HRCT PULMONARY FEATURES OF ILD PATIENTS ASSOCIATED WITH CONNECTIVE TISSUE DISEASES- AN OVERVIEW
Abstract
Connective tissue diseases (CTDs) include a spectrum of disorders that affect the connective tissue of the human body, They include autoimmune disorders such as rheumatoid arthritis (RA), systemic lupus erythematous (SLE), scleroderma, polymyositis/dermatomyositis and mixed connective tissue disease (MCTD) characterized by immune-mediated chronic inflammation and the development of fibrosis. In this review article we will present cases of patients diagnosed with connective tissue disorders who developed interstitial lung diseases. Rheumatoid arthritis RA-ILD in our case is presented with usual interstitial pneumonia pattern (UIP) and NSIP pattern, SLE, scleroderma, dermatomyositis, MCTD are more common presented with nonspecific interstitial pneumonia pattern (NSIP). Sjogren syndrome in our case is presented on HRCT as organizing pneumonia (OP), but we can not exclude for sure primary postinfectious cause. Lymphocytic interstitial pneumonia pattern (LIP) is also presented in one patient diagnosed with Sjogren syndrome. HRCT of the lungs is a key component in the multidisciplinary approach to the diagnosis of connective tissue diseases associated with interstitial lung disease. The evaluation of radiological lung changes in correlation with clinical data not only leads to a correct diagnosis in many cases, but in this context plays an important role and helps in determining the prognosis, monitoring the effectiveness of treatment, detecting disease progression or complications, and evaluating patients with worsening or acute symptoms.
Key words: Connective tissue diseases, ILD, HRCT, lungs, UIP, NSIP, OP, LIP.
References
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2. Woodhead F, et al. Pulmonary complications of connective tissue diseases. Clin Chest Med. 2008.
3. Park IN, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 2007.
4. Silva CS, Müller NL. Interstitial Lung Disease in the Setting of Collagen Vascular Disease. Elsevier. doi:10.1053/j.ro.2009.07.005.
5. Bois R, King Jr TE. Challenges in pulmonary fibrosis: The NSIP/UIP debate. Thorax. 2007 Nov; 62(11): 1008-1012.
6. Chung JH, Cox CW, Montner SM, Adegunsoye A, Oldham JM, Husain AN, Vij R, Noth I, Lynch DA, Strek ME. CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease from Idiopathic Pulmonary Fibrosis. AJR. 2018; 210.
7. Oliviera SD, Filho JAA, Paiva AFL, Ikari ES, Chate RC, Nomura CH. Interstitial pneumonias: review of the latest American thoracic society/European society classification. Radiol Bras. 2018 Sept-Oct; 51(5): 321-327.
8. Mink SN, Maycher B. Comparative manifestation and diagnostic accuracy of the high-resolution computed tomography in usual interstitial pneumonia and nonspecific interstitial pneumonia. Curr Opin Pulm Med. 2012 Sep; 18(5): 530-534.
9. Yoo H, Hino T, Hwang J, Franks TJ, Han J, Im Y, Lee HY, Chung MP, Hatabu H, Lee KS. Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): Evolving concept of CT findings, pathology, and management. Eur J Radiol Open. 2022.
10. Chung JH, Cox CW, Montner SM, Adegunsoye A, Oldham JM, Husain AN, Vij R, Noth I, Lynch DA, Strek ME. CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease from Idiopathic Pulmonary Fibrosis. AJR. 2019.
11. Qubo AA, Capaccione KM, Bernstein EJ, Padilla M, Salvatore M. The Role of Radiology in Progressive Fibrosing Interstitial Lung Disease. Front Med. 2022.
12. Raghu G, Chen SY, Yeh WS, Maroni B, Li Q, Lee YC, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med. 2014; 2: 566-572.
13. Ebner L, Christodoulidis S, Stathopoulos T, Geiser T, Stalder O, Limacher A, et al. Meta-analysis of the radiological and clinical features of usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP). Plos ONE. 2020; 15: e0226084.
14. Adegunsoye A, Oldham JM, Bellam SK, Montner SM, Churpek MM, Noth I, et al. CT honeycombing identifies a progressive fibrotic phenotype with increased mortality across diverse interstitial lung diseases. Ann Am Thorac Soc. 2019; 16: 580-588.
15. Hansell DM, Bankier AA, MacMahon H, McLoud TC, Muller NL, Remy J. Fleischner Society; glossary of terms for thoracic imaging. Radiology. 2008; 246(3): 697-722.
16. Tcherakian C, Cottin V, Brillet PY, et al. Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease. Thorax. 2011; 66(3): 226-231.
