo-Jimenez M, Fraile-Rodriguez G, et al. Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group. Semin Arthritis Rheum. 2016;46:225–31.

    CAS  PubMed  Google Scholar 

  13. Trallero AE, Selva O’CA, Scire CA, Codullo V, Castaneda S, Ortego CN, et al. Clinical spectrum time course comparison between pl-7, pl-12 and ej positive antisynthetase syndrome. Ann Rheum Dis. 2017;76:1272–3.

    Google Scholar 

  14. 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.

    CAS  PubMed  Google Scholar 

  15. Gonzalez-Gay MA, Montecucco C, Selva-O’Callaghan A, Trallero-Araguas E, Molberg O, Andersson H, et al. Timing of onset affects arthritis presentation pattern in antisyntethase syndrome. Clin Exp Rheumatol. 2018;36:44–9.

    PubMed  Google Scholar 

  16. Lefevre G, Meyer A, Launay D, Machelart I, DeBandt M, Michaud J, et al. Seronegative polyarthritis revealing antisynthetase syndrome: a multicentre study of 40 patients. Rheumatol Oxf Engl. 2015;54:927–32.

    CAS  Google Scholar 

  17. Nagashima T, Iwamoto M, Minota S. Antisynthetase syndrome associated with long-standing rheumatoid arthritis. Rheumatol Int. 2011;31:705–6.

    PubMed  Google Scholar 

  18. Cavagna L, Nuno L, Scire 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. Med Baltim. 2015;94:e1144.

    CAS  Google Scholar 

  19. Cavagna L, Monti S, Grosso V, Boffini N, Scorletti E, Crepaldi G, et al. The multifaceted aspects of interstitial lung disease in rheumatoid arthritis. Biomed Res Int. 2013;2013:759760.

    PubMed  PubMed Central  Google Scholar 

  20. Cavagna L, Fusetti C, Montecucco C, Caporali R. Anticyclic citrullinated peptide antibodies as markers of erosive arthritis in antisynthetase syndrome. J Rheumatol. 2010;37:1967 author reply 1968.

    PubMed  Google Scholar 

  21. Ide V, Bossuyt X, Blockmans D, De Langhe E. Prevalence and clinical correlates of rheumatoid factor and anticitrullinated protein antibodies in patients with idiopathic inflammatory myopathy. RMD Open. 2018;4:e000661.

    PubMed  PubMed Central  Google Scholar 

  22. Mumm GE, McKown KM, Bell CL. Antisynthetase syndrome presenting as rheumatoid-like polyarthritis. J Clin Rheumatol. 2010;16:307–12.

    PubMed  Google Scholar 

  23. Labrador-Horrillo M, Martinez MA, Selva-O’Callaghan A, Delgado JF, Martínez-Gómez X, Trallero-Araguás E, et al. Anti-cyclic citrullinated peptide and anti-keratin antibodies in patients with idiopathic inflammatory myopathy. Rheumatol Oxf Engl. 2009;48:676–9.

    CAS  Google Scholar 

  24. Miller JB, Danoff SK, Bingham CO, Paik JJ, Mecoli CA, Tiniakou E, et al. Sonographic findings from inflammatory arthritis due to antisynthetase syndrome. Clin Rheumatol. 2019;38:1477–83.

    PubMed  Google Scholar 

  25. Meyer A, Lefevre G, Bierry G, Duval A, Ottaviani S, Meyer O, et al. In antisynthetase syndrome, ACPA are associated with severe and erosive arthritis: an overlapping rheumatoid arthritis and antisynthetase syndrome. Medicine (Baltimore). 2015;94:e523.

    CAS  Google Scholar 

  26. 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.

    CAS  PubMed  Google Scholar 

  27. Waseda Y, Johkoh T, Egashira R, Sumikawa H, Saeki K, Watanabe S, et al. Antisynthetase syndrome: pulmonary computed tomography findings of adult patients with antibodies to aminoacyl-tRNA synthetases. Eur J Radiol. 2016;85:1421–6.

