below then 
SCREENING AT-RISK PATIENTS
A suggested algorithm to identify SSc-ILD in patients with SSc
A clinical management algorithm for SSc was defined1,*
Screen all SSc patients for
ILD using HRCT
- FVC and DLCO should be performed at baseline and at regular intervals
- Every patient should receive an ILD-related physical examination
- Order HRCT at baseline to confirm lung fibrosis and periodically to assess progression
HCRT screening results are:
*A study using a robust modified Delphi process was conducted to establish expert consensus statements for the identification and management of SSc-ILD. This study provides the first evidence-based expert consensus statements for SSc-ILD management developed using well-established methods. It is based on a panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD.1
Diagnose ILD and
assess severity
- HRCT is the primary tool for diagnosis; FVC, DLCO, and clinical symptoms are supportive
- Use HRCT, FVC, DLCO, exercise-induced blood oxygen desaturation, clinical symptoms, and quality of life to assess ILD severity
*A study using a robust modified Delphi process was conducted to establish expert consensus statements for the identification and management of SSc-ILD. This study provides the first evidence-based expert consensus statements for SSc-ILD management developed using well-established methods. It is based on a panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD.1
Continue monitoring
for ILD
Frequency of screening and use of HRCT should be guided by risk of ILD, in combination with lung function and symptoms
Restart*A study using a robust modified Delphi process was conducted to establish expert consensus statements for the identification and management of SSc-ILD. This study provides the first evidence-based expert consensus statements for SSc-ILD management developed using well-established methods. It is based on a panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD.1
Decide whether pharmacological therapy is required
- Some patients may not need pharmacological therapy
- Patients not on pharmacological therapy should be monitored
- Factors to consider include disease severity, patient quality of life, and available clinical guidelines
*A study using a robust modified Delphi process was conducted to establish expert consensus statements for the identification and management of SSc-ILD. This study provides the first evidence-based expert consensus statements for SSc-ILD management developed using well-established methods. It is based on a panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD.1
Follow up closely and
continue to evaluate for
initiation of pharmacological
therapy if indicated
Restart*A study using a robust modified Delphi process was conducted to establish expert consensus statements for the identification and management of SSc-ILD. This study provides the first evidence-based expert consensus statements for SSc-ILD management developed using well-established methods. It is based on a panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD.1
Assess ILD progression using multiple methods
- Use HRCT (depending on clinical need), FVC, DLCO, exercise-induced blood oxygen desaturation, and clinical symptoms to assess ILD progression
*A study using a robust modified Delphi process was conducted to establish expert consensus statements for the identification and management of SSc-ILD. This study provides the first evidence-based expert consensus statements for SSc-ILD management developed using well-established methods. It is based on a panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD.1
Is patient showing inadequate treatment response/disease progression?
*A study using a robust modified Delphi process was conducted to establish expert consensus statements for the identification and management of SSc-ILD. This study provides the first evidence-based expert consensus statements for SSc-ILD management developed using well-established methods. It is based on a panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD.1
Reassess therapy to:
- Evaluate pharmacological treatment
- Evaluate for lung transplant
- Consider autologous hematopoietic stem cell transplant for selected patients
*A study using a robust modified Delphi process was conducted to establish expert consensus statements for the identification and management of SSc-ILD. This study provides the first evidence-based expert consensus statements for SSc-ILD management developed using well-established methods. It is based on a panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD.1
*A study using a robust modified Delphi process was conducted to establish expert consensus statements for the identification and management of SSc-ILD. This study provides the first evidence-based expert consensus statements for SSc-ILD management developed using well-established methods. It is based on a panel of 27 Europe-based pulmonologists, rheumatologists, and internists with expertise in SSc-ILD.1
Reference:
1. Hoffman-Vold et al. Rheumatology. 2020;2(2):E71-E83.