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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/ACCP 2024), the British Society for Rheumatology (BSR 2024,2022), the American Thoracic Society (ATS 2023), 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 ACCP/ACR 2024 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

  • Bronchoalveolar lavage

Respiratory support

Oxygen therapy: as per BSR 2024 guidelines, offer supportive long-term oxygen and/or ambulatory oxygen therapy for severe exertional hypoxemia.
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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 (setting of care)

  • Management of pulmonary hypertension (general principles)

  • Management of pulmonary hypertension (pharmacotherapy)

  • Management of pulmonary hypertension (anticoagulant therapy)

  • Management of pulmonary hypertension (supportive care)

  • 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 ACCP/ACR 2024 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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