Advertisement

Interstitial Lung Disease Associated with Connective Tissue Diseases

Chapter
First Online:
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 1304)

Abstract

Pulmonary manifestations of connective tissue diseases (CTD) carry high morbidity and potential mortality, and the most serious pulmonary type is interstitial lung disease (ILD). Identifying and promptly intervening CTD-ILD with immune suppressor therapy will change the natural course of the disease resulting in survival improvement. Compared to idiopathic pulmonary fibrosis, the most common presentation of idiopathic interstitial pneumonia (IIP), CTD-ILD carries a better prognosis due to the response to immune suppressor therapy. Nonspecific interstitial pneumonia (NSIP) is the most common type of CTD-ILD that is different from the fibrotic classical presentation of IPF, known as usual interstitial pneumonia (UIP). An exception is rheumatoid arthritis that presents more frequently with UIP type. Occasionally, IPF may not have typical radiographic features of UIP, and a full assessment to differentiate IPF from CTD-ILD is necessary, including the intervention of a multidisciplinary team and the histopathology. Interstitial pneumonia with autoimmune features (IPAF) shows promising advantages to identify patients with ILD who have some features of a CTD without a defined autoimmune disease and who may benefit from immune suppressors. A composition of clinical, serological, and morphologic features in patients presenting with ILD will fulfill criteria for IPAF. In summary, the early recognition and treatment of CTD-ILD, differentiation from IPF-UIP, and identification of patients with IPAF fulfill the assessment by the clinician for an optimal care.

Keywords

Interstitial lung disease Connective tissue disease Interstitial pneumonia with autoimmune features 
This is a preview of subscription content, log in to check access.