    PubMed  Google Scholar 

  28. Debray MP, Borie R, Revel MP, Naccache JM, Khalil A, Toper C, et al. Interstitial lung disease in anti-synthetase syndrome: initial and follow-up CT findings. Eur J Radiol. 2015;84:516–23.

    PubMed  Google Scholar 

  29. 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.

    CAS  PubMed  Google Scholar 

  30. Tanizawa K, Handa T, Nakashima R, Kubo T, Hosono Y, Watanabe K, et al. The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies. Respir Med. 2017;127:57–64.

    PubMed  Google Scholar 

  31. Aggarwal R, McBurney C, Schneider F, Yousem SA, Gibson KF, Lindell K, et al. Myositis-associated usual interstitial pneumonia has a better survival than idiopathic pulmonary fibrosis. Rheumatol U K. 2017;56:384–9.

    Google Scholar 

  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.

    PubMed  PubMed Central  Google Scholar 

  33. Herrick AL. The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol. 2012;8:469–79.

    CAS  Google Scholar 

  34. Török L, Dankó K, Cserni G, Szûcs G. PM-SCL autoantibody positive scleroderma with polymyositis (mechanic’s hand: clinical aid in the diagnosis). J Eur Acad Dermatol Venereol JEADV. 2004;18:356–9.

    PubMed  Google Scholar 

  35. Cox JT, Gullotti DM, Mecoli CA, Lahouti AH, Albayda J, Paik J, et al. “Hiker’s feet”: a novel cutaneous finding in the inflammatory myopathies. Clin Rheumatol. 2017.

  36. Cavagna L, Monti S, Caporali R, Gatto M, Iaccarino L, Doria A. How I treat idiopathic patients with inflammatory myopathies in the clinical practice. Autoimmun Rev. 2017.

  37. 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.

    CAS  Google Scholar 

  38. Oddis CV. Therapy for myositis. Curr Opin Rheumatol. 1993;5:742–8.

    CAS  PubMed  Google Scholar 

  39. Oddis CV, Aggarwal R. Treatment in myositis. Nat Rev Rheumatol. 2018;14:279–89.

    CAS  PubMed  Google Scholar 

  40. Labirua-Iturburu A, Selva-O’Callaghan A, Martínez-Gómez X, Trallero-Araguás E, Labrador-Horrillo M, Vilardell-Tarrés M. Calcineurin inhibitors in a cohort of patients with antisynthetase-associated interstitial lung disease. Clin Exp Rheumatol. 2013;31:436–9.

    PubMed  Google Scholar 

  41. Polosa R, Edwards CJ. Tacrolimus for antisynthetase syndrome with interstitial lung disease? Eur Respir J. 2008;32:244–5 author reply 245-246.

    CAS  PubMed  Google Scholar 

  42. Wilkes MR, Sereika SM, Fertig N, Lucas MR, Oddis CV. Treatment of antisynthetase-associated interstitial lung disease with tacrolimus. Arthritis Rheum. 2005;52:2439–46.

    CAS  PubMed  Google Scholar 

  43. 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;31:213–7.

    CAS  PubMed  Google Scholar 

  44. Jankowska M, Butto B, Debska-Slizień A, Rutkowski B. Beneficial effect of treatment with cyclosporin a in a case of refractory antisynthetase syndrome. Rheumatol Int. 2007;27:775–80.

    PubMed  Google Scholar 

  45. Cavagna L, Caporali R. Therapeutic options in anti-jo-1 antisynthetase syndrome with interstitial lung disease: comment on the article by Marie et al. Arthritis Care Res. 2013;65:1548.

    Google Scholar 

  46. 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.

    PubMed  Google Scholar 

  47. Jensen ML, Løkke A, Hilberg O, Hyldgaard C, Bendstrup E, Tran D. Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease: a retrospective cohort study. Eur Clin Respir J. 2019; [cited 2019 Jul 10];6. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6394310/.

  48. Ben Salem T, Abdelkafi C, Lamloum M, Ben Ghorbel I, Houman MH. Pulmonary manifestations in antisynthetase syndrome. Tunis Med. 2018;96:101–6.

