Advertisement

Current Rheumatology Reports

, 15:359 | Cite as

Interstitial Lung Disease in Inflammatory Myopathies: Clinical Phenotypes and Prognosis

  • Patrick D. W. KielyEmail author
  • Felix Chua
INFLAMMATORY MUSCLE DISEASE (I LUNDBERG, SECTION EDITOR)
Part of the following topical collections:
  1. Topical Collection on Inflammatory Muscle Disease

Abstract

Interstitial lung disease (ILD) is a very important complication of the idiopathic inflammatory myositides (IIM), with a prevalence of approximately 40 %. Characteristic HRCT changes, most commonly NSIP, together with a restrictive ventilatory defect and an associated decline in DLco support the presence of ILD. The strongest risk factors are the presence of the anti-amino-acyl-tRNA synthetases (ARS) and anti-MDA-5 antibodies, but a raised index of suspicion for ILD should also apply to IIM patients of black ethnicity. Overall, the prognosis of ILD in IIM is good; between 50 and 66 % of cases have a stable disease course over a substantial period of time. The remaining proportion will show signs of worsening lung disease within 12 months of diagnosis. Whereas ARS antibodies and black ethnicity have no influence on ILD prognosis, detection of the anti-MDA-5 antibody carries a poor ILD outcome, in which hyperferritinaemia appears to be an important diagnostic and prognostic feature.

Keywords

Interstitial lung disease Idiopathic inflammatory myositis Inflammatory myopathy Polymyositis Dermatomyositis Clinically amyopathic dermatomyositis Anti-synthetase syndrome Anti-MDA-5 antibody KL-6 Calprotectin BAFF Ferritin Phenotypes Prognosis 

Notes

Compliance with Ethics Guidelines

Conflict of Interest

Patrick D.W. Kiely declares that he has no conflict of interest.

Felix Chua declares that he has no conflict 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