References

  1. 1.
    Klemperer P. The concept of connective-tissue disease. Circulation. 1962;25:869–71.PubMedCrossRefPubMedCentralGoogle Scholar
  2. 2.
    Klemperer P. The concept of collagen diseases in medicine. Am Rev Respir Dis. 1961;83:331–9.PubMedPubMedCentralGoogle Scholar
  3. 3.
    Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66(7):940–4.PubMedPubMedCentralCrossRefGoogle Scholar
  4. 4.
    Connors GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest. 2010;138(6):1464–74.PubMedCrossRefPubMedCentralGoogle Scholar
  5. 5.
    Olson AL, Swigris JJ, Sprunger DB, Fischer A, Fernandez-Perez ER, Solomon J, et al. Rheumatoid arthritis-interstitial lung disease-associated mortality. Am J Respir Crit Care Med. 2011;183(3):372–8.PubMedPubMedCentralCrossRefGoogle Scholar
  6. 6.
    Turesson C, O’Fallon WM, Crowson CS, Gabriel SE, Matteson EL. Extra-articular disease manifestations in rheumatoid arthritis: incidence trends and risk factors over 46 years. Ann Rheum Dis. 2003;62(8):722–7.PubMedPubMedCentralCrossRefGoogle Scholar
  7. 7.
    Nobata K, Kasai T, Fujimura M, Mizuguchi M, Nishi K, Ishiura Y, et al. Pulmonary sarcoidosis with usual interstitial pneumonia distributed predominantly in the lower lung fields. Intern Med. 2006;45(6):359–62.PubMedCrossRefPubMedCentralGoogle Scholar
  8. 8.
    Yamamoto S. Histopathological features of pulmonary asbestosis with particular emphasis on the comparison with those of usual interstitial pneumonia. Osaka City Med J. 1997;43(2):225–42.PubMedPubMedCentralGoogle Scholar
  9. 9.
    Kelly C, Iqbal K, Iman-Gutierrez L, Evans P, Manchegowda K. Lung involvement in inflammatory rheumatic diseases. Best Pract Res Clin Rheumatol. 2016;30(5):870–88.PubMedCrossRefPubMedCentralGoogle Scholar
  10. 10.
    Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, 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
  11. 11.
    Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44–68.PubMedCrossRefPubMedCentralGoogle Scholar
  12. 12.
    Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med. 2002;165(12):1581–6.PubMedCrossRefPubMedCentralGoogle Scholar
  13. 13.
    Park JH, Kim DS, Park IN, Jang SJ, Kitaichi M, Nicholson AG, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med. 2007;175(7):705–11.PubMedCrossRefPubMedCentralGoogle Scholar
  14. 14.
    Fischer A, Swigris JJ, Groshong SD, Cool CD, Sahin H, Lynch DA, et al. Clinically significant interstitial lung disease in limited scleroderma: histopathology, clinical features, and survival. Chest. 2008;134(3):601–5.PubMedCrossRefPubMedCentralGoogle Scholar
  15. 15.
    Lee HK, Kim DS, Yoo B, Seo JB, Rho JY, Colby TV, et al. Histopathologic pattern and clinical features of rheumatoid arthritis-associated interstitial lung disease. Chest. 2005;127(6):2019–27.PubMedCrossRefPubMedCentralGoogle Scholar
  16. 16.
    Kim DS, Yoo B, Lee JS, Kim EK, Lim CM, Lee SD, et al. The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19(2):121–7.PubMedPubMedCentralGoogle Scholar
  17. 17.
    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(10):1410–5.PubMedCrossRefPubMedCentralGoogle Scholar
  18. 18.
    Hernandez-Gonzalez F, Prieto-Gonzalez S, Brito-Zeron P, Cuerpo S, Sanchez M, Ramirez J, et al. Impact of a systematic evaluation of connective tissue disease on diagnosis approach in patients with interstitial lung diseases. Medicine (Baltimore). 2020;99(4):e18589.CrossRefGoogle Scholar
  19. 19.
    Idiopathic Pulmonary Fibrosis Clinical Research N, Raghu G, Anstrom KJ, King TE Jr, Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366(21):1968–77.CrossRefGoogle Scholar
  20. 20.
    Graney BA, Fischer A. Interstitial pneumonia with autoimmune features. Ann Am Thorac Soc. 2019;16(5):525–33.PubMedPubMedCentralCrossRefGoogle Scholar
  21. 21.
