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The Lung in Rheumatic Diseases

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Pulmonary Manifestations of Rheumatic Disease

Abstract

Lung disease associated with the rheumatic diseases represents a significant challenge both in terms of our understanding of the mechanisms of disease and appropriate treatment. Substantial morbidity and mortality can result when lung disease is not recognized early and even when it is, the risk of respiratory failure and even death are still real possibilities. While our understanding of lung disease is best understood in systemic sclerosis, aggressive and progressive parenchymal lung diseases are well described in both RA and inflammatory myositis and in undifferentiated connective tissue disease with varied pathologic expression, phenotypes, and natural history. Treatments that utilize standard immunosuppression may be beneficial in some patients with inflammatory and fibrotic disease but novel treatments designed and clinical trials performed in patients specifically with lung disease and rheumatic diseases are sorely lacking. In all such patients, a multidisciplinary approach to care involving pulmonologists, rheumatologists, pathologists, and radiologists is critical to better understand and advance our knowledge and care of this challenging group of patients.

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References

  1. Bouros D, Wells AU, Nicholson AG, et al. Histologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Med. 2002;165:1581–6.

    Article  Google Scholar 

  2. Park JH, Kim DS, Park IN, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease related subtypes. Am J Respir Crit Care Med. 2007;175:705–11.

    Article  PubMed  Google Scholar 

  3. Kim EJ, Elicker BM, Maldonado F, et al. Usual interstitial pneumonia in rheumatoid arthritis associated interstitial lung disease. Eur Respir J. 2010;35:1322–8.

    Article  CAS  PubMed  Google Scholar 

  4. Raghu G, Anstrom KJ, King Jr TE, Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. Idiopathic Pulmonary Fibrosis Clinical Research Network. N Engl J Med. 2012;366(21):1968–77.

    Article  CAS  PubMed  Google Scholar 

  5. Goh NS, Desai SR, Veeraraghaven S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177:1248–54.

    Article  PubMed  Google Scholar 

  6. Bonella F, Volpe A, Caranachi P, et al. Surfactant protein D and KL-6 serum levels in systemic sclerosis: correlation with lung and systemic involvement. Sarcoidosis Vasc Diffuse Lung Dis. 2011;28:27–33.

    CAS  PubMed  Google Scholar 

  7. Steen V, Domsic RT, Lucas M, Fertig N, Medsger Jr TA. A clinical and serologic comparison of African American and Caucasian patients with systemic sclerosis. Arthritis Rheum. 2012;64(9):2986–94.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. Manetti M, Guiducci S, Romano E, et al. Increased serum levels and tissue expression of matrix metalloproteinase-12 n patients with systemic sclerosis: correlation with severity of skin and pulmonary fibrosis and vascular damage. Ann Rheum Dis. 2012;71(6):1064–72.

    Article  CAS  PubMed  Google Scholar 

  9. Lee CG, Herzog EL, Ahangari F, et al. Chitinase I is a biomarker for and therapeutic target in scleroderma associated interstitial lung disease that augments TGF-B1 signaling. J Immunol. 2012;189(5):2635–44.

    Article  CAS  PubMed  Google Scholar 

  10. Christmans RB, Wells AU, Capelozzi VL, Silver RM. GE reflux incites interstitial lung disease in systemic sclerosis: clinical, radiologic, histopathologic and treatment evidence. Semin Arthritis Rheum. 2010;40(3):241–9.

    Article  Google Scholar 

  11. Roth MD, Tseng CH, Clements PJ, et al. Predicting treatment outcomes and responder subsets in scleroderma-related ILD. Arthritis Rheum. 2011;63(9):2797–808.

    Article  PubMed Central  PubMed  Google Scholar 

  12. Sharif R, Mayes MD, Tan FK, et al. IRF5 polymorphisms predicts prognosis in pts with SS. Ann Rheum Dis. 2012;71(7):1197–202.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  13. Sfriso P, Cozzi F, Oliviero F, et al. CXCL11 in BAL and PFT decline in SS. Clin Exp Rheumatol. 2012;30(2 suppl 71):S71–5.

