Nintedanib and intensive immunosuppressive therapy to treat rapidly progressive interstitial lung disease presenting anti-ARS antibodies

Yanagihara, Toyoshi; Suzuki, Kunihiro; Egashira, Ayaka; Ogo, Naruhiko; Asoh, Tatsuma; Nara, Tsukasa; Takatsuki, Kensaku; Yoshizawa, Seiji; Chong, Sy Giin; Hamada, Naoki; Maeyama, Takashige

doi:10.1016/j.rmcr.2020.101272

Cited by 2 publications

(1 citation statement)

References 17 publications

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“…First, these patients experienced more RPILD (40%) and tended to present fever (84%) within 3 months from disease onset, providing important clues for early recognition of the more inflammatory phenotype. Indeed, RPILD has been reported in 7.8–29.2% of ASyS patients ( 14 ). The link between recurrent fever attacks and RPILD suggests that RPILD may be a component of profound systemic inflammation.…”

Section: Discussionmentioning

confidence: 99%

Description and Analysis of a Novel Subtype of the Anti-Synthetase Syndrome Characterized by Frequent Attacks of Fever and Systemic Inflammation in a Single-Center Cohort Study

et al. 2021

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ObjectivesThe aim of this study was to investigate anti-synthetase syndrome (ASyS) patients who presented with recurrent episodes of fever and systemic inflammation.MethodsA retrospective cohort of Chinese ASyS patients (n=126) in our center (between January 2013 and January 2020) was included. Patients presenting with concomitant autoimmune rheumatic diseases or malignancies were subsequently excluded. The number of non-infectious fever attacks and attack frequency were recorded and calculated. Patients with two or more attacks and within the upper three quartiles of attack frequency were defined as high-inflammation group. Univariate and multivariate analyses were carried out to characterize the high-inflammation subtype.ResultsOut of 113 eligible patients with an average of 5 years follow up, 25 patients were defined as the high-inflammation group (16 for anti-Jo1, 9 for anti-PL7), with an average of 1.12 attack/patient-year. Compared to low-inflammation group (0–1 attack only and a frequency lower than 0.5 attack/patient-year), the high-inflammation group had higher occurrence of fever and rapid progressive interstitial lung disease (RPILD) as the first presentation (84% vs. 21% and 40% vs. 9%, respectively, both p<0.01). Anti-PL-7 was related to the more inflammatory phenotype (p=0.014). Cumulative disease-modifying agent exposures (>=3) were much higher in the high-inflammation group (60% vs. 26%), while biological agents, i.e., rituximab and tocilizumab, showed better “drug survival” for Jo-1+ and PL-7+ ASyS patients with high inflammation, respectively, in our cohort.ConclusionsASyS with recurrent systemic inflammatory episodes reflects a subtype of more aggressive and refractory disease in the spectrum of ASyS. Increased awareness of this subtype might lead to more appropriate management.

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Section: Discussionmentioning

confidence: 99%

Description and Analysis of a Novel Subtype of the Anti-Synthetase Syndrome Characterized by Frequent Attacks of Fever and Systemic Inflammation in a Single-Center Cohort Study

et al. 2021

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Antifibrotic Drugs for COVID-19: From Orphan Drugs to Blockbusters?

Corrie

Muzaffar-Ur-Rehman²,

Kukatil

et al. 2021

CRMR

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: Antifibrotic agents are known to treat idiopathic pulmonary fibrosis. The two antifibrotic agents approved and in usage are Pirfenidone and Nintedanib granted by the USFDA in 2014. They are both known to decrease inflammation in the lungs. The fact that COVID-19 has shown to cause inflammation and fibrosis in the lungs frames the theory of their usage in the treatment of the disease by reducing lung scaring and allowing faster discharge of patients with post-COVID complications. The need for them to change their status from orphans to blockbusters has not happened yet due to fewer data and less research available on them as well as various other economic and patient-related factors. Since COVID-19 is widespread and causes many complications of the lungs that are similar to what these two drugs treat. We believe that the status of these drugs could be changed due to an increase in demand for them.

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Nintedanib and intensive immunosuppressive therapy to treat rapidly progressive interstitial lung disease presenting anti-ARS antibodies

Cited by 2 publications

References 17 publications

Description and Analysis of a Novel Subtype of the Anti-Synthetase Syndrome Characterized by Frequent Attacks of Fever and Systemic Inflammation in a Single-Center Cohort Study

Description and Analysis of a Novel Subtype of the Anti-Synthetase Syndrome Characterized by Frequent Attacks of Fever and Systemic Inflammation in a Single-Center Cohort Study

Antifibrotic Drugs for COVID-19: From Orphan Drugs to Blockbusters?

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