Ask AI

Search

Updates

Table of contents

Connective tissue disease-associated interstitial lung disease

What's new

Updated 2024 BSR guidelines for the management ofinterstitial lung disease in systemic sclerosis.

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 ...
Show more

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.
C
Show 2 more
Create free account

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.
B
Create free account

More topics in this section

  • 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.
C
Create free account

More topics in this section

  • 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.
B
Create free account

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.
B
Refer patients with ILD to a regional ILD in case of perceived difficulties in diagnosis and/or management.
B
Create free account

More topics in this section

  • 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.
B
Create free account

More topics in this section

  • 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.
C
Show 4 more
Create free account