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

  1. 1.
    Mills ES, Mathews WH. Interstitial pneumonitis in dermatomyositis. J Am Med Assoc. 1956;160:1467–70.PubMedCrossRefGoogle Scholar
  2. 2.
    Schnabel A, Reuter M, Biederer J, et al. Interstitial lung disease in polymyositis and dermatomyositis: clinical course and response to treatment. Semin Arthritis Rheum. 2003;32:273–84.PubMedCrossRefGoogle Scholar
  3. 3.
    Dickey BF, Myers AR. Pulmonary disease in polymyositis/dermatomyositis. Semin Arthritis Rheum. 1984;14:60–76.PubMedCrossRefGoogle Scholar
  4. 4.
    Benbassat J, Gefel D, Larholt K, et al. Prognostic factors in polymyositis/dermatomyositis. Arthritis Rheum. 1985;28:249–55.PubMedCrossRefGoogle Scholar
  5. 5.
    Frazier AR, Miller RD. Interstitial pneumonitis in association with polymyositis and dermatomyositis. Chest. 1974;65:403–7.PubMedCrossRefGoogle Scholar
  6. 6.
    Salmeron G, Greenberg SD, Lidsky MD. Polymyositis and diffuse interstitial lung disease: a review of the pulmonary histopathologic findings. Arch Int Med. 1981;141:100–10.CrossRefGoogle Scholar
  7. 7.
    Tazelaar HD, Viggiano RW, Pickersgill J, Colby TV. Interstitial lung disease in polymyositis and dermatomyositis: clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis. 1990;141:727–33.PubMedCrossRefGoogle Scholar
  8. 8.
    Marie I, Hatron P, Hachulla E, et al. Pulmonary involvement in polymyositis and dermatomyositis. J Rheumatol. 1998;25:1336–43.PubMedGoogle Scholar
  9. 9.
    • Chua F, Higton AM, Colebatch AN, et al. Idiopathic inflammatory myositis-associated interstitial lung disease: ethnicity differences and lung function trends in a British cohort. Rheumatology. 2012;51:1870–6. Describes the association of ILD with black ethnicity in IIM patients. PubMedCrossRefGoogle Scholar
  10. 10.
    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;59:677–85.PubMedCrossRefGoogle Scholar
  11. 11.
    Marie I, Hachulla E, Chérin P, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002;47:614–22.PubMedCrossRefGoogle Scholar
  12. 12.
    Wells AU, Hirani N. Interstitial lung disease guideline. Thorax. 2008;63:v1–58.PubMedCrossRefGoogle Scholar
  13. 13.
    • Raghu G, Collard HR, Egan JJ, et al. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. Am J Respir Crit Care Med. 2011;183:788–824. Important consensus statement on Idiopathic Pulmonary Fibrosis, new diagnostic criteria compared to previous statement in 2000. PubMedCrossRefGoogle Scholar
  14. 14.
    Douglas WW, Tazelaar HD, Hartman TE, et al. Polymyositis-dermatomyositis-associated Interstitial lung disease. Am J Respir Crit Care Med. 2001;164:1182–5.PubMedCrossRefGoogle Scholar
  15. 15.
    Aduen JF, Zisman DA, Mobin SI, et al. Retrospective study of pulmonary function tests in patients presenting with isolated reduction in single-breath diffusion capacity: implications for the diagnosis of combined obstructive and restrictive lung disease. Mayo Clin Proc. 2007;82:48–54.PubMedGoogle Scholar
  16. 16.
    Morganroth PA, Kreider ME, Okawa J, et al. Interstitial lung disease in classic and skin-predominant dermatomyositis. Arch Dermatol. 2010;146:729–38.PubMedCrossRefGoogle Scholar
  17. 17.
    Tillie-Leblond I, Wislez M, Valeyre D, et al. Interstitial lung disease and anti-Jo-1 antibodies: difference between acute and gradual onset. Thorax. 2008;63:53–9.PubMedCrossRefGoogle Scholar
  18. 18.
    Marie I, Hachulla E, Hatron P, et al. Polymyositis and dermatomyositis: short term and long term outcome, and predictive factors of prognosis. J Rheumatol. 2001;28:2230–7.PubMedGoogle Scholar
  19. 19.
    Bonnefoy O, Ferretti G, Calaque O, et al. Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis. Eur J Radiol. 2004;49:235–44.PubMedCrossRefGoogle Scholar
  20. 20.
    Chinoy H, Salway F, Fertig N, et al. Clinical, serological and HLA profiles in non-Caucasian UK idiopathic inflammatory myopathy. Rheumatology. 2009;48:591–2.PubMedCrossRefGoogle Scholar
