Diagnosis of
CTD-ILDs 

High-resolution computed tomography (HRCT) combined with multidisciplinary discussion can enable confident interstitial lung disease (ILD) diagnosis in connective tissue diseases (CTDs)1–3

Julie

HRCT IS THE GOLD STANDARD TO RELIABLY DIAGNOSE OR EXCLUDE ILD1,2

Any CTD patient with complaints of dyspnea, cough with or without sputum, and Velcro crackles on chest auscultation lasting more than a month, should undergo workup including 
chest HRCT and PFTs2

PFTs SHOULD BE PERFORMED IN PATIENTS WITH UNEXPLAINED SYMPTOMS OR PHYSICAL EXAMINATION FINDINGS THAT ARE CONSISTENT WITH ILD1,4

  • ILD is characterized by PFTs showing restrictive lung physiology and impaired DLCO2

  • A normal PFT can indicate the presence of ILD if previous measurements showed supranormal values, illustrating the importance of comparison to previous tests1

  • Reduced DLCO is often the first physiologic manifestation of ILD,1 whereas a restrictive ventilatory defect, with proportionately reduced FVC and TLC, is characteristic of more advanced disease1,4 

  • CPETs can be useful in CTD-ILD to determine if the dyspnea is primarily caused by ILD, pulmonary vascular disease, cardiac disease or another etiology

HOW DO PATIENTS WITH CTDs PERCEIVE PULMONARY SYMPTOMS?

Awareness of patient language can help identify the symptoms of ILD in CTDs:6

  • Patients with ILD seldom refer to the actual act of breathing, but instead difficulty with breathing is indicated by the ability to perform activities central to functionality or life priorities6

  • Patients with ILD describe their relationship between cough and dyspnea – with cough arising from activities demanding inspiration, and cough exacerbating dyspnea6

  • Patient language may help discern dyspnea (demands of breathing in activities leading to difficulty breathing and chest tightness) from fatigue6

 

WHAT ARE THE COMMON PATIENT DESCRIPTORS FOR PULMONARY SYMPTOMS OF CTD-ILDs?

Common descriptors for ‘shortness of breath’ or ‘breathlessness’, and uncomfortable sensations related to inspiration, used by patients with CTD-ILDs6

Traditional descriptors such as ‘shortness of breath’ or ‘breathlessness’ were seldom voiced, instead phrases such as the ones in the figure below were used5
Common CTD-ILD patient descriptors related to inspiration from multicenter survey

Survey of 6 multicenter focus groups including 45 patients with CTD-ILDs (IIM-ILD (n=11), RA-ILD (n=13), SSc-ILD (n=17), and various other CTD diagnoses (n=4)).6

A MULTIDISCIPLINARY TEAM APPROACH IS ALSO REGARDED AS A GOLD STANDARD FOR DIAGNOSING ILD3

Close collaboration between rheumatologist, pulmonologist, radiologist, and pathologist is important in the diagnosis of ILD in patients with CTD7

A multidisciplinary team including a range of specialists should be involved in ILD diagnosis

Multidisciplinary diagnosis is associated with higher levels of diagnostic confidence and better interobserver agreement when compared to the individual roles in isolation3

In instances when an MDT is challenging to convene for a patient with ILD, multidisciplinary discussion can be conducted virtually or by telephone to enable each specialty group to contribute8

Learn more about how to proactively collaborate in an MDT for ILD diagnosis

How can you break the news of an ILD diagnosis to your patients with CTDs?

Breaking bad news is a complex communication task. The SPIKES model involves delivering bad news in clinical practice using six elements

Learn more about the SPIKES model that can help you break the bad news of an ILD diagnosis to your patients

sscild

HRCT is the gold standard method for noninvasive diagnosis of SSc-ILD9

How can you diagnose ILD in your patients with SSc?

raild

HRCT combined with multidisciplinary evaluation provides high confidence in diagnosis of ILD in patients with RA10–12

How can you diagnose ILD in your patients with RA?

pssild

HRCT combined with multidisciplinary evaluation increase diagnostic accuracy of ILD in patients with primary Sjögren's syndrome13–17

How can you diagnose ILD in your patients with primary Sjögren’s syndrome?

Once a CTD-ILD diagnosis has been reached, how should you manage your patient?

Footnotes
  • CCP, cyclic citrullinated peptide; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; CXR, chest X-ray; DLCO, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; NSIP, non-specific interstitial pneumonia; PFT, pulmonary function test; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc-ILD, systemic sclerosis-associated interstitial lung disease. 

  1.  Geerts S, Wuyts W, Langhe ED, 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. Walsh SLF. Multidisciplinary evaluation of interstitial lung diseases: current insights. Eur Respir Rev. 2017;26:170002.

  4. Spagnolo P, Cordier JF, Cottin V. Connective tissue diseases, multimorbidity and the ageing lung. Eur Respir J. 2016;47(5):1535–1558.

  5. Assayag D, Ryerson CJ. Determining respiratory impairment in connective tissue disease-associated interstitial lung disease. Rheum Dis Clin North Am. 2015;41(2):213–23.

  6. Mittoo S, Frankel S, LeSage D, et al. Patient perspectives in OMERACT provide an anchor for future metric development and improved approaches to healthcare delivery in connective tissue disease related interstitial lung disease (CTD-ILD). Curr Respir Med Rev. 2015;11(2):175–183.

  7. Dellaripa PF. Interstitial lung disease in the connective tissue diseases; a paradigm shift in diagnosis and treatment. Clin Immunol. 2018;186:71–73.

  8. Chung JH, Goldin JG. Interpretation of HRCT Scans in the Diagnosis of IPF: Improving Communication Between Pulmonologists and Radiologists. Lung. 2018;196:561–567. 

  9. Chowaniec M, Skoczyńska M, Sokolik R, et al. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56, 249–254.

  10. Cassone G, Manfredi A, Vacchi C, et al. Treatment of rheumatoid arthritis-associated interstitial lung disease: lights and shadows. J Clin Med. 2020;9(4):1082.

  11. Iqbal K and Kelly C. Treatment of rheumatoid arthritis-associated interstitial lung disease: a perspective review. Ther Adv Musculoskelet Dis. 2015;7(6):247–267.

  12. 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.

  13. Wells AU, Denton CP. Interstitial lung disease in connective tissue disease— mechanisms and management. Nat Rev Rheumatol. 2014;10(12):728–739.

  14. Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A. Lung complications of Sjogren syndrome. Eur Respir Rev. 2020;29(157):200021.

  15. 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.

  16. Manfredi A, Sebastiani M, Cerri S, et al. Prevalence and characterization of non-sicca onset primary Sjögren syndrome with interstitial lung involvement. Clin Rheumatol. 2017;36(6):1261–1268.

  17. Lee AS, Scofield RH, Hammitt KM, et al. Consensus guidelines for evaluation and management of pulmonary disease in Sjögren’s. Chest. 2020 Oct 20. doi: 10.1016/j. chest.2020.10.011.

  18. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.

  19. Ryerson CJ, Cayou C, Topp F, et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med. 2014;108(1):203-210. 

  20. Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980. 

  21. Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print]. 

  22. Sgalla G, Cerri S, Ferrari R, et al. Mindfulness-based stress reduction in patients with interstitial lung diseases: a pilot, single-centre observational study on safety and efficacy. BMJ Open Respir Res. 2015;2(1):e000065.  

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