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Connective tissue disease-associated interstitial lung disease

What's new

Added 2023 ACR, 2023 ATS, 2022 ERS, 2022 BSR, and 2022 JCR guidelines for the diagnosis and management of connective tissue disease-associated interstitial lung disease.

Guidelines

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), the American Thoracic Society (ATS 2023), the British Society for Rheumatology (BSR 2022), the European Respiratory Society (ERS 2022), the European Society of Cardiology (ESC/ERS 2022), the...
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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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Diagnostic investigations

Medical history: as per ALAT/ATS/ERS/JRS 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

Diagnostic procedures

Transbronchial lung biopsy, indications: as per ALAT/ATS/ERS/JRS 2022 guidelines, 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

Respiratory support

Oxygen therapy: as per PDSS-CEP 2021 guidelines, Offer long-term oxygen therapy in patients with Sjögren'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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Medical management

Setting of care
As per BTS/ITS/TSANZ 2008 guidelines:
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

Nonpharmacologic interventions

Smoking cessation: as per BTS/ITS/TSANZ 2008 guidelines, 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

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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