AUTOIMMUNE ILD - OVERVIEW

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OVERVIEW

Connective tissue diseases (CTDs), also known as rheumatologic diseases or autoimmune diseases, are disorders that exhibit autoimmune-mediated damage due to circulating auto-antibodies that target various body organs and cause symptomatic presentation.1

There are many rheumatologic diseases:

  • Rheumatoid arthritis (RA)
  • Sjögren's syndrome
  • Systemic lupus erythematosus (SLE)
  • Polymyositis/dermatomyositis (PM/DM)
  • Antisynthetase syndrome
  • Mixed connective tissue disease (MCTD)
  • Systemic sclerosis (SSc)
  • Interstitial pneumonia with autoimmune features (IPAF)

Rheumatologic diseases have various manifestations within each disease, as well as across all rheumatologic diseases.2,3

  • However, one potential manifestation that many rheumatologic diseases have in common is lLD.1,3
  • ILD is common among rheumatologic diseases, and is associated with significant morbidity and mortality1,3
  • Histologic presentation of autoimmune ILD is variable, ranging from inflammatory to fibrotic1
  • Clinical manifestations of autoimmune ILD also vary greatly, ranging from clinically insignificant or mild symptoms to respiratory failure1
  • A large percentage of patients with autoimmune ILD will develop a progressive ILD resulting in irreversible damage to the lung parenchyma1

PREVALENCE4-11

CTD-ILD-prevalence

EPIDEMIOLOGY

The overall rate of autoimmune ILD is estimated to be 15%1,12

ILD has high incidence rates in several rheumatologic diseases, including SSc, PM/DM, and MCTD1

In cases of ILD without identified cause, patients should be screened for rheumatologic diseases, particularly when they exhibit rapidly progressive or chronic ILD.13

REFERENCES

  1. Koo S-M, U S-T. Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist's point of view. Korean J Intern Med. 2017;32(4):600-610. doi: 10.3904/kjim.2016.212.
  2. Reddy BY, Hantash BM. Cutaneous Connective Tissue Diseases: Epidemiology, Diagnosis, and Treatment. Open Dermatol J. 2009;3(1):22-31. doi: 10.2174/1874372200903010022.
  3. Wallace B, Vummidi M, Khanna D, et al. Management of connective tissue diseases associated interstitial lung disease: a review of the published literature. Curr Opin Rheumatol. 2016;28(3):236-245. doi: 10.1097/BOR.0000000000000270.
  4. Castelino FV, Varga J. Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management. Arthritis Res Ther. 2010;12(4):213. doi: 10.1186/ar3097.
  5. Witt LJ, Curran JJ, Strek ME. The Diagnosis and Treatment of Antisynthetase Syndrome. Clin Pulm Med. 2016;23(5):218-226. doi: 10.1097/CPM.0000000000000171.
  6. Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S. Pulmonary manifestations of Sjögren's syndrome. Eur Respir Rev. 2016;25(140):110-123. doi: 10.1183/16000617.0011-2016.
  7. Spagnolo P, Cordier JF, Cottin V. Connective tissue diseases, multimorbidity and the ageing lung. Eur Respir J. 2016;47(5):1535-1558. doi: 10.1183/13993003.00829-2015.
  8. Ha YJ, Lee YJ, Kang EH. Lung Involvements in Rheumatic Diseases: Update on the Epidemiology, Pathogenesis, Clinical Features, and Treatment. Biomed Res Int. 2018;2018:6930297. Published 2018 May 8. doi: 10.1155/2018/6930297.
  9. de Lauretis A, Veeraraghavan S, Renzoni E. Review series: Aspects of interstitial lung disease: connective tissue disease-associated interstitial lung disease: how does it differ from IPF? How should the clinical approach differ?. Chron Respir Dis. 2011;8(1):53-82. doi: 10.1177/1479972310393758.
  10. Graney BA, Fischer A. Advocating for early interstitial lung disease detection in mixed connective tissue disease. Rheumatology (Oxford). 2018;57(2):204-205. doi: 10.1093/rheumatology/kex256.
  11. Steele R, Hudson M, Lo E, Baron M; Canadian Scleroderma Research Group. Clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis. Arthritis Care Res (Hoboken). 2012;64(4):519-524. doi: 10.1002/acr.21583.
  12. Strange C, Highland KB. Interstitial lung disease in the patient who has connective tissue disease. Clin Chest Med. 2004;25(3):549-559, vii. doi: 10.1016/j.ccm.2004.05.009.
  13. Bauer PR, Kalra S, Osborn TG, et al. Influence of autoimmune biomarkers on interstitial lung diseases: A tertiary referral center based case-control study. Respir Med. 2015;109(3): 397-405. doi: 10.1016/j.rmed.2015.01.011.
  14. Raghu G, Brown KK. Interstitial lung disease: clinical evaluation and keys to an accurate diagnosis. Clin Chest Med. 2004;25(3):409-419, v. doi: 10.1016/j.ccm.2004.05.007.
  15. van Manen MJG, Birring SS, Vancheri C, et al. Cough in idiopathic pulmonary fibrosis. Eur Respir Rev. 2016;25(141):278-286. doi: 10.1183/16000617.0090-2015.
  16. European Respiratory Society. Interstitial Lung Diseases in European Lung White Book. 256-269.
  17. Cosgrove GP, Bianchi P, Danese S, et al. Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulm Med. 2018:18(1):9. 10.1186/s12890-017-0560-x.
  18. Nathan N, Corvol H, Amselem S, et al. Biomarkers in Interstitial lung diseases. Paediatr Respir Rev. 2015;16(4):219-224.doi: 10.1016/j.prrv.2015.05.002.

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