    PubMed  Google Scholar 

  49. Vuillard C, Pineton de Chambrun M, de Prost N, Guérin C, Schmidt M, Dargent A, et al. Clinical features and outcome of patients with acute respiratory failure revealing anti-synthetase or anti-MDA-5 dermato-pulmonary syndrome: a French multicenter retrospective study. Ann Intensive Care. 2018; [cited 2019 Jul 11];8. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131681/.

  50. •• Bauhammer J, Blank N, Max R, Lorenz H-M, Wagner U, Krause D, et al. Rituximab in the treatment of Jo1 antibody-associated Antisynthetase syndrome: anti-Ro52 positivity as a marker for severity and treatment response. J Rheumatol. 2016;43:1566–74This study assessed the effectiveness of RTX in ASSD-ILD, especially in a sub-cohort of patients with positivity for anti-Ro 52 in which other IS, including CYC, seemed to be less effective.

    CAS  PubMed  Google Scholar 

  51. Andersson H, Sem M, Lund MB, Aaløkken TM, Günther A, Walle-Hansen R, et al. Long-term experience with rituximab in anti-synthetase syndrome-related interstitial lung disease. Rheumatol Oxf Engl. 2015;54:1420–8.

    CAS  Google Scholar 

  52. Fischer A, Brown KK, Du Bois RM, Frankel SK, Cosgrove GP, Fernandez-Perez ER, et al. Mycophenolate Mofetil improves lung function in connective tissue disease-associated interstitial lung disease. J Rheumatol. 2013;40:640–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  53. Hervier B, Masseau A, Mussini J-M, Audrain M, Hamidou MA. Long-term efficacy of mycophenolate mofetil in a case of refractory antisynthetase syndrome. Joint Bone Spine. 2009;76:575–6.

    PubMed  Google Scholar 

  54. Majithia V, Harisdangkul V. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology. 2005;44:386–9.

    CAS  PubMed  Google Scholar 

  55. Swigris JJ, Olson AL, Fischer A, Lynch DA, Cosgrove GP, Frankel SK, 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.

    CAS  PubMed  Google Scholar 

  56. 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.

    PubMed  Google Scholar 

  57. Morganroth PA, Kreider ME, Werth VP. Mycophenolate mofetil for interstitial lung disease in dermatomyositis. Arthritis Care Res. 2010;62:1496–501.

    Google Scholar 

  58. Olivo Pallo PA, de Souza FHC, Miossi R, Shinjo SK. Mycophenolate mofetil in patients with refractory systemic autoimmune myopathies: case series. Adv Rheumatol Lond Engl. 2018;58:34.

    Google Scholar 

  59. Kashif M, Arya D, Niazi M, Khaja M. A rare case of necrotizing myopathy and Fibrinous and organizing pneumonia with anti-EJ Antisynthetase syndrome and Sjögren’s syndrome (SSA) antibodies. Am J Case Rep. 2017;18:448–53.

    PubMed  PubMed Central  Google Scholar 

  60. Pisoni CN, Cuadrado MJ, Khamashta MA, Hughes GRV, D’Cruz DP. Mycophenolate mofetil treatment in resistant myositis. Rheumatology. 2007;46:516–8.

    CAS  PubMed  Google Scholar 

  61. Rowin J, Amato AA, Deisher N, Cursio J, Meriggioli MN. Mycophenolate mofetil in dermatomyositis: is it safe? Neurology. 2006;66:1245–7.

    CAS  PubMed  Google Scholar 

  62. Danieli MG, Calcabrini L, Calabrese V, Marchetti A, Logullo F, Gabrielli A. Intravenous immunoglobulin as add on treatment with mycophenolate mofetil in severe myositis. Autoimmun Rev. 2009;9:124–7.

    CAS  PubMed  Google Scholar 

  63. Edge JC, Outland JD, Dempsey JR, Callen JP. Mycophenolate Mofetil as an effective corticosteroid-sparing therapy for recalcitrant Dermatomyositis. Arch Dermatol. 2006;142:65–9.