  21. 21.
    Akira M, Hara H, Sakatani M. Interstitial Lung Disease in Association with Polymyositis-Dermatomyositis: Long-term Follow-up CT Evaluation in Seven Patients. Radiology. 2010;1999:333–8.Google Scholar
  22. 22.
    Kang EH, Lee EB, Shin KC, et al. Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology. 2005;44:1282–6.PubMedCrossRefGoogle Scholar
  23. 23.
    Fathi M, Dastmalchi M, Rasmussen E, et al. Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis. Ann Rheum Dis. 2004;63:297–301.PubMedCrossRefGoogle Scholar
  24. 24.
    Mielnik P, Wiesik-Szewczyk E, Olesinska M, et al. Clinical features and prognosis of patients with idiopathic inflammatory myopathies and anti-Jo-1 antibodies. Autoimmunity. 2006;39:243–7.PubMedCrossRefGoogle Scholar
  25. 25.
    La Corte R, Lo Mo Naco A, Locaputo A, et al. In patients with a ntisynthetase syndrome the occurrence of anti-Ro/SSA antibodies causes a more severe interstitial lung disease. Autoimmunity. 2006;39:249–53.PubMedCrossRefGoogle Scholar
  26. 26.
    Vancsa A, Csipo I, Nemeth J, et al. Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients. Rheumatol Int. 2009;29:989–94.PubMedCrossRefGoogle Scholar
  27. 27.
    Solomon J, Swigris JJ, Brown KK. Myositis-related interstitial lung disease and antisynthetase syndrome. J Bras Pneumol. 2011;37:100–9.PubMedCrossRefGoogle Scholar
  28. 28.
    Gunawardena H, Betteridge ZE, Mc Hugh NJ. Myositis-specific antibodies: their clinical and pathogenic significance in disease expression. Rheumatology. 2009;48:607–12.PubMedCrossRefGoogle Scholar
  29. 29.
    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;52:1571–6.PubMedCrossRefGoogle Scholar
  30. 30.
    • Nakashima R, Imura Y, Kobayashi S, et al. The RIG-I-like receptor IFIH1/MDA5 is a dermatomyositis-specific autoantigen identified by the anti-CADM-140 antibody. Rheumatology. 2010;49:433–40. Series demonstrating the characteristic phenotype on anti-MDA-5-positive DM patients, compared to anti-MDA-5-negative DM patients. PubMedCrossRefGoogle Scholar
  31. 31.
    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;60:2193–200.PubMedCrossRefGoogle Scholar
  32. 32.
    Cao H, Pan M, Kang Y. 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.CrossRefGoogle Scholar
  33. 33.
    • Betteridge ZE, Gunawardena H, McHugh NJ. Novel autoantibodies and clinical phenotypes in adult and juvenile myositis. Arthritis Res Ther. 2011;13:209–14. Review of the spectrum of novel myositis-specific autoantibodies and their clinical phenotypes. PubMedCrossRefGoogle Scholar
  34. 34.
    • 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. 2010;49:1713–9. Comparison of the phenotypic differences of patients with anti-MDA-5-associated ILD and anti-synthetase-associated ILD. PubMedCrossRefGoogle Scholar
  35. 35.
    Targoff IN, Johnson AE, Miller FW. Antibody to signal recognition particle in polymyositis. Arthritis Rheum. 1990;33:1361–70.PubMedCrossRefGoogle Scholar
  36. 36.
    Kao AH, Lacomis D, Lucas M, et al. Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy. Arthritis Rheum. 2004;50:209–15.PubMedCrossRefGoogle Scholar
  37. 37.
    Hengstman GJD, ter Laak HJ, Vree Egberts WTM, et al. Anti-signal recognition particle autoantibodies: marker of a necrotising myopathy. Ann Rheum Dis. 2006;65:1635–8.PubMedCrossRefGoogle Scholar
  38. 38.
    • Fathi M, Barbasso Helmers S, Lundberg IE. KL-6: a serological biomarker for interstitial lung disease in patients with polymyositis and dermatomyositis. J Intern Med. 2012;271:589–97. Association of KL-6 with ILD in patients with PM/DM, and relation to pulmonary function tests; a promising biomarker for use in clinical practice. PubMedCrossRefGoogle Scholar
  39. 39.
    Kubo M, Ihn H, Yamane K, et al. Serum KL-6 in adult patients with polymyositis and dermatomyositis. Rheumatology. 2000;39:632–6.PubMedCrossRefGoogle Scholar
  40. 40.
