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Connective tissue disease-associated interstitial lung disease

Key sources
The following summarized guidelines for the evaluation and management of connective tissue disease-associated interstitial lung disease are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023; 2021), the American Thoracic Society (ATS 2023), the Latin American Thoracic Association (ALAT/ERS/JRS/ATS 2022; 2018), the European Respiratory Society (ERS 2022), the British Society for Rheumatology (BSR 2022), the Japan College of Rheumatology (JCR 2022), the European Society of Cardiology (ESC/ERS 2022), the American College of Chest Physicians (ACCP 2018), the French Society of Pulmonology (SPLF 2017), the European League Against Rheumatism (EULAR 2017), the British Thoracic Society (BTS 2017; 2015; 2013), and the British Thoracic Society (BTS/TSANZ/ITS 2008).
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Guidelines

1.Screening and diagnosis

Indications for screening: as per ACR 2023 guidelines, consider obtaining screening for ILD with pulmonary function testing, HRCT of the chest, or both, in patients with SARDs at increased risk of developing ILD.
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2.Diagnostic investigations

Medical history: as per ATS 2018 guidelines, elicit a detailed history of both medication use and environmental exposures at home, work, and other places the patient frequently visits to exclude potential causes of ILD.
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  • Chest CT

  • Pulmonary function testing

  • Laboratory testing (initial tests)

  • Laboratory testing (serologic tests)

  • Laboratory testing (other tests)

  • TTE

3.Diagnostic procedures

Transbronchial lung biopsy, indications, ALAT/JRS/ERS/ATS: consider performing transbronchial lung cryobiopsy as an alternative to surgical lung biopsy for making a histopathological diagnosis in patients with ILD of undetermined type in centers with experience performing and interpreting transbronchial lung biopsy.
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  • Transbronchial lung biopsy (technical considerations)

  • Surgical lung biopsy

  • BAL

4.Respiratory support

Oxygen therapy: as per ACR 2021 guidelines, offer long-term oxygen therapy in patients withSjögren's disease with suspected ILD and clinically significant resting hypoxemia (defined by resting oxygen saturation < 88%, PaO2 < 55 mmHg or < 60 mmHg with complication of chronic hypoxemia, such as cor pulmonale).
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5.Medical management

Setting of care
Ensure access to a multidisciplinary team based in a regional center with expertise in ILD for all patients with ILD.
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Refer patients with ILD to a regional ILD in case of perceived difficulties in diagnosis and/or management.
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  • Initial treatment (corticosteroids)

  • Initial treatment (immunosuppressants)

  • Initial treatment (antifibrotic agents)

  • Initial treatment (IVIG/plasma exchange)

  • Management of progressive disease (corticosteroids)

  • Management of progressive disease (immunosuppressants)

  • Management of progressive disease (antifibrotic agents)

  • Management of progressive disease (IVIG/plasma exchange)

  • Management of progressive disease (stem cell and/or lung transplantation)

  • Management of rapidly progressive disease (corticosteroids)

  • Management of rapidly progressive disease (immunosuppressants)

  • Management of rapidly progressive disease (antifibrotic agents)

  • Management of rapidly progressive disease (combination therapy)

  • Management of rapidly progressive disease (IVIG/plasma exchange)

  • Management of rapidly progressive disease (stem cell and/or lung transplantation)

  • Management of acute respiratory failure

  • Management of pulmonary hypertension

  • Management of chronic cough

6.Nonpharmacologic interventions

Smoking cessation: advise smoking cessation in patients with ILD. Offer specialist support and nicotine replacement therapy or bupropion in current smokers.
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  • Pulmonary rehabilitation

7.Follow-up and surveillance

Follow-up: as per ACR 2023 guidelines, consider obtaining pulmonary function testing, HRCT of the chest, or both, and ambulatory desaturation testing for monitoring patients with SARD-associated ILD.
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