17. Gabriel SE. The epidemiology of rheumatoid arthritis. Rheum Dis Clin North Am. 2001; 27: 269–281. doi: 10.1016/s0889-857x(05)70201-5.
18. Hunninghake GW, Zimmerman MB, Schwartz DA, et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2001; 164: 193–196. doi: 10.1164/ajrccm.164.2.2101090.
19. American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias: this joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002; 165: 277–304. doi: 10.1164/ajrccm.165.2.ats01.
20. Sverzellati N, Lynch DA, Hansell DM, Johkoh T, King Jr TE, Travis WD. American Thoracic Society–European Respiratory Society Classification of the Idiopathic Interstitial Pneumonias: Advances in Knowledge since 2002. Radiographics. 2015; 35: 1849–1871.
21. Saito Z, Kaneko Y, Hasegawa T, Yoshida M, Odashima K, Horikiri T, et al. Predictive factors for relapse of cryptogenic organizing pneumonia. BMC Pulm Med. 2019; 19: 10.
22. Zare Mehrjardi M, Kahkouee S, Pourabdollah M. Radio-pathological correlation of organizing pneumonia (OP): A pictorial review. Br J Radiol. 2017; 90: 20160723.
23. Dhamija E, Weerakkody Y, Mayock P, et al. Cryptogenic organizing pneumonia. Reference article, Radiopaedia.org (Accessed on 25 Jan 2024).
24. Baque-Juston M, Pellegrin A, Leroy S, Marquette CH, Padovani B. Organizing pneumonia: What is it? A conceptual approach and pictorial review. Diagn Interv Imaging. 2014; 95(9): 771-777.
25. Kloth C, Thaiss WM, Beer M, Bösmüller H, Baumgartner K, Fritz J, Horger M. A pictorial essay. The many faces of cryptogenic organizing pneumonia (COP). J Clin Imaging Sci. 2022; 12(29).
26. Muller NL, Guerry-Force ML, Staples CA, et al. Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and radiologic findings. Radiology. 1987; 162: 151–156.
27. Lee KS, Kullnig P, Hartman TE, Muller NL. Cryptogenic organizing pneumonia: CT findings in 43 patients. AJR. 1994; 162: 543–546.
28. Muller NL, Staples CA, Miller RR. Bronchiolitis obliterans organizing pneumonia: CT features in 14 patients. AJR. 1990; 154: 983–987.
29. Akira M, Yamamoto S, Sakatani M. Bronchiolitis obliterans organizing pneumonia manifesting as multiple large nodules or masses. AJR. 1998; 170: 291–295.
30. Weerakkody Y, Yap J, Hacking C, et al. Lymphoid interstitial pneumonia. Reference article, Radiopaedia.org (Accessed on 26 Jan 2024).
31. Ferguson EC, Berkowitz EA. Lung CT: Part 2, The interstitial pneumonias-clinical, histologic, and CT manifestations. AJR Am J Roentgenol. 2012; 199(4): W464-476.
32. Dähnert W. Radiology Review Manual. Lippincott Williams & Wilkins. 2011. ISBN: 1609139437.
33. Weerakkody Y, Yap J, Hacking C, et al. Lymphoid interstitial pneumonia. Reference article, Radiopaedia.org (Accessed on 26 Jan 2024).
34. Ferguson EC, Berkowitz EA. Lung CT: Part 2, The interstitial pneumonias-clinical, histologic, and CT manifestations. AJR Am J Roentgenol. 2012; 199(4): W464-476.
35. Lymphoid Interstitial Pneumonia: A Narrative Review. doi:10.1378/chest.122.6.2150.
36. Honda O, Johkoh T, Ichikado K, et al. Differential diagnosis of lymphocytic interstitial pneumonia and malignant lymphoma on high-resolution CT. AJR Am J Roentgenol. 1999; 173.
37. Johkoh T, Müller NL, Pickford HA, et al. Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology. 1999; 212(2): 567-572.
38. Cha SI, Fessler MB, Cool CD, Schwarz MI, Brown KK. Lymphoid interstitial pneumonia: clinical features, associations, and prognosis. Eur Respir J. 2006; 28: 364-369. DOI: 10.1183/09031936.06.00076705.
39. Atzeni F, Gerardi MC, Barilaro G, Masala IF, Benucci M, Sarzi-Puttini P. Interstitial lung disease in systemic autoimmune rheumatic diseases: a comprehensive review. Expert Rev Clin Immunol. 2018 Jan; 14(1): 69-82.
40. Salinas M, Solomon JJ, Fernández Pérez ER. Connective Tissue Disease-Interstitial Lung Disease-Associated Mortality Rates and Years of Potential Life Lost in the United States. Chest. 2021 Oct; 160(4): 1368-1371.
41. Egashira R. High-Resolution CT Findings of Myositis-Related Interstitial Lung Disease. Medicina. 2021 Jul 6; 57(7): 692. doi: 10.3390/medicina57070692.
42. Egashira R. High-Resolution CT Findings of Myositis-Related Interstitial Lung Disease. Medicina. 2021 Jul 6; 57(7): 692. doi: 10.3390/medicina57070692.
43. Palmucci S, Galioto F, Fazio G, Ferlito A, Cancemi G, Di Mari A, Sambataro G, Sambataro D, Zanframundo G, Mauro LA, Foti PV, Vancheri C, Basile A. Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay. La Radiologia Medica. 2019 Jan 10; 124(1): 13-30.
44. Cohen AJ, Collard HR, Heffner JE, et al. Diagnosis and management of idiopathic pulmonary fibrosis and other interstitial lung diseases. Chest. 2011; 139(1): 56-58.
45. Yagihashi T, Itoh H, Kondo T, et al. Chest CT findings in patients with connective tissue disease-associated interstitial lung disease: comparison between rheumatoid arthritis, systemic sclerosis, and polymyositis/dermatomyositis. Radiology. 2012; 265(2): 450-456.
46. Weitzenblum E, Chaouat A. Pulmonary complications of connective tissue diseases. Curr Opin Rheumatol. 2005 Mar; 17(2): 222-228.
47. Tufano A, Ball M, Carretta A. Idiopathic pulmonary fibrosis and collagen vascular diseases. Pulmonary Medicine. 2012.
48.Wells AU, Cullinan P, Hansell DM, et al. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med. 1994;149:1583–1590.
49.Renzoni E, Rottoli P, Coviello G, et al. Clinical, laboratory and radiological findings in pulmonary fibrosis with and without connective tissue disease. Clin Rheumatol. 1997;16:570–577.
50.Muir TE, Tazelaar HD, Colby TV, et al. Organizing diffuse alveolar damage associated with progressive systemic sclerosis. Mayo Clin Proc. 1997;72:639–642.
51.Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655–2666.
52.Sharp GC, Irvin WS, Tan EM, et al. Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). Am J Med. 1972;52:148–159.
53.Bodolay E, Szekanecz Z, Devenyi K, et al. Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD). Rheumatology (Oxford). 2005;44:656–661.
54.Prakash UB. Respiratory complications in mixed connective tissue disease. Clin Chest Med. 1998;19:733–746.
55.Kozuka T, Johkoh T, Honda O, et al. Pulmonary involvement in mixed connective tissue disease: high-resolution CT findings in 41 patients. J Thorac Imag. 2001;16:94–98.
56.Weerakkody Y, Sharma R, Knipe H, et al. Anti-synthetase syndrome. Reference article, Radiopaedia.org (Accessed on 14 Feb 2025) https://doi.org/10.53347/rID-17810.
57.Debray M, Borie R, Revel M et al. Interstitial Lung Disease in Anti-Synthetase Syndrome: Initial and Follow-Up CT Findings. Eur J Radiol. 2015;84(3):516-23. doi:10.1016/j.ejrad.2014.11.026.
58.Marie I, Josse S, Decaux O et al. Clinical Manifestations and Outcome of Anti-PL7 Positive Patients with Antisynthetase Syndrome. Eur J Intern Med. 2013;24(5):474-9. doi:10.1016/j.ejim.2013.01.002.
59.Waseda Y., Johkoh T., Egashira R., Sumikawa H., Saeki K., Watanabe S., Matsunuma R., Takato H., Ichikawa Y., Hamaguchi Y., et al. Antisynthetase syndrome: Pulmonary computed tomography findings of adult patients with antibodies to aminoacyl-tRNA synthetases. Eur. J. Radiol. 2016;85:1421–1426. doi: 10.1016/j.ejrad.2016.05.012.
60.Zamora A.C., Hoskote S.S., Abascal-Bolado B., White D., Cox C.W., Ryu J.H., Moua T. Clinical features and outcomes of interstitial lung disease in anti-Jo-1 positive antisynthetase syndrome. Respir. Med. 2016;118:39–45. doi: 10.1016/j.rmed.2016.07.009.
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Published
2025-05-02
How to Cite
DIMITRIJEVIKJ, Kristina.
HRCT PULMONARY FEATURES OF ILD PATIENTS ASSOCIATED WITH CONNECTIVE TISSUE DISEASES- AN OVERVIEW.
Journal of Morphological Sciences, [S.l.], v. 8, n. 1, p. 81-92, may 2025.
ISSN 2545-4706.
Available at: <https://www.jms.mk/jms/article/view/vol8no1-10>. Date accessed: 01 oct. 2025.
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