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Systemic sclerosis-associated interstitial lung disease with progressive pulmonary fibrosis — SCE Rheumatology

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ModerateInterstitial Lung DiseaseSystemic sclerosis-associated interstitial lung disease with progressive pulmonary fibrosisSCE Rheumatology

A 58-year-old woman with diffuse cutaneous systemic sclerosis and fibrotic non-specific interstitial pneumonia has received mycophenolate mofetil 1.5 g twice daily for 20 months. Treatment is well tolerated and adherence is confirmed. During the last 9 months, her exertional breathlessness has increased. Forced vital capacity has fallen from 82% to 73% predicted and TLCO from 58% to 51% predicted. High-resolution CT demonstrates increased basal reticulation and traction bronchiectasis, without substantial new ground-glass opacity. Her modified Rodnan skin score is unchanged, C-reactive protein is normal, and she has no active inflammatory arthritis or myositis. Infection, anaemia, pulmonary hypertension and clinically important aspiration have been excluded. The interstitial lung disease multidisciplinary team confirms progression of her fibrotic lung disease. Which is the most appropriate next disease-modifying treatment plan?

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Correct answer: D — Continue mycophenolate and add nintedanib

Explanation lettering: D = shown as B · E = shown as C · C = shown as D · B = shown as E

The FVC has fallen by 9 percentage points but by approximately 11% relative to baseline, exceeding the 10% relative-decline criterion accepted by NICE as evidence of progressive fibrosing ILD. This is corroborated by worsening breathlessness and radiological extension of fibrosis despite adequate mycophenolate treatment. The 2024 BSR systemic sclerosis guideline recommends mycophenolate as first-line therapy for SSc-ILD and nintedanib for a progressive pulmonary fibrosis phenotype. NICE recommends nintedanib for chronic progressive fibrosing ILD and positions it as an add-on after progression despite conventional treatment; treatment controlling the underlying autoimmune disease may be continued. The SENSCIS subgroup analysis found no evidence that concomitant mycophenolate abolished the antifibrotic effect of nintedanib. A delays treatment despite established multidomain progression. B is plausible because rituximab is an immunomodulatory option for progressive SSc-ILD, but the predominantly fibrotic progression and absence of active systemic inflammation favour antifibrotic escalation. D is better supported in early inflammatory diffuse disease, particularly with progressive skin involvement or raised acute-phase reactants. E is an alternative immunosuppressive strategy when mycophenolate is unsuitable or when severe inflammatory disease requires induction; replacing a tolerated immunosuppressive backbone does not specifically address this progressive fibrotic phenotype.

Reference: The 2024 British Society for Rheumatology guideline for management of systemic sclerosis (1 November 2024) — https://pubmed.ncbi.nlm.nih.gov/39255973/ Nintedanib for treating progressive fibrosing interstitial lung diseases: Recommendations (17 November 2021) — https://www.nice.org.uk/guidance/ta747/chapter/1-Recommendations Nintedanib for treating progressive fibrosing interstitial lung diseases: Committee discussion (17 November 2021) — https://www.nice.org.uk/guidance/TA747/chapter/3-committee-discussion