    PubMed  Google Scholar 

  14. Tiev KP, Hua-Huy T, Kettaneh A, et al. Alveolar concentration of nitric oxide predicts in PFT deterioration in scleroderma. Thorax. 2012;67(2):157–63.

    Article  PubMed  Google Scholar 

  15. Tashkin DP, Elashof R, Clement P, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655–66.

    Article  CAS  PubMed  Google Scholar 

  16. Hoyles RK, Ellis RW, Wellsbury J, et al. A multicenter prospective randomized double blind placebo controlled trial of corticosteroids and intravenous cyclophosphamide followed by azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum. 2006;54:1962–70.

    Article  Google Scholar 

  17. Mendoza FA, Nagle SJ, Lee JB, Jimenez SA. A prospective observational study of mycophenolate mofetil treatment in progressive diffuse cutaneous systemic sclerosis of recent onset. J Rheumatol. 2012;39(6):1241–7.

    Article  CAS  PubMed  Google Scholar 

  18. Daoussis D, Liossis SN, Tsamandas AC, Kalogeropoulou C, Paliogianni F, Sirinian C, Yiannopoulos G, Andonopoulos AP. Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis. Clin Exp Rheumatol. 2012;30(2 Suppl 71):S17–22.

    PubMed  Google Scholar 

  19. Beyer C, Distler O, Distler JH. Innovative antifibrotic therapies in SS. Curr Opin Rheumatol. 2012;24(3):274–80.

    Article  CAS  PubMed  Google Scholar 

  20. Tsuchiya Y, Takayanagi N, Sugiura H, et al. Lung disease directly associated with rheumatoid arthritis and their relationship to outcome. Eur Respir J. 2011;37(6):1411–7.

    Article  CAS  PubMed  Google Scholar 

  21. Olson AL, Swigris JJ, Springer DB, et al. rheumatoid arthritis—interstitial lung disease-associated mortality. Am J Respir Crit Care Med. 2011;183(3):372–6.

    Article  PubMed  Google Scholar 

  22. Kim EJ, Collard HR, King Jr TE. Rheumatoid arthritis-associated interstitial lung disease: the relevance of histopathologic and radiographic pattern. Chest. 2009;136(5):1397–405.

    Article  PubMed Central  PubMed  Google Scholar 

  23. Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26(4):586–93.

    Article  CAS  PubMed  Google Scholar 

  24. Furukawa H, Oka S, Shimada K, et al. Associate of human leukocyte antigen with interstitial lung disease in rheumatoid arthritis: a protective role of shared epitopes. PLoS One. 2012;7(5):e33133.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  25. Lynch 3rd JP, Weigt SS, DerHovanessian A, Fishbein MC, Gutierrez A, Belperio JA. Obliterative (constrictive) bronchiolitis. Semin Respir Crit Care Med. 2012;33(5):509–32.

    Article  PubMed  Google Scholar 

  26. Perez-Alvarez R, Perez-de-Lis M, Diaz-Lagares C, et al. Interstitial lung disease induced or exacerbated by TNF-targeted therapies: analysis of 122 cases. Semin Arthritis Rheum. 2011;41(2):256–64.

    Article  CAS  PubMed  Google Scholar 

  27. Dixon WG, Hyrich KL, Watson KD, et al. Influence of anti-TNF therapy on mortality in patients with rheumatoid arthritis associated interstitial lung disease: results from the British Society for Rheumatology Biologics Register. Ann Rheum Dis. 2010;69(6):1086–91.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  28. Hadjinicoaou AV, Nisar MK, Bhagat S, et al. Non infectious pulmonary complications of newer biologic agents for rheumatic diseases; a systemic review of the literature. Rheumatology (Oxford). 2011;50(12):2297–305.

    Article  Google Scholar 

  29. Cao H, Pan M, Kang Y, et al. Clinical manifestations of dermatomyositis and clinical amyopathic dermatomyositis patient with positive expression of anti-MDA5 antibody. Arthritis Care Res. 2012;64(10):1602–10.