    CAS  PubMed  Google Scholar 

  64. Gelber AC, Nousari HC, Wigley FM. Mycophenolate mofetil in the treatment of severe skin manifestations of dermatomyositis: a series of 4 cases. J Rheumatol. 2000;27:1542–5.

    CAS  PubMed  Google Scholar 

  65. 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.

    CAS  Google Scholar 

  66. • Fasano S, Gordon P, Hajji R, Loyo E, Isenberg DA. Rituximab in the treatment of inflammatory myopathies: a review. Rheumatology. 2017;56:26–36A systematic review that summarizes the evidence on treatment for Rituximab for IIM. Number of patients, along with specific diagnosis, indication of treatment and outcome are reproted.

    CAS  PubMed  Google Scholar 

  67. 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.

    CAS  PubMed  Google Scholar 

  68. Muñoz-Beamud F, Isenberg DA. Rituximab as an effective alternative therapy in refractory idiopathic inflammatory myopathies. :8.

  69. Nalotto L, Iaccarino L, Zen M, Gatto M, Borella E, Domenighetti M, et al. Rituximab in refractory idiopathic inflammatory myopathies and antisynthetase syndrome: personal experience and review of the literature. Immunol Res. 2013;56:362–70.

    CAS  PubMed  Google Scholar 

  70. Limaye V, Hissaria P, Liew C-L, Koszyka B. Efficacy of rituximab in refractory antisynthetase syndrome. Intern Med J. 2012;42:e4–7.

    CAS  PubMed  Google Scholar 

  71. Marie I, Dominique S, Janvresse A, Levesque H, Menard J-F. Rituximab therapy for refractory interstitial lung disease related to antisynthetase syndrome. Respir Med. 2012;106:581–7.

    CAS  PubMed  Google Scholar 

  72. Couderc M, Gottenberg J-E, Mariette X, Hachulla E, Sibilia J, Fain O, et al. Efficacy and safety of rituximab in the treatment of refractory inflammatory myopathies in adults: results from the AIR registry. Rheumatology. 2011;50:2283–9.

    CAS  PubMed  Google Scholar 

  73. Zappa MC, Trequattrini T, Mattioli F, Rivitti R, Vigliarolo R, Marcoccia A, et al. Rituximab treatment in a case of antisynthetase syndrome with severe interstitial lung disease and acute respiratory failure. Multidiscip Respir Med. 2011;6:183–8.

    PubMed  PubMed Central  Google Scholar 

  74. Frikha F, Rigolet A, Behin A, Fautrel B, Herson S, Benveniste O. Efficacy of rituximab in refractory and relapsing myositis with anti-JO1 antibodies: a report of two cases. Rheumatology. 2009;48:1166–8.

    CAS  PubMed  Google Scholar 

  75. 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.

    CAS  Google Scholar 

  76. Vandenbroucke E, Grutters JC, Altenburg J, Boersma WG, ter Borg EJ, van den Bosch JMM. Rituximab in life threatening antisynthetase syndrome. Rheumatol Int. 2009;29:1499–502.

    CAS  PubMed  Google Scholar 

  77. Brulhart L, Waldburger J-M, Gabay C. Rituximab in the treatment of antisynthetase syndrome [7]. Ann Rheum Dis. 2006;65:974–5.

    CAS  PubMed  PubMed Central  Google Scholar 

  78. Gottenberg J-E, Guillevin L, Lambotte O, Combe B, Allanore Y, Cantagrel A, et al. Tolerance and short term efficacy of rituximab in 43 patients with systemic autoimmune diseases. Ann Rheum Dis. 2005;64:913–20.

    CAS  PubMed  Google Scholar 

  79. Lambotte O, Kotb R, Maigne G, Blanc F-X, Goujard C, Delfraissy JF. Efficacy of rituximab in refractory polymyositis. J Rheumatol. 2005;32:1369–70.