    Strange C, Highland KB. Interstitial lung disease in the patient who has connective tissue disease. Clin Chest Med. 2004;25(3):549–59, vii.PubMedCrossRefPubMedCentralGoogle Scholar
  22. 22.
    Duron L, Cohen-Aubart F, Diot E, Borie R, Abad S, Richez C, et al. Shrinking lung syndrome associated with systemic lupus erythematosus: a multicenter collaborative study of 15 new cases and a review of the 155 cases in the literature focusing on treatment response and long-term outcomes. Autoimmun Rev. 2016;15(10):994–1000.PubMedCrossRefPubMedCentralGoogle Scholar
  23. 23.
    Borrell H, Narvaez J, Alegre JJ, Castellvi I, Mitjavila F, Aparicio M, et al. Shrinking lung syndrome in systemic lupus erythematosus: a case series and review of the literature. Medicine (Baltimore). 2016;95(33):e4626.CrossRefGoogle Scholar
  24. 24.
    Demoruelle MK, Mittoo S, Solomon JJ. Connective tissue disease-related interstitial lung disease. Best Pract Res Clin Rheumatol. 2016;30(1):39–52.PubMedCrossRefPubMedCentralGoogle Scholar
  25. 25.
    Fischer A, du Bois R. Interstitial lung disease in connective tissue disorders. Lancet. 2012;380(9842):689–98.PubMedCrossRefPubMedCentralGoogle Scholar
  26. 26.
    Pitcher RD, Zar HJ. Radiographic features of paediatric pneumocystis pneumonia -- a historical perspective. Clin Radiol. 2008;63(6):666–72.PubMedCrossRefPubMedCentralGoogle Scholar
  27. 27.
    Mittoo S, Gelber AC, Christopher-Stine L, Horton MR, Lechtzin N, Danoff SK. Ascertainment of collagen vascular disease in patients presenting with interstitial lung disease. Respir Med. 2009;103(8):1152–8.PubMedCrossRefPubMedCentralGoogle Scholar
  28. 28.
    Marigliano B, Soriano A, Margiotta D, Vadacca M, Afeltra A. Lung involvement in connective tissue diseases: a comprehensive review and a focus on rheumatoid arthritis. Autoimmun Rev. 2013;12(11):1076–84.PubMedCrossRefPubMedCentralGoogle Scholar
  29. 29.
    Fischer A, Swigris JJ, du Bois RM, Groshong SD, Cool CD, Sahin H, et al. Minor salivary gland biopsy to detect primary Sjogren syndrome in patients with interstitial lung disease. Chest. 2009;136(4):1072–8.PubMedPubMedCentralCrossRefGoogle Scholar
  30. 30.
    Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjogren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis. 2017;76(1):9–16.PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, et al. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology. 1990;176(3):755–9.PubMedCrossRefPubMedCentralGoogle Scholar
  32. 32.
    Maricq HR, LeRoy EC. Patterns of finger capillary abnormalities in connective tissue disease by "wide-field" microscopy. Arthritis Rheum. 1973;16(5):619–28.PubMedCrossRefPubMedCentralGoogle Scholar
  33. 33.
    Cutolo M, Sulli A, Pizzorni C, Accardo S. Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis. J Rheumatol. 2000;27(1):155–60.Google Scholar
  34. 34.
    Fischer A, Meehan RT, Feghali-Bostwick CA, West SG, Brown KK. Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease. Chest. 2006;130(4):976–81.PubMedCrossRefPubMedCentralGoogle Scholar
  35. 35.
    Smith V, Decuman S, Sulli A, Bonroy C, Piettte Y, Deschepper E, et al. Do worsening scleroderma capillaroscopic patterns predict future severe organ involvement? A pilot study. Ann Rheum Dis. 2012;71(10):1636–9.PubMedCrossRefPubMedCentralGoogle Scholar
  36. 36.
    Smith V, Riccieri V, Pizzorni C, Decuman S, Deschepper E, Bonroy C, et al. Nailfold capillaroscopy for prediction of novel future severe organ involvement in systemic sclerosis. J Rheumatol. 2013;40(12):2023–8.PubMedCrossRefPubMedCentralGoogle Scholar
  37. 37.
    van Roon AM, Huisman CC, van Roon AM, Zhang D, Stel AJ, Smit AJ, et al. Abnormal Nailfold Capillaroscopy is common in patients with connective tissue disease and associated with abnormal pulmonary function tests. J Rheumatol. 2019;46(9):1109–16.PubMedCrossRefPubMedCentralGoogle Scholar
  38. 38.
