Interstitial lung disease in systemic sclerosis: current and future treatment

Giacomelli, Roberto; Liakouli, Vasiliki; Berardicurti, Onorina; Ruscitti, Piero; Benedetto, Paola Di; Carubbi, Francesco; Guggino, Giuliana; Bartolomeo, Salvatore Di; Ciccia, Francesco; Triolo, Giovanni; Cipriani, Paola

doi:10.1007/s00296-016-3636-7

Cited by 78 publications

(64 citation statements)

References 89 publications

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“…SSc-ILD represents a high unmet medical need as no pharmacologic treatment is specifically approved and treatment options are limited [6,7]. Non-selective immunosuppressiontargeting a disease pathophysiology of SSc that involves vascular damage, inflammation, and skin fibrosisis the main treatment used [8][9][10].…”

Section: Introductionmentioning

confidence: 99%

Nintedanib in patients with systemic sclerosis-associated interstitial lung disease: A Japanese population analysis of the SENSCIS trial

Kuwana

Ogura

Makino

et al. 2020

Modern Rheumatology

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Objective: We examined the efficacy and safety of nintedanib in Japanese patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) in the global Safety and Efficacy of Nintedanib in Systemic Sclerosis (SENSCIS) trial. Methods: Randomised patients received oral nintedanib 150 mg (N ¼ 34) twice daily or placebo (N ¼ 36) until the last patient reached 52 weeks of treatment (up to 100 weeks). Data were analysed using a subgroup analysis model with Japanese and non-Japanese patients as subgroup variables. Results: In Japanese patients, the adjusted annual rate of forced vital capacity (FVC) decline over 52 weeks was-86.2 mL/year (nintedanib) and-90.9 mL/year (placebo); treatment difference, 4.67 mL/year (95% confidence interval, À103.28, 112.63). Treatment effect heterogeneity between Japanese and non-Japanese patients was not detected (treatment-by-visit-by-subgroup interaction; p ¼ .49). FVC decline was smaller for nintedanib versus placebo through 100 weeks in Japanese patients. The most commonly reported adverse events with nintedanib were gastrointestinal and liver disorder events; most were mild-to-moderate in severity. Conclusion: In both Japanese and non-Japanese patients with SSc-ILD, nintedanib slowed the progression of ILD, with no heterogeneity detected between the subgroups. The safety profile for nintedanib in Japanese patients was similar to that observed in patients with idiopathic pulmonary fibrosis (ClinicalTrials.gov: NCT02597933).

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Section: Introductionmentioning

confidence: 99%

Nintedanib in patients with systemic sclerosis-associated interstitial lung disease: A Japanese population analysis of the SENSCIS trial

Kuwana

Ogura

Makino

et al. 2020

Modern Rheumatology

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“…Six months after quitting of immunosuppressant, the recovery in FVC fell into baseline, suggesting the requirement of prolonged immunosuppression therapy to maintain stability of lung function [168]. However, it should be noted that, despite studies supporting the benefit of CYC therapy in preventing deterioration in the lung function and premature death in patients with SSc-ILD, recent systematic review and meta-analysis of RCTs and observational prospective cohort studies failed to validate any clinically significant improvement in pulmonary function in SSc patients treated with CYC [169,170].…”

Section: Management Of Interstitial Pneumonia Associated With Connectmentioning

confidence: 99%

Interstitial Pneumonia Associated with Connective Tissue Disease: An Overview and an Insight

Ishii¹

2017

Contemporary Topics of Pneumonia

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Interstitial pneumonia (IP) refers to involvement of the lung parenchyma by varying degrees of inflammation and fibrosis, in contrast to airspace disease typically seen in bacterial pneumonia. IP lies in the center of a heterogenous group of diffuse interstitial lung diseases (ILDs), either idiopathic or linked to underlying disorders. One of the major categories of disorders frequently associated with IP is a connective tissue disease (CTD), in which autoimmunemediated tissue injury leads to multiple organ impairment. Today, IP represents the most critical pulmonary complication in CTD, resulting in significant morbidity and mortality.Despite growing understanding of the pathology of IPs, as well as the accumulating knowledge from both basic and clinical studies of CTDs, the pathogenesis of CTD-associated IP remains unclear. This chapter will provide an overview of the general understanding of ILD and illustrate the current state of knowledge on IP associated with CTD, in order to fully comprehend the entirety of its complex pictures. Moreover, we will propose a new insight into the immune pathogenesis of CTD-IP by presenting evidence which robustly indicates that T cells trigger initial development of IP in polymyositis/dermatomyositis, suggesting potential approaches for controlling such particular T cells in therapeutic interventions for IP.

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“…Several alternative approaches may be considered, including B cell depletion therapies (rituximab), anti-TGF-β antibody, tyrosine kinase inhibitors (imatinib, dasatinib), anti-IL-6 antibody, anti-IL-13 antibody, pirfenidone and haematopoietic stem cell transplantation (HSCT). Finally, lung transplantation may be limited to those patients, with severe SSc-ILD, unresponsive to pharmacologic therapy [64]. It is important to remember that, although often used, during the first stages of treatment, prednisone doses over 15 mg a day can be dangerous in order to trigger a scleroderma renal crisis.…”

Section: Lung Involvementmentioning

confidence: 99%

Survival, Mortality, Causes of Death and Risk Factors of Poor Outcome

Rubio‐Rivas¹

2017

Systemic Sclerosis

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Systemic sclerosis is a rare autoimmune disorder with a historically bad prognosis. Survival has been improving over time and we can currently estimate a 1-year survival, 94.9; a 5-year survival, 84.4; a 10-year survival, 70.9 and a 20-year survival, 44.9%, from the time of diagnosis. Accordingly, mortality has been decreasing over time, being the overall standardized mortality ratio (SMR) 2.72 (1.90-3.83), SMR 2.4 after 1990. Among the SSc-related causes of death, the lung death is the most important cause and its relative percentage is increasing over time since the introduction of ACE inhibitors for the treatment of scleroderma renal crisis (SRC) in early 1990s. Among the SSc-non-related causes of death, cancer, infection and cardiovascular disorders are the leading causes of death. Risk factor predictors of poor outcomes are an elder age at diagnosis, the male gender, diffuse subset, visceral involvement and non-Raynaud's phenomenon onset.

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Interstitial lung disease in systemic sclerosis: current and future treatment

Cited by 78 publications

References 89 publications

Nintedanib in patients with systemic sclerosis-associated interstitial lung disease: A Japanese population analysis of the SENSCIS trial

Nintedanib in patients with systemic sclerosis-associated interstitial lung disease: A Japanese population analysis of the SENSCIS trial

Interstitial Pneumonia Associated with Connective Tissue Disease: An Overview and an Insight

Survival, Mortality, Causes of Death and Risk Factors of Poor Outcome

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