#Interféronopathies
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thelancet.com
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Watch our webinar to discover the importance of innate immunity in the development of neurological diseases, including interferonopathies, multiple sclerosis, and neurodegenerative diseases.

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#neurology #brainhealth
Blue graphic with pink and blue arrows pointing to text saying: "Watch our joint webinar: Innate immunity regulation of brain health". The Cell Press and The Lancet logos appear in the top left and right corners respectively. Copyright: Elsevier Ltd.
December 1, 2025 at 10:52 AM
iic-umu-imib.bsky.social
🔬🐟 Today we celebrate science at UMU!
Beatriz Bernal Bermúdez will defend her PhD on zebrafish modeling of rare genetic diseases, focusing on NEXMIF-related encephalopathy and IFIH1-mediated type I interferonopathies.
November 21, 2025 at 8:44 AM
ansharp.bsky.social
Fighting sleep, trying to learn everything I can about interferonopathies. 😵‍💫
November 6, 2025 at 4:10 AM
dlingenfelter.bsky.social
“COVID toes” result from a strong local interferon response to SARS-CoV-2, causing vascular inflammation and microthrombosis.

This immune reaction clears the virus systemically but damages skin capillaries.
davidlingenfelter.substack.com/p/the-pathop...
🧬 Scientific Announcement: Unraveling the Pathophysiology of Pernio-Like Lesions in COVID-19 To the scientific and medical research community: We are pleased to share a comprehensive synthesis of the pathophysiological mechanisms underlying pernio-like lesions—commonly referred to as “COVID toes”—as observed during the SARS-CoV-2 pandemic. This newly published analysis by Dr. David Lingenfelter offers a unified model integrating virological, immunological, and thrombotic pathways to explain this paradoxical dermatological manifestation. 🔍 Key Findings: Primary Driver Identified: A robust, localized type I interferon (IFN-I) response is the central mechanism. This immune reaction is triggered by sparse viral components—often undetectable by standard PCR or serology—within acral microvasculature. Collateral Damage Mechanism: While systemically protective, the IFN-I response induces localized endotheliitis and lymphocytic vasculitis, creating a prothrombotic environment that leads to microvascular occlusion and ischemia. Histopathological Parallels: The lesions mirror those seen in autoimmune conditions like chilblain lupus, suggesting a shared final pathway of immune-mediated vascular injury. Clinical Paradox Resolved: The presence of “COVID toes” in otherwise asymptomatic or mildly symptomatic individuals with negative viral tests is reframed as a visible marker of a successful antiviral immune response—not a failure of viral detection. Implications for Long COVID: A subset of patients experience persistent or recurrent lesions, indicating potential chronic immune activation or vascular remodeling. 🧪 Research Implications: This model challenges earlier assumptions of direct viral cytotoxicity and positions “COVID toes” as a transient, acquired interferonopathy. It underscores the importance of host immune phenotype and genetic predisposition in shaping clinical outcomes of SARS-CoV-2 infection. 📣 Call to Action: We invite immunologists, dermatopathologists, and virologists to explore the implications of this interferon-driven vasculopathy. Further research is needed to: Identify genetic markers of susceptibility Investigate therapeutic modulation of IFN-I pathways Clarify the role of other viral or environmental triggers in similar acral lesions For full details, refer to the original publication by Dr. Lingenfelter on Substack. Here's a detailed summary of the main points from the article titled "The Pathophysiology of Pernio-Like Lesions in COVID-19" by David Lingenfelter, PhD: 🦶 What Are “COVID Toes”? “COVID toes” are pernio-like lesions (chilblains) that appeared during the pandemic, mostly in healthy children and young adults. They manifest as red or purple discoloration, swelling, and sometimes blisters or ulcers on toes and fingers. Most patients had mild or no COVID symptoms and tested negative for the virus. 🧬 Unified Pathophysiological Model The article proposes a three-part mechanism for how these lesions develop: 1. Type I Interferon (IFN-I) Response The central driver is a strong, localized IFN-I immune response triggered by sparse viral components. This response clears the virus systemically but causes local inflammation and vascular damage. It resembles genetic interferonopathies like Familial Chilblain Lupus. 2. Endothelial Damage & Vasculitis IFN-I activates endothelial cells, leading to swelling and dysfunction. Dense lymphocytic infiltration causes vasculitis and endotheliitis. This inflammation creates a prothrombotic environment. 3. Microthrombosis Small blood clots form in skin capillaries due to inflammation and complement activation. These clots cause localized ischemia, resulting in the visible lesions. 🔬 Histopathological Findings Skin biopsies show lymphocytic vasculitis, perieccrine inflammation, and endothelial swelling. These features mirror those seen in autoimmune conditions like chilblain lupus. 🧪 Virological Evidence ACE2 receptors (used by SARS-CoV-2) are present in skin structures. Some studies found viral proteins in skin, but most failed to detect viral RNA. A theory suggests cleaved spike proteins—not full virus—may trigger immune responses. 🧠 Clinical Paradox Explained Despite negative tests, the lesions may indicate a successful immune response that cleared the virus before systemic symptoms developed. “COVID toes” are not signs of viral failure but of immune success with localized collateral damage. 🔍 Future Research Directions Investigate genetic predispositions (e.g., TLR7, TREX1 variants). Understand persistent or recurrent cases (“long COVID toe”). Explore other triggers beyond SARS-CoV-2. This synthesis reframes “COVID toes” as a visible marker of a robust antiviral defense, rather than a direct viral injury. It’s a fascinating example of how the immune system’s success can paradoxically cause localized pathology.
September 9, 2025 at 11:26 PM
adin.lustre.us
This all supports that a good portion of us have ‘mild’ Type I interferonopathies
September 5, 2025 at 8:42 AM
labwaggoner.bsky.social
STING-driven ZBP1 necroptosis is central pathogenic mechanism in STING-Associated Vasculopathy with onset in Infancy (SAVI) @nature.com
ZBP1-RIPK3-MLKL axis is therapeutic target in interferonopathies w/ excessive necroptosis
www.nature.com/articles/s41...
August 20, 2025 at 5:35 PM
revmedinterne.bsky.social
French protocol for diagnosis and management of type 1 interferonopathies

