An updated safety review of the drug treatments for idiopathic pulmonary fibrosis

Sgalla, Giacomo; Comes, Alessia; Richeldi, Luca

doi:10.1080/14740338.2021.1921143

Cited by 9 publications

(3 citation statements)

References 77 publications

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“…To date, even if presently collected data confirm the tolerability profile of the two antifibrotic drugs, many issues remain unsolved. In fact, drug discontinuation still occurs in a considerable proportion of patients, and first line therapy for the individual patients must be optimised [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

Long-Term Safety of Antifibrotic Drugs in IPF: A Real-World Experience

et al. 2022

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Pirfenidone and nintedanib are the only two drugs approved for the treatment of idiopathic pulmonary fibrosis (IPF). Both proved to be safe and well-tolerated in clinical trials, but real-world data and direct comparisons are scarce. This real-life study explored the safety profile of pirfenidone and nintedanib with a prolonged follow-up. We retrospectively collected clinical status, adverse events (AEs), and treatment changes from IPF patients who had started an antifibrotic treatment at our centre from December 2011 to December 2020, including 192 patients treated with pirfenidone and 89 with nintedanib. The majority of patients in both groups experienced one or more AEs during the follow-up. A higher proportion of AEs in the nintedanib group were effectively treated with behavioural modifications or additional medications compared with the pirfenidone group (52.5% vs. 40.6%, p = 0.04). Overall, a difference in the impact of AEs due to nintedanib versus pirfenidone resulted in a lower permanent discontinuation of therapy (8.3% vs. 18.3%, p = 0.02), with the latter being associated with a higher risk of drug discontinuation at 48 months after initiation (OR =2.52, p = 0.03). Our study confirms the safety profile of antifibrotic drugs in IPF but highlights that AEs due to nintedanib are usually easier to manage and lead to fewer cases of permanent discontinuation of therapy.

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Section: Introductionmentioning

confidence: 99%

Long-Term Safety of Antifibrotic Drugs in IPF: A Real-World Experience

et al. 2022

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“…Interestingly, although limited by the low number of patients, we observed that c-ANCA A number of these patients are initially classified as IPF, and therefore they need a careful assessment by the pulmonologist of clinical manifestations and laboratory findings during their followup for an early and appropriate management and treatment of vasculitis. In addition, this subgroup of patients has a poor prognosis more related to ILD than to vasculitis, and, in analogy to IPF, anti-fibrotic agents might be useful (8,39,40). This clinical subgroup supports the biological hypothesis of a fibrosing effect of ANCA-MPO (41).…”

Section: Discussionmentioning

confidence: 99%

Interstitial lung disease in microscopic polyangiitis and granulomatosis with polyangiitis: demographic, clinical, serological and radiological features of an Italian cohort from the Italian Society for Rheumatology

Manfredi

Cassone

Izzo

et al. 2022

Clinical and Experimental Rheumatology

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ObjectivesInterstitial lung disease (ILD) has been described as a possible pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV), mainly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Aim of this cross-sectional Italian national study was to describe demographic, clinical and serological profile of ILD related to MPA and GPA and investigate possible correlations between radiologic patterns of ILD and vasculitis features. MethodsWe enrolled 95 consecutive patients with AAV-ILD, 56 affected by MPA (58.9%) and 39 by GPA (41.1%). Results NSIP was the most frequently detected ILD pattern, observed in c-ANCA patients in 60.9% of cases, followed by UIP pattern mainly observed in p-ANCA patients (47.7%, p=0.03). ILD represented the first clinical manifestation, preceding vasculitis diagnosis in 22.1% of cases and, globally, ILD was already detectable at AAV diagnosis in 66.3% of patients. The diagnosis of ILD preceded that of AAV in 85.7% of p-ANCA positive-patients, while only one patient with c-ANCA developed ILD before AAV (p= 0.039). Multivariate analysis confirmed the correlation of UIP pattern with p-ANCA-positivity and a diagnosis of ILD before AAV, also whenadjusted for age and sex. ConclusionsOur study confirms that UIP is a frequent pattern of lung disease in AAVILD patients. Our results also suggest that ILD can represent an early complication of AAV but also occur in the course of the disease, suggesting the need of a careful evaluation by both pulmonologist and rheumatologist to achieve an early diagnosis. Further prospective studies are needed to define ILD prevalence and evolution in AAV patients.

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“…Activation of these pathways is associated with growth stimulation and expansion of the mesenchymal compartment as well as diverse aspects of the immune response 20 . While IPF has been refractory to therapy, nintedanib (NIN; BIBF 1120) and pirfenidone have been found to achieve control of disease progression 25 , suggesting a similar approach may apply to CD. These novel, multi-modal agents result in disease control, with reduced rate of decline in forced vital capacity, fewer acute exacerbations, and increased health-related quality of life 24 .…”

Section: Introductionmentioning

confidence: 99%

Nintedanib regulates intestinal smooth muscle hyperplasia and phenotype in vitro and in TNBS colitis in vivo

Kataria

Kerr

Lourenssen

et al. 2022

Sci Rep

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Chronic inflammation of the human intestine in Crohn’s disease (CD) causes bowel wall thickening, which typically progresses to stricturing and a recurrent need for surgery. Current therapies have limited success and CD remains idiopathic and incurable. Recent evidence shows a key role of intestinal smooth muscle cell (ISMC) hyperplasia in stricturing, which is not targeted by current anti-inflammatory therapeutics. However, progression of idiopathic pulmonary fibrosis, resembling CD in pathophysiology, is controlled by the tyrosine kinase inhibitors nintedanib (NIN) or pirfenidone, and we investigated these drugs for their effect on ISMC. In a culture model of rat ISMC, NIN inhibited serum- and PDGF-BB-stimulated growth and cell migration, and promoted the differentiated phenotype, while increasing secreted collagen. NIN did not affect signaling through PDGF-Rβ or NFκB but did inhibit cytokine-induced expression of the pro-inflammatory cytokines IL-1β and TNFα, supporting a transcriptional level of control. In TNBS-induced colitis in mice, which resembles CD, NIN decreased ISMC hyperplasia as well as expression of TNFα and IL-1β, without effect in control animals. NIN also inhibited growth of human ISMC in response to human serum or PDGF-BB, which further establishes a broad range of actions of NIN that support further trial in human IBD.

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An updated safety review of the drug treatments for idiopathic pulmonary fibrosis

Cited by 9 publications

References 77 publications

Long-Term Safety of Antifibrotic Drugs in IPF: A Real-World Experience

Long-Term Safety of Antifibrotic Drugs in IPF: A Real-World Experience

Interstitial lung disease in microscopic polyangiitis and granulomatosis with polyangiitis: demographic, clinical, serological and radiological features of an Italian cohort from the Italian Society for Rheumatology

Nintedanib regulates intestinal smooth muscle hyperplasia and phenotype in vitro and in TNBS colitis in vivo

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