    Corrado A, Correale M, Mansueto N, Monaco I, Carriero A, Mele A, et al. Nailfold capillaroscopic changes in patients with idiopathic pulmonary arterial hypertension and systemic sclerosis-related pulmonary arterial hypertension. Microvasc Res. 2017;114:46–51.PubMedCrossRefPubMedCentralGoogle Scholar
  39. 39.
    Hofstee HM, Vonk Noordegraaf A, Voskuyl AE, Dijkmans BA, Postmus PE, Smulders YM, et al. Nailfold capillary density is associated with the presence and severity of pulmonary arterial hypertension in systemic sclerosis. Ann Rheum Dis. 2009;68(2):191–5.PubMedCrossRefPubMedCentralGoogle Scholar
  40. 40.
    Fischer A, Pfalzgraf FJ, Feghali-Bostwick CA, Wright TM, Curran-Everett D, West SG, et al. Anti-th/to-positivity in a cohort of patients with idiopathic pulmonary fibrosis. J Rheumatol. 2006;33(8):1600–5.PubMedPubMedCentralGoogle Scholar
  41. 41.
    Wells AU, Cullinan P, Hansell DM, Rubens MB, Black CM, Newman-Taylor AJ, et al. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med. 1994;149(6):1583–90.PubMedCrossRefPubMedCentralGoogle Scholar
  42. 42.
    Wells AU, Margaritopoulos GA, Antoniou KM, Denton C. Interstitial lung disease in systemic sclerosis. Semin Respir Crit Care Med. 2014;35(2):213–21.PubMedCrossRefPubMedCentralGoogle Scholar
  43. 43.
    Moore OA, Goh N, Corte T, Rouse H, Hennessy O, Thakkar V, et al. Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford). 2013;52(1):155–60.CrossRefGoogle Scholar
  44. 44.
    Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177(11):1248–54.PubMedCrossRefPubMedCentralGoogle Scholar
  45. 45.
    Hyldgaard C, Hilberg O, Pedersen AB, Ulrichsen SP, Lokke A, Bendstrup E, et al. A population-based cohort study of rheumatoid arthritis-associated interstitial lung disease: comorbidity and mortality. Ann Rheum Dis. 2017;76(10):1700–6.PubMedCrossRefPubMedCentralGoogle Scholar
  46. 46.
    Kelly CA, Saravanan V, Nisar M, Arthanari S, Woodhead FA, Price-Forbes AN, et al. Rheumatoid arthritis-related interstitial lung disease: associations, prognostic factors and physiological and radiological characteristics--a large multicentre UK study. Rheumatology (Oxford). 2014;53(9):1676–82.CrossRefGoogle Scholar
  47. 47.
    Solomon JJ, Chung JH, Cosgrove GP, Demoruelle MK, Fernandez-Perez ER, Fischer A, et al. Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J. 2016;47(2):588–96.PubMedCrossRefPubMedCentralGoogle Scholar
  48. 48.
    Kondoh Y, Taniguchi H, Ogura T, Johkoh T, Fujimoto K, Sumikawa H, et al. Disease progression in idiopathic pulmonary fibrosis without pulmonary function impairment. Respirology. 2013;18(5):820–6.PubMedCrossRefPubMedCentralGoogle Scholar
  49. 49.
    Cottin V, Nunes H, Brillet PY, Delaval P, Devouassoux G, Tillie-Leblond I, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26(4):586–93.PubMedCrossRefPubMedCentralGoogle Scholar
  50. 50.
    Kim EA, Lee KS, Johkoh T, Kim TS, Suh GY, Kwon OJ, et al. Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. Radiographics : a review publication of the Radiological Society of North America, Inc. 2002;22 Spec No:S151-65.Google Scholar
  51. 51.
    Walsh SL, Sverzellati N, Devaraj A, Keir GJ, Wells AU, Hansell DM. Connective tissue disease related fibrotic lung disease: high resolution computed tomographic and pulmonary function indices as prognostic determinants. Thorax. 2014;69(3):216–22.PubMedCrossRefPubMedCentralGoogle Scholar
  52. 52.
    Monaghan H, Wells AU, Colby TV, du Bois RM, Hansell DM, Nicholson AG. Prognostic implications of histologic patterns in multiple surgical lung biopsies from patients with idiopathic interstitial pneumonias. Chest. 2004;125(2):522–6.PubMedCrossRefPubMedCentralGoogle Scholar
  53. 53.
    Flaherty KR, Thwaite EL, Kazerooni EA, Gross BH, Toews GB, Colby TV, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003;58(2):143–8.PubMedPubMedCentralCrossRefGoogle Scholar
  54. 54.
    Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011;140(5):1292–9.PubMedPubMedCentralCrossRefGoogle Scholar
  55. 55.
    Flaherty KR, Khanna D. Idiopathic or connective tissue disease-associated interstitial lung disease: a case of HRCT mimicry. Thorax. 2014;69(3):205–6.PubMedPubMedCentralCrossRefGoogle Scholar
  56. 56.
    Kim EJ, Elicker BM, Maldonado F, Webb WR, Ryu JH, Van Uden JH, et al. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J. 2010;35(6):1322–8.PubMedCrossRefPubMedCentralGoogle Scholar
  57. 57.
    Zamora-Legoff JA, Krause ML, Crowson CS, Ryu JH, Matteson EL. Progressive decline of lung function in rheumatoid arthritis-associated interstitial lung disease. Arthritis Rheumatol. 2017;69(3):542–9.PubMedPubMedCentralCrossRefGoogle Scholar
  58. 58.
    Mira-Avendano IC, Abril A. Pulmonary manifestations of Sjogren syndrome, systemic lupus erythematosus, and mixed connective tissue disease. Rheum Dis Clin N Am. 2015;41(2):263–77.CrossRefGoogle Scholar
  59. 59.
    Kinder BW, Shariat C, Collard HR, Koth LL, Wolters PJ, Golden JA, et al. Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function. Lung. 2010;188(2):143–9.PubMedPubMedCentralCrossRefGoogle Scholar
  60. 60.
    Mosca M, Tani C, Neri C, Baldini C, Bombardieri S. Undifferentiated connective tissue diseases (UCTD). Autoimmun Rev. 2006;6(1):1–4.PubMedCrossRefPubMedCentralGoogle Scholar
  61. 61.
    Mira-Avendano I, Abril A, Burger CD, Dellaripa PF, Fischer A, Gotway MB, et al. Interstitial lung disease and other pulmonary manifestations in connective tissue diseases. Mayo Clin Proc. 2019;94(2):309–25.PubMedCrossRefPubMedCentralGoogle Scholar
  62. 62.
    Lynch DA, Sverzellati N, Travis WD, Brown KK, Colby TV, Galvin JR, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6(2):138–53.CrossRefGoogle Scholar
  63. 63.
    Capobianco J, Grimberg A, Thompson BM, Antunes VB, Jasinowodolinski D, Meirelles GS. Thoracic manifestations of collagen vascular diseases. Radiographics : a review publication of the Radiological Society of North America, Inc. 2012;32(1):33–50.CrossRefGoogle Scholar
  64. 64.
    Kligerman SJ, Groshong S, Brown KK, Lynch DA. Nonspecific interstitial pneumonia: radiologic, clinical, and pathologic considerations. Radiographics : a review publication of the Radiological Society of North America, Inc. 2009;29(1):73–87.CrossRefGoogle Scholar
  65. 65.
    Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 2010;138(2):251–6.PubMedPubMedCentralCrossRefGoogle Scholar
  66. 66.
    Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S. Pulmonary manifestations of Sjogren’s syndrome. Eur Respir Rev. 2016;25(140):110–23.PubMedCrossRefPubMedCentralGoogle Scholar
  67. 67.
    Panchabhai TS, Farver C, Highland KB. Lymphocytic interstitial pneumonia. Clin Chest Med. 2016;37(3):463–74.PubMedCrossRefPubMedCentralGoogle Scholar
  68. 68.
    Argyropoulou OD, Chatzis LG, Rontogianni D, Tzioufas AG. Autoimmune epithelitis beyond the exocrine glands: an unusual case of anti-Ro/La and Scl-70 lymphocytic interstitial pneumonia. Clin Exp Rheumatol. 2019;37 Suppl 118(3):249–51.Google Scholar
  69. 69.
    Skopouli FN, Moutsopoulos HM. Autoimmune epitheliitis: Sjogren’s syndrome. Clin Exp Rheumatol. 1994;12(Suppl 11):S9–11.PubMedPubMedCentralGoogle Scholar
  70. 70.
    Daniels TE, Cox D, Shiboski CH, Schiodt M, Wu A, Lanfranchi H, et al. Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjogren’s syndrome among 1,726 registry participants. Arthritis Rheum. 2011;63(7):2021–30.PubMedPubMedCentralCrossRefGoogle Scholar
  71. 71.
    Yousem SA, Colby TV, Carrington CB. Follicular bronchitis/bronchiolitis. Hum Pathol. 1985;16(7):700–6.PubMedCrossRefPubMedCentralGoogle Scholar
  72. 72.
    Ryu JH, Myers JL, Swensen SJ. Bronchiolar disorders. Am J Respir Crit Care Med. 2003;168(11):1277–92.PubMedCrossRefPubMedCentralGoogle Scholar
  73. 73.
