Interstitial lung disease
ICD-10 J84.9 · ICD-11 CB05

Treatment of Interstitial Lung Disease in Systemic Sclerosis (SSc-ILD)

Clinical Scenario

Interstitial lung disease arising in the context of systemic sclerosis (SSc-ILD) represents a distinct clinical challenge. The coexisting autoimmune disease drives both inflammatory and fibrotic processes in the lung, and management must address this combined pathology rather than ILD in isolation.

Treatment Approach

Treatment in SSc-ILD is guided by features such as cutaneous disease extent, inflammatory markers, and the degree of fibrosis visible on HRCT. Both immunosuppressive therapy and antifibrotic agents have a role, with agent selection depending on the individual patient's profile.

Specific agents, combinations, subgroup criteria, and the full sequencing algorithm are detailed in the complete structured protocol…
Clinical Goals
Instant Access to Structured Evidence-Based Regimens

References

  1. We recommend using tocilizumab in a subgroup (S, M) and suggest using MMF, rituximab (C, VL), and cyclophosphamide (C, L) in patients with SSc-ILD.
  2. We suggest using MMF in patients with SSc-ILD (conditional recommendation, very low certainty of evidence).
  3. We recommend using tocilizumab in SSc-ILD patients with early diffuse cutaneous SSc and increased inflammatory markers or recent skin fibrosis progression (strong recommendation, moderate certainty of evidence).
  4. We suggest using nintedanib in patients with SSc-ILD (conditional recommendation, moderate certainty of evidence).
  5. We suggest using MMF in patients with SSc-ILD, as the included studies suggested improvement or stabilisation of FVC % and the extent of ILD on HRCT, improvement of breathlessness, cough and quality of life, and improved skin scores with manageable side-effects.
DOI: 10.1183/13993003.02533-2024 View source ↗