Nintedanib for Systemic Sclerosis–Associated Interstitial Lung Disease

Distler, Oliver; Gahlemann, Martina; Maher, Toby M.

doi:10.1056/nejmoa1903076

Cited by 1,185 publications

(1,062 citation statements)

References 31 publications

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“…An aggressive immunosuppression was not able to revert fibrotic lung damage in IPF or SSc however, remaining associated with the known side effects [33]. These patients can benefit from the use of conventional therapy (controlling systemic DA) associated with anti-fibrotic drugs, as recently reported for Nintedanib in SSc [34].…”

Section: Discussionmentioning

confidence: 84%

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Sambataro

Pignataro³

et al. 2020

Diagnostics

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Interstitial lung disease (ILD) is the main cause of death in systemic sclerosis (SSc) patients. Usually, patients have lung involvement characterized by ground glass opacities (GGOs), but honeycombing (HC) is also possible. The Wells score is a semi-quantitative index, which is able to assess ILD by distinguishing its main components. The aim of this work is to evaluate the Wells score in relation to the disease activity (DA) index. We enrolled 40 consecutive SSc-ILD patients (26 diffuse cutaneous form, dcSSc, and 14 limited form, lcSSc). All patients were evaluated by the European Scleroderma Study Group (ESSG) index, high-resolution computed tomography, transthoracic echocardiogram, pulmonary function tests (PTSs), and nailfold videocapillaroscopy for the number of microhemorrhages (NEMO) score. In our study, the total extent of ILD (TE-ILD), fibrosis and GGOs correlated with dyspnea (p = 0.03, 0.01 and 0.01 respectively), but not with the ESSG index. Considering only the dcSSc patients, TE-ILD and GGOs correlated with the ESSG index (r = 0.5 p = 0.009), while fibrosis grade correlated with disease duration and systolic pulmonary artery pressure. In conclusion, our data suggest that GGO correlates with DA, while fibrosis may be a sign of disease damage. The quantification of pulmonary involvement using the Wells score can be a useful tool for assessing the appropriate treatment in SSc patients.

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Section: Discussionmentioning

confidence: 84%

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Sambataro

Pignataro³

et al. 2020

Diagnostics

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“…70 Announced earlier this year were the results of the much anticipated SENSCIS trial, a randomised placebo-controlled trial, which assessed nintedanib versus placebo in SSc-ILD over 52 weeks. 71 Whilst a modest reduced rate of annual FVC decline (À52 mL year 1 in nintedanib versus À93.3 mL year 1 in placebo, P = 0.04) was reported in the nintedanib group, there was a high proportion of reported adverse events of which diarrhoea was most common (75.7% in nintedanib group and 31% in placebo respectively). Interestingly, almost 50% of patients were receiving mycophenolate prior to enrolment, challenging discussion as to whether the combination of anti-fibrotic and immunosuppressant therapy is of additional benefit in this cohort.…”

Section: Immunosuppressionmentioning

confidence: 86%

“…Announced earlier this year were the results of the much anticipated SENSCIS trial, a randomised placebo‐controlled trial, which assessed nintedanib versus placebo in SSc‐ILD over 52 weeks . Whilst a modest reduced rate of annual FVC decline (−52 mL year 1 in nintedanib versus −93.3 mL year 1 in placebo, P = 0.04) was reported in the nintedanib group, there was a high proportion of reported adverse events of which diarrhoea was most common (75.7% in nintedanib group and 31% in placebo respectively).…”

Section: Treatment Of Ildmentioning

confidence: 99%

Idiopathic and immune‐related pulmonary fibrosis: diagnostic and therapeutic challenges

2019

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Interstitial lung disease (ILD) encompasses a large group of pulmonary conditions sharing common clinical, radiological and histopathological features as a consequence of fibrosis of the lung interstitium. The majority of ILDs are idiopathic in nature with possible genetic predisposition, but is also well recognised as a complication of connective tissue disease or with certain environmental, occupational or drug exposures. In recent years, a concerted international effort has been made to standardise the diagnostic criteria in ILD subtypes, formalise multidisciplinary pathways and standardise treatment recommendations. In this review, we discuss some of the current challenges around ILD diagnostics, the role of serological testing, especially, in light of the new classification of Interstitial Pneumonia with Autoimmune Features (IPAF) and discuss the evidence for therapies targeted at idiopathic and immune‐related pulmonary fibrosis.

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“…As shown in Figure , a clinician may consider anti‐fibrotic therapies in patients with progressive ILD or those intolerant of immuno‐modulatory therapy (eg, due to recurrent infections). The SENSCIS trial, a large Phase 3 trial, demonstrated a smaller decline in FVC with the addition of nintedanib (an intracellular multiple tyrosine kinase inhibitor) to background MMF or no therapy vs placebo . Based on published data, sequential combination therapy could be considered for patients with high risk of progression (not addressed in the SENSCIS trail), where progression has occurred under monotherapy, or contraindication to the immunosuppressive therapy.…”

Section: Treatmentmentioning

confidence: 99%

Management of systemic sclerosis‐associated interstitial lung disease in the current era

Amjadi

Roofeh

Namas³

et al. 2020

Int J of Rheum Dis

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Nintedanib for Systemic Sclerosis–Associated Interstitial Lung Disease

Cited by 1,185 publications

References 31 publications

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Idiopathic and immune‐related pulmonary fibrosis: diagnostic and therapeutic challenges

Management of systemic sclerosis‐associated interstitial lung disease in the current era

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