    Ford JA, Liu X, Chu SH, Lu B, Cho MH, Silverman EK, et al. Asthma, chronic obstructive pulmonary disease, and subsequent risk for incident rheumatoid arthritis among women: a prospective cohort study. Arthritis Rheumatol. 2020;72(5):704–13.PubMedPubMedCentralCrossRefGoogle Scholar
  74. 74.
    Willis VC, Demoruelle MK, Derber LA, Chartier-Logan CJ, Parish MC, Pedraza IF, et al. Sputum autoantibodies in patients with established rheumatoid arthritis and subjects at risk of future clinically apparent disease. Arthritis Rheum. 2013;65(10):2545–54.PubMedPubMedCentralGoogle Scholar
  75. 75.
    Reynisdottir G, Olsen H, Joshua V, Engstrom M, Forsslund H, Karimi R, et al. Signs of immune activation and local inflammation are present in the bronchial tissue of patients with untreated early rheumatoid arthritis. Ann Rheum Dis. 2016;75(9):1722–7.PubMedCrossRefPubMedCentralGoogle Scholar
  76. 76.
    Gizinski AM, Mascolo M, Loucks JL, Kervitsky A, Meehan RT, Brown KK, et al. Rheumatoid arthritis (RA)-specific autoantibodies in patients with interstitial lung disease and absence of clinically apparent articular RA. Clin Rheumatol. 2009;28(5):611–3.PubMedPubMedCentralCrossRefGoogle Scholar
  77. 77.
    Demoruelle MK, Solomon JJ, Fischer A, Deane KD. The lung may play a role in the pathogenesis of rheumatoid arthritis. Int J Clin Rheumatol. 2014;9(3):295–309.CrossRefGoogle Scholar
  78. 78.
    Fischer A, Solomon JJ, du Bois RM, Deane KD, Olson AL, Fernandez-Perez ER, et al. Lung disease with anti-CCP antibodies but not rheumatoid arthritis or connective tissue disease. Respir Med. 2012;106(7):1040–7.PubMedPubMedCentralCrossRefGoogle Scholar
  79. 79.
    Rangel-Moreno J, Hartson L, Navarro C, Gaxiola M, Selman M, Randall TD. Inducible bronchus-associated lymphoid tissue (iBALT) in patients with pulmonary complications of rheumatoid arthritis. J Clin Invest. 2006;116(12):3183–94.PubMedPubMedCentralCrossRefGoogle Scholar
  80. 80.
    Raj R, Raparia K, Lynch DA, Brown KK. Surgical lung biopsy for interstitial lung diseases. Chest. 2017;151(5):1131–40.PubMedCrossRefPubMedCentralGoogle Scholar
  81. 81.
    Travis WD, Hunninghake G, King TE Jr, Lynch DA, Colby TV, Galvin JR, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med. 2008;177(12):1338–47.PubMedCrossRefPubMedCentralGoogle Scholar
  82. 82.
    Kono M, Nakamura Y, Yoshimura K, Enomoto Y, Oyama Y, Hozumi H, et al. Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. Respir Med. 2016;117:40–7.PubMedCrossRefPubMedCentralGoogle Scholar
  83. 83.
    Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46(4):976–87.PubMedCrossRefPubMedCentralGoogle Scholar
  84. 84.
    Enomoto Y, Takemura T, Hagiwara E, Iwasawa T, Fukuda Y, Yanagawa N, et al. Prognostic factors in interstitial lung disease associated with primary Sjogren’s syndrome: a retrospective analysis of 33 pathologically-proven cases. PLoS One. 2013;8(9):e73774.PubMedPubMedCentralCrossRefGoogle Scholar
  85. 85.
    Flaherty KR, Travis WD, Colby TV, Toews GB, Kazerooni EA, Gross BH, et al. Histopathologic variability in usual and nonspecific interstitial pneumonias. Am J Respir Crit Care Med. 2001;164(9):1722–7.PubMedCrossRefPubMedCentralGoogle Scholar
  86. 86.
    Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol. 1994;18(2):136–47.PubMedCrossRefPubMedCentralGoogle Scholar
  87. 87.
    Cottin V, Cordier JF. Cryptogenic organizing pneumonia. Semin Respir Crit Care Med. 2012;33(5):462–75.PubMedCrossRefPubMedCentralGoogle Scholar
  88. 88.
    Thille AW, Esteban A, Fernandez-Segoviano P, Rodriguez JM, Aramburu JA, Vargas-Errazuriz P, et al. Chronology of histological lesions in acute respiratory distress syndrome with diffuse alveolar damage: a prospective cohort study of clinical autopsies. Lancet Respir Med. 2013;1(5):395–401.PubMedCrossRefPubMedCentralGoogle Scholar
  89. 89.