    Kumanovics G, Minier T, Radics J, et al. Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis. Clin Exp Rheumatol. 2008;26:414–20.PubMedGoogle Scholar
  41. 41.
    Mortensen OH, Andersen K, Fischer C, et al. Calprotectin is released from human skeletal muscle tissue during exercise. J Physiol. 2008;586(14):3551–62.PubMedCrossRefGoogle Scholar
  42. 42.
    Seeliger S, Vogl T, Engels IH, et al. Expression of calcium-binding proteins MRP8 and MRP14 in inflammatory muscle diseases. Am J Pathol. 2003;163:947–56.PubMedCrossRefGoogle Scholar
  43. 43.
    Korthagen NM, Nagtegaal MM, van Moorsel CHM, et al. MRP14 is elevated in the bronchoalveolar lavage fluid of fibrosing interstitial lung diseases. Clin Exp Immunol. 2010;161:342–7.PubMedGoogle Scholar
  44. 44.
    Avery DT, Kalled SL, Ellyard JI, et al. BAFF selectively enhances the survival of plasmablasts generated from human memory B cells. J Clin Invest. 2003;112:286–97.PubMedGoogle Scholar
  45. 45.
    Petri M, Stohl W, Chatham W, et al. Association of plasma B lymphocyte stimulator levels and disease activity in systemic lupus erythematosus. Arthritis Rheum. 2008;58:2453–9.PubMedCrossRefGoogle Scholar
  46. 46.
    Krystufkova O, Vallerskog T, Barbasso Helmers S, et al. Increased serum levels of B cell activating factor (BAFF) in subsets of patients with idiopathic inflammatory myopathies. Ann Rheum Dis. 2009;68:836–43.PubMedCrossRefGoogle Scholar
  47. 47.
    • 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. 2010;49:1354–60. Association of hyperferritinaemia with poor prognosis in DM. PubMedCrossRefGoogle Scholar
  48. 48.
    Lakhanpal S, Lie JT, Conn DL, Martin WJ. Pulmonary disease in polymyositis/dermatomyositis: a clinicopathological analysis of 65 autopsy cases. Ann Rheum Dis. 1987;46:23–9.PubMedCrossRefGoogle Scholar
  49. 49.
    Arsura EL, Greenberg AS. Adverse impact of interstitial pulmonary fibrosis on prognosis in polymyositis and dermatomyositis. Semin Arthritis Rheum. 1988;18:29–37.PubMedCrossRefGoogle Scholar
  50. 50.
    Yamasaki Y, Yamada H, Ohkubo M, et al. Longterm survival and associated risk factors in patients with adult-onset idiopathic inflammatory myopathies and amyopathic dermatomyositis: experience in a single institute in Japan. J Rheumatol. 2011;38:1636–43.PubMedCrossRefGoogle Scholar
  51. 51.
    Maugars YM, Berthelot JM, Abbas AA, et al. Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol. 1996;14:263–74.PubMedGoogle Scholar
  52. 52.
    Sultan SM, Ioannou Y, Moss K, Isenberg DA. Outcome in patients with idiopathic inflammatory myositis: morbidity and mortality. Rheumatology. 2002;41:22–6.PubMedCrossRefGoogle Scholar
  53. 53.
    Fujisawa T, Suda T, Nakamura Y, et al. Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis. J Rheumatol. 2005;32:58–64.PubMedGoogle Scholar
  54. 54.
    Yoshifuji H, Fujii T, Kobayashi S, et al. Anti-aminoacyl-tRNA synthetase antibodies in clinical course prediction of interstitial lung disease complicated with idiopathic inflammatory myopathies. Autoimmunity. 2006;39:233–41.PubMedCrossRefGoogle Scholar
  55. 55.
    Arakawa H, Yamada H, Kurihara Y, et al. Nonspecific interstitial pneumonia associated with polymyositis and dermatomyositis. Serial high-resolution CT findings and functional correlation. Chest. 2003;123:1096–103.PubMedCrossRefGoogle Scholar
  56. 56.
    Mino M, Noma S, Taguchi Y, et al. Pulmonary involvement in polymyositis and dermatomyositis: sequential evaluation with CT. Am J Roentgenol. 1997;169:83–7.CrossRefGoogle Scholar
  57. 57.
    Stone KB, Oddis CV, Fertig N, et al. Anti-Jo-1 antibody levels correlate with disease activity in idiopathic inflammatory myopathy. Arthritis Rheum. 2007;56:3125–31.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of RheumatologySt George’s Healthcare NHS TrustLondonUK
  2. 2.Department of Respiratory MedicineSt George’s Healthcare NHS TrustLondonUK

Personalised recommendations