    Article  Google Scholar 

  30. Marie I, Hatron PY, Dominiqye 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):3439–47.

    Article  CAS  PubMed  Google Scholar 

  31. Stanciu R, Guiguet M, Musset DT, et al. Antisynthetase syndrome with anti-Jo-1 antibodies in 48 patients: pulmonary involvement predicts disease-modifying antirheumatic drug use. J Rheumatol. 2012;39:1835–9.

    Article  PubMed  Google Scholar 

  32. Kalluri M, Sahn SA, Oddis CV, et al. Clinical profile of anti-PL-12 autoantibody. Cohort study and review of the literature. Chest. 2009;135(6):1550–6.

    Article  CAS  PubMed  Google Scholar 

  33. Le Goff B, Chérin P, Cantagrel A, et al. Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Rheum. 2009;61(1):108.

    Article  PubMed  Google Scholar 

  34. Henderson LA, Loring SH, Gill RR, et al. Shrinking lung syndrome as a manifestation of pleuritis: a new model based on pulmonary physiological studies. J Rheumatol. 2013;40(3):273–81.

    Article  PubMed  Google Scholar 

  35. Watanabe M, Naniwa T, Hara M, Arakawa T, Maeda TSO. Pulmonary manifestations in Sjogren’s syndrome: correlation analysis between chest computed tomographic findings and clinical subsets with poor prognosis in 80 patients. J Rheumatol. 2010;37(2):365–73.

    Article  PubMed  Google Scholar 

  36. Swartz MA, Vivino FB. Dramatic reversal of lymphocytic interstitial pneumonitis in Sjögren’s syndrome with rituximab. J Clin Rheumatol. 2011 Dec;17(8):454. J Rheumatol. 2010;37(2):365.

    Article  Google Scholar 

  37. Seo P, Yuan IM, Holbrook JT, et al. For the WGET Research Group. Damage caused by Wegener’s granulomatosis and its treatment: prospective data Form the Wegener’s Granulomatosis Etanercept Trial (WGET). Arthritis Rheum. 2005;52:2168–78.

    Article  CAS  PubMed  Google Scholar 

  38. Roubille C, Haraui B. Interstitial lung diseases induced or exacerbated by DMARDS and biologic agents in rheumatoid arthritis: a systemic literature review. Semin Arthritis Rheum. 2013 Oct 5. pii: S0049-0172(13)00201-1. doi: 10.1016/j.semarthrit.2013.09.005. [Epub ahead of print].

  39. Kim YJ, Song M, Ryu JC. Mechanisms underlying methotrexate-induced pulmonary toxicity. Expert Opin Drug Saf. 2009;8:451–8.

    Article  CAS  PubMed  Google Scholar 

  40. Guillon JM, Joly P, Autran B, et al. Minocycline-induced cell mediated hypersensitivity pneumonitis. Ann Intern Med. 1992;117:476–81.

    Article  CAS  PubMed  Google Scholar 

  41. Furukama H, Oka S, Shimada K. HLA-A *31:01 and methotrexate-induced interstitial lung disease in Japanese rheumatoid arthritis patients: a multidrug hypersensitivity marker? Ann Rheum Dis. 2013;72:153–5.

    Article  Google Scholar 

  42. Fischer A, West SG, Swigris JJ, et al. CTD associated ILD: a call for clarification. Chest. 2010;138(2):251–6.

    Article  PubMed Central  PubMed  Google Scholar 

  43. Castellino F, Goldberg H, Dellaripa PF. The impact of rheumatologic evaluation in the management of patients with interstitial lung disease. Rheumatology. 2011;50:483–93.

    Article  Google Scholar 

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Correspondence to Paul F. Dellaripa M.D. .

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Dellaripa, P.F., Flaherty, K.R. (2014). The Lung in Rheumatic Diseases. In: Dellaripa, P., Fischer, A., Flaherty, K. (eds) Pulmonary Manifestations of Rheumatic Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-0770-0_1

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  • DOI: https://doi.org/10.1007/978-1-4939-0770-0_1

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