    Cardinal-Fernandez P, Lorente JA, Ballen-Barragan A, Matute-Bello G. Acute respiratory distress syndrome and diffuse alveolar damage. New insights on a complex relationship. Ann Am Thorac Soc. 2017;14(6):844–50.PubMedCrossRefPubMedCentralGoogle Scholar
  90. 90.
    Cardinal-Fernandez P, Bajwa EK, Dominguez-Calvo A, Menendez JM, Papazian L, Thompson BT. The presence of diffuse alveolar damage on open lung biopsy is associated with mortality in patients with acute respiratory distress syndrome: a systematic review and meta-analysis. Chest. 2016;149(5):1155–64.PubMedCrossRefPubMedCentralGoogle Scholar
  91. 91.
    Cipriani NA, Strek M, Noth I, Gordon IO, Charbeneau J, Krishnan JA, et al. Pathologic quantification of connective tissue disease-associated versus idiopathic usual interstitial pneumonia. Arch Pathol Lab Med. 2012;136(10):1253–8.PubMedCrossRefPubMedCentralGoogle Scholar
  92. 92.
    Song JW, Do KH, Kim MY, Jang SJ, Colby TV, Kim DS. Pathologic and radiologic differences between idiopathic and collagen vascular disease-related usual interstitial pneumonia. Chest. 2009;136(1):23–30.PubMedCrossRefPubMedCentralGoogle Scholar
  93. 93.
    Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B, et al. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med. 2007;176(7):691–7.PubMedPubMedCentralCrossRefGoogle Scholar
  94. 94.
    Corte TJ, Copley SJ, Desai SR, Zappala CJ, Hansell DM, Nicholson AG, et al. Significance of connective tissue disease features in idiopathic interstitial pneumonia. Eur Respir J. 2012;39(3):661–8.PubMedCrossRefPubMedCentralGoogle Scholar
  95. 95.
    LeRoy EC, Maricq HR, Kahaleh MB. Undifferentiated connective tissue syndromes. Arthritis Rheum. 1980;23(3):341–3.PubMedCrossRefPubMedCentralGoogle Scholar
  96. 96.
    Bodolay E, Csiki Z, Szekanecz Z, Ben T, Kiss E, Zeher M, et al. Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD). Clin Exp Rheumatol. 2003;21(3):313–20.PubMedPubMedCentralGoogle Scholar
  97. 97.
    Conti V, Esposito A, Cagliuso M, Fantauzzi A, Pastori D, Mezzaroma I, et al. Undifferentiated connective tissue disease - an unsolved problem:revision of literature and case studies. Int J Immunopathol Pharmacol. 2010;23(1):271–8.PubMedCrossRefPubMedCentralGoogle Scholar
  98. 98.
    Vaz CC, Couto M, Medeiros D, Miranda L, Costa J, Nero P, et al. Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients. Clin Rheumatol. 2009;28(8):915–21.PubMedCrossRefPubMedCentralGoogle Scholar
  99. 99.
    Oldham JM, Adegunsoye A, Valenzi E, Lee C, Witt L, Chen L, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47(6):1767–75.PubMedPubMedCentralCrossRefGoogle Scholar
  100. 100.
    Cotton CV, Spencer LG, New RP, Cooper RG. The utility of comprehensive autoantibody testing to differentiate connective tissue disease associated and idiopathic interstitial lung disease subgroup cases. Rheumatology (Oxford). 2017;56(8):1264–71.Google Scholar
  101. 101.
    Bryson T, Sundaram B, Khanna D, Kazerooni EA. Connective tissue disease-associated interstitial pneumonia and idiopathic interstitial pneumonia: similarity and difference. Semin Ultrasound CT MR. 2014;35(1):29–38.PubMedCrossRefPubMedCentralGoogle Scholar
  102. 102.
    Hervier B, Uzunhan Y. Inflammatory myopathy-related interstitial lung disease: from pathophysiology to treatment. Front Med. 2019;6:326.CrossRefGoogle Scholar
  103. 103.
    Betteridge ZE, Gunawardena H, McHugh NJ. Novel autoantibodies and clinical phenotypes in adult and juvenile myositis. Arthritis Res Ther. 2011;13(2):209.PubMedPubMedCentralCrossRefGoogle Scholar

Copyright information

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2021

Authors and Affiliations

  1. 1.Division of Rheumatology, Department of MedicineAlbany Medical CollegeAlbanyUSA
  2. 2.RheumatologyAlaska Native Medical CenterAnchorageUSA
  3. 3.Division of Pulmonary & Critical Care MedicineAlbany Medical CollegeAlbanyUSA

Personalised recommendations

Cite chapter

Buy options