HRCT imaging of pulmonary fibrosis in CTD-ILDs

High-resolution computed tomography (HRCT) is the gold standard for accurate interstitial lung disease (ILD) diagnosis in connective tissue diseases (CTDs)1,2

Julie

IN PATIENTS WITH SUSPECTED ILD, THE USE OF CHEST HRCT IS ESSENTIAL1

HRCT is the gold standard to reliably diagnose or exclude ILD1,2 Extent of fibrosis on HRCT suggests a poor prognosis in multiple CTD-ILD subtypes, and can provide further evidence of stability or worsening in patients with unclear evidence of progression.1

Which risk factors indicate poor prognosis and early mortality in CTD-ILDs?

The non-specific symptoms, heterogeneity and unpredictable disease course of ILDs can make it easy to overlook pulmonary fibrosis and a challenge to detect, often leading to a delayed diagnosis3–5 

Screening for CTD-ILD can have the dual benefits of identifying early-stage disease and more specifically identifying those at greatest risk for progression and functional decline6

IMAGING ATLAS OF ILDs

Improve your ability to diagnose fibrotic ILDs.

This atlas can assist you through the process of diagnosing an ILD, helping you recognize key clues and characteristics on chest HRCT and histopathology cuts from surgical lung biopsy.

Download the imaging atlas of HRCT scans

4 imagining scans, representing fibrotic ILDs and characteristic for them lung pathologies.

THE RADIOLOGIST HAS A CENTRAL ROLE IN THE DIAGNOSIS OF CTD-ILDs IN COLLABORATION WITH A RHEUMATOLOGIST7

The clinical history of patients with CTDs can assist the radiologist in:8

  • Contributing to multidisciplinary team discussion

  • Interpreting radiological features on HRCT scans

  • Offering valuable information to make a differential diagnosis of suspected ILD 

Collaborate with radiologists to agree on the use of a standardized HRCT reporting template9-11

With a radiologist, get access to the benefit of a standardized structured HRCT report to help reduce the risk of under-reporting important findings9

Download standardized structured HRCT report

Typical radiographic characteristics on HRCT indicative of fibrosis in CTD-ILDs include:12,13

  • traction bronchiectasis, with or without ground-glass opacities

  • Reticulations, with or without ground-glass opacities

  • Honeycombing, with or without ground-glass opacities

Background lexicon of HRCT patterns:

  • Usual interstitial pneumonia (UIP) is characterized by a less homogenous radiographic pattern, relative alteration of lung architecture, variable severity of fibrotic lesions, basilar-predominant honeycombing, and subpleural reticulation with traction bronchiectasis12,13

  • Non-specific interstitial pneumonia (NSIP) is characterized by a more homogenous radiographic pattern, relative preservation of lung architecture, and relatively symmetric subpleural ground-glass opacities with immediate subpleural sparing12,13

  • Lymphoid interstitial pneumonia (LIP) is characterized by scattered thin-walled cysts and ground-glass opacification12

Most SSc-ILD patients have a fibrotic NSIP pattern on HRCT, with a high proportion of ground-glass opacities, including presence of reticulation and traction bronchiectasis, although UIP pattern may be seen in a small proportion of patients.14,15

UIP is the most common HRCT pattern in RA-ILD, which is characterized by reticulation, honeycombing and traction bronchiectasis.16

Explore the role of HRCT in screening and diagnosing CTD-ILDs

 

Footnotes
  • CT, computed tomography; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; HP, hypersensitivity pneumonitis; HRCT, high-resolution computed tomography; ILA, interstitial lung abnormalities; ILD, interstitial lung disease; LIP, lymphoid interstitial pneumonia; MCTD-ILD, mixed connective tissue disease-associated interstitial lung disease; NSIP, non-specific interstitial pneumonia; PFT, pulmonary function test; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease; UIP, usual interstitial pneumonia.