    PubMed  Google Scholar 

  80. de Souza FHC, Miossi R, de Moraes JCB, Bonfá E, Shinjo SK. Favorable rituximab response in patients with refractory idiopathic inflammatory myopathies. Adv Rheumatol Lond Engl. 2018;58:31.

    Google Scholar 

  81. Barsotti S, Cioffi E, Tripoli A, Tavoni A, d’Ascanio A, Mosca M, et al. The use of rituximab in idiopathic inflammatory myopathies: description of a monocentric cohort and review of the literature. Reumatismo. 2018:78–84.

    CAS  PubMed  Google Scholar 

  82. Sabha MM, Simo HT, Shadid RM, Altorok NI. Successful treatment of antisynthetase syndrome presenting as rhabdomyolysis with rituximab. Rheumatol Int. 2018;38:1125–30.

    PubMed  Google Scholar 

  83. Doyle TJ, Dhillon N, Madan R, Cabral F, Fletcher EA, Koontz DC, et al. Rituximab in the treatment of interstitial lung disease associated with antisynthetase syndrome: a multi-center retrospective case review. J Rheumatol. 2018;45:841–50.

    CAS  PubMed  PubMed Central  Google Scholar 

  84. Tansley S, Shaddick G, Christopher-Stine L, Sharp C, Dourmishev L, Maurer B, et al. Developing standardised treatment for adults with myositis and different phenotypes: an international survey of current prescribing preferences. Clin Exp Rheumatol. 2016;34:880–4.

    PubMed  Google Scholar 

  85. • Casal-Dominguez M, Pinal-Fernandez I, Huapaya J, Albayda J, Paik JJ, Johnson C, et al. Efficacy and adverse effects of methotrexate compared with azathioprine in the antisynthetase syndrome. Clin Exp Rheumatol. 2019;To our knowledge this is the first study comparing outcomes of MTX and AZA treatment specifically in ASSD, showing similar effectiveness on myositis but higher incidence of pneumonitis in MTX group.

  86. Meyer A, Scirè CA, Talarico R, Alexander T, Amoura Z, Avcin T, et al. Idiopathic inflammatory myopathies: narrative review of unmet needs in clinical practice guidelines. RMD Open. 2018;4:e000784.

    PubMed  Google Scholar 

  87. Marie I, Hatron P-Y, Cherin P, Hachulla E, Diot E, Vittecoq O, et al. Functional outcome and prognostic factors in anti-Jo1 patients with antisynthetase syndrome. Arthritis Res Ther. 2013;15:R149.

    PubMed  PubMed Central  Google Scholar 

  88. Spath M, Schroder M, Schlotter-Weigel B, Walter MC, Hautmann H, Leinsinger G, et al. The long-term outcome of anti-Jo-1-positive inflammatory myopathies. J Neurol. 2004;251:859–64.

    PubMed  Google Scholar 

  89. Takai M, Katsurada N, Nakashita T, Misawa M, Mochizuki T, Kaneko N, et al. Rapidly progressive interstitial lung disease associated with Dermatomyositis treated with combination of immunosuppressive therapy, direct Hemoperfusion with a Polymyxin B immobilized Fiber column and intravenous immunoglobulin. Intern Med Tokyo Jpn. 2015;54:2225–9.

    CAS  Google Scholar 

  90. • Huapaya JA, Hallowell R, Silhan L, Pinal-Fernandez I, Casal-Dominguez M, Johnson C, et al. Long-term treatment with human immunoglobulin for antisynthetase syndrome-associated interstitial lung disease. Respir Med. 2019;154:6–11A retrospective analysis reporting data of effectiveness of IVIg in refractory ASSD-ILD, that showed the efficacy of IVIg in improving FVC% and DLCO%.

    CAS  PubMed  Google Scholar 

  91. Malkan A, Cappelen-Smith C, Beran R, Griffith N, Toong C, Wang M-X, et al. Anti-synthetase syndrome associated with anti PL-12 and anti-signal recognition particle antibodies and a necrotizing auto-immune myositis. J Clin Neurosci Off J Neurosurg Soc Australas. 2015;22:396–8.

    CAS  Google Scholar 

  92. Bozkirli DEE, Kozanoglu I, Bozkirli E, Yucel E. Antisynthetase syndrome with refractory lung involvement and myositis successfully treated with double filtration plasmapheresis. J Clin Apheresis. 2013;28:422–5.

    PubMed  Google Scholar 

  93. Beaumel A, Muis-Pistor O, Tebib J-G, Coury F. Antisynthetase syndrome treated with tocilizumab. Jt Bone Spine Rev Rhum. 2016;83:361–2.

    Google Scholar 

  94. Lange U, Piegsa M, Müller-Ladner U, Strunk J. Anti-Jo-1 antibody positive polymyositis--successful therapy with leflunomide. Autoimmunity. 2006;39:261–4.

    PubMed  Google Scholar 

  95. Musiał J, Undas A, Celińska-Lowenhoff M. Polymyositis associated with infliximab treatment for rheumatoid arthritis. Rheumatol Oxf Engl. 2003;42:1566–8.

    Google Scholar 

  96. Urata Y, Wakai Y, Kowatari K, Nitobe T, Mizushima Y. Polymyositis associated with infliximab treatment for rheumatoid arthritis. Mod Rheumatol. 2006;16:410–1.

    PubMed  Google Scholar 

  97. Ishikawa Y, Yukawa N, Kawabata D, Ohmura K, Fujii T, Usui T, et al. A case of antisynthetase syndrome in a rheumatoid arthritis patient with anti-PL-12 antibody following treatment with etanercept. Clin Rheumatol. 2011;30:429–32.

    PubMed  Google Scholar 

  98. Castañeda S, Cavagna L, Gonzalez-Gay M. Comments on the “2017 classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups”. Points Concern Arthritis Rheumatol. 2018;70.

  99. Cavagna L, Castañeda S, Sciré C, Gonzalez-Gay MA. Antisynthetase syndrome or what else? Different perspectives indicate the need for new classification criteria. Ann Rheum Dis. 2018;77:e50.

    PubMed  Google Scholar 

  100. Scirè CA, Gonzalez-Gay MA, Selva-O’Callaghan A, Cavagna L. Clinical spectrum time course of interstitial pneumonia with autoimmune features in patients positive for antisynthetase antibodies. Respir Med. 2017.

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  1. Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation of Pavia, Viale Camillo Golgi 19, 27100, Pavia, Italy

    Giovanni Zanframundo MD, Emiliano Marasco MD, Ludovico De Stefano MD, Valentina Morandi MD, Paolo Delvino MD & Lorenzo Cavagna MD

  2. Division of Pneumology, University and IRCCS Policlinico S. Matteo Foundation of Pavia, Viale Camillo Golgi 19, 27100, Pavia, Italy

    Claudia La Carrubba MD, Lorenzo Volpiano MD, Claudio Tirelli MD & Federica Meloni MD

  3. Department of Clinical and Experimental Medicine, A. O. U. “Policlinico-Vittorio Emanuele”, University of Catania, Viale Camillo Golgi 19, 27100, Pavia, Italy

    Gianluca Sambataro MD

Authors
  1. Giovanni Zanframundo MD
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  2. Emiliano Marasco MD
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  3. Claudia La Carrubba MD
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  4. Ludovico De Stefano MD
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  5. Lorenzo Volpiano MD
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  6. Claudio Tirelli MD
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  7. Valentina Morandi MD
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  8. Paolo Delvino MD
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  9. Gianluca Sambataro MD
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  10. Federica Meloni MD
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  11. Lorenzo Cavagna MD
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Correspondence to Lorenzo Cavagna MD.

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This article is part of the Topical Collection on Other CTD: Inflammatory Myopathies and Sjogren’s

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Zanframundo, G., Marasco, E., La Carrubba, C. et al. Update on Treatment of Antisynthetase Syndrome: A Brief Review. Curr Treat Options in Rheum 6, 18–28 (2020). https://doi.org/10.1007/s40674-020-00139-w

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