www.sciencedirect.com/science/arti...
June 22, 2025 at 11:33 AM
adin.lustre.us
academic.oup.com/intimm/artic...

There may be a ton of different roads that lead to dysfunction, but I think a significant fraction of the #ME/CFS & #LC patient populations are dealing with *treatable* interferonopathies!
Correlation of interferons and autoimmune aspects in long COVID-19 patients
Potential roles for interferons in long COVID
academic.oup.com
June 8, 2025 at 7:32 AM
alexis-verger.cpesr.fr
Very exciting work, congrats to all !

An intriguing regulation mechanism of kinase activity by one of its substrates in relation to #LLPS and interferonopathies
damien-glon.bsky.social Damien Glon @damien-glon.bsky.social · May 28
🚨Proud to share the preprint from my postdoc in Yves Gaudin’s lab, in collab with Frédéric Rieux-Laucat’s lab.
🧠Our key finding?
 NAP1 plays a dual role in regulating innate immunity.
📌This work highlights how phase separation modulates antiviral responses and explains some interferonopathies.
🔗⬇️
NAP1 switches from an activator to a limiter of interferon induction by trapping TBK1 in condensates
TBK1 kinase is a central regulator of type I IFN production. Upon activation of the IFN-β induction pathway, TBK1-adaptor proteins (NAP1, SINTBAD, TANK) form condensates with liquid properties. We sho...
www.biorxiv.org
June 6, 2025 at 8:56 AM
castltrastondrs.bsky.social
#Medsky🧪 #IDsky #immunosky #publichealth #Interferonopathies drive #autoimmunity but can also impair host responses to pathogens, including #viralinfections. Human gain of function (GOF) mutations in STAT1—a critical signaling molecule downstream of interferons (IFNs)
June 2, 2025 at 8:00 PM
damien-glon.bsky.social
🚨Proud to share the preprint from my postdoc in Yves Gaudin’s lab, in collab with Frédéric Rieux-Laucat’s lab.
🧠Our key finding?
 NAP1 plays a dual role in regulating innate immunity.
📌This work highlights how phase separation modulates antiviral responses and explains some interferonopathies.
🔗⬇️
NAP1 switches from an activator to a limiter of interferon induction by trapping TBK1 in condensates
TBK1 kinase is a central regulator of type I IFN production. Upon activation of the IFN-β induction pathway, TBK1-adaptor proteins (NAP1, SINTBAD, TANK) form condensates with liquid properties. We sho...
www.biorxiv.org
May 28, 2025 at 6:17 AM
castltrastondrs.bsky.social
Excessive type I IFN, perhaps counterintuitively, appears to underlie a greater number of #autoinflammatory and/or autoimmune conditions known as type I #interferonopathies, whose study has revealed multiple molecular programs involved in the induction of type I IFN signaling.
May 2, 2025 at 5:33 AM
filiere-fai2r.bsky.social
📣 Nouveau registre Européen !

FAI²R, en collaboration avec le CRMR RAISE, a le plaisir d’annoncer le lancement officiel du registre européen EU-IFNp, dédié aux interféronopathies génétiques de type 1.