  1. Geerts S, Wuyts W, de Langhe E, et al. Connective tissue disease associated interstitial pneumonia: a challenge for both rheumatologists and pulmonologists. Sarcoidosis Vasc Dif. 2017;34:326–335.
  2. Wallace B, Vummidi D, Khanna D. Management of connective tissue diseases associated interstitial lung disease: a review of the published literature. Curr Opin Rheumatol. 2016;28(3):236–245.
  3. Wells AU, Brown KK, Flaherty KR, et al. What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51(5):1800692. 
  4. Gulati M. Diagnostic assessment of patients with interstitial lung disease. Prim Care Respir J. 2011;20:120–127.
  5. Greiffo FR, Eickelberg O, Fernandez IE. Systems medicine advances in interstitial lung disease. Eur Respir Rev. 2017;26:170021.
  6. Fischer A, Strek ME, Cotton V, et al. Proceedings of the American College of Rheumatology/Association of Physicians of Great Britain and Ireland Connective Tissue Disease–Associated Interstitial Lung Disease Summit: a multidisciplinary approach to address challenges and opportunities. Arthritis Rheumatol. 2019;71(2):182–195.
  7. De Lorenzis E, Bosello SL, Varone F, et al. Multidisciplinary evaluation of interstitial lung diseases: new opportunities linked to rheumatologist involvement. Diagnostics (Basel). 2020;10(9):664. 
  8. Chung J, Goldin J. Interpretation of HRCT scans in the diagnosis of IPF: improving communication between pulmonologists and radiologists. Lung. 2018;196(5):561–567.
  9. Sverzellati N, Odone A, Silva M, et al. Structured reporting for fibrosing lung disease: a model shared by radiologist and pulmonologist. Radiol Med. 2018;123(4):245–253.
  10. Irion KL, Souza Jr AS, Marchiori E, da Silveira Dalla-Bona JP, Hochhegger B. Assessment of pulmonologists’ receptivity to a structured radiology report for interstitial lung disease. J Bras Pneumol. 2020;46(5):e20200164.
  11. Berkowitz EA, Bernheim A, Little BP. Introducing ILD-RADS: a pilot study of an interstitial lung disease standardized reporting template. J Am Coll Radiol. 2019;16(9 Pt A):1169–1172.
  12. Esposito AJ, Chu SG, Madan R, et al. Thoracic manifestations of rheumatoid arthritis. Clin Chest Med. 2019;40(3):545–560. 
  13. Denton CP, Wells AU, Coghlan JG. Major lung complications of systemic sclerosis. Nat Rev Rheumatol. 2018;14:511–527.
  14. Cottin V, Hirani N, Hotchkin D, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.
  15. Perelas A, Silver RM, Arrossi AV, et al. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020;8(3):304–320.
  16. Iqbal K, Kelly C. Treatment of rheumatoid arthritis-associated interstitial lung disease: a perspective review. Ther Adv Musculoskelet Dis. 2015;7(6):247–267.
  17. Dawson JK, Fewins HE, Desmond J, et al. Predictors of progression of HRCT diagnosed fibrosing alveolitis in patients with rheumatoid arthritis. Ann Rheum Dis. 2002;61:517–521.
  18. Kawano-Dourado L, Doyle TJ, Bonfiglioli K, et al. Baseline characteristics and progression of a spectrum of interstitial lung abnormalities and disease in rheumatoid arthritis. Chest. 2020:S0012-3692(20)31412-4. doi: 10.1016/j.chest.2020.04.061.
  19. Zamora-Legoff JA, Krause ML, Crowson CS, et al. Progressive decline of lung function in rheumatoid arthritis associated interstitial lung disease. Arthritis Rheumatol. 2017;69(3):542–549. 
  20. Yamakawa H, Sato S, Tsumiyama E, et al. Predictive factors of mortality in rheumatoid arthritis-associated interstitial lung disease analysed by modified HRCT classification of idiopathic pulmonary fibrosis according to the 2018 ATS/ERS/JRS/ALAT criteria. J Thorac Dis. 2019;11(12):5247–5257.
  21. Hoffmann-Vold AM, Fretheim H, Halse AK, et al. Tracking impact of interstitial lung disease in systemic sclerosis in a complete nationwide cohort. Am J Respir Crit Care Med. 2019;200:1258–1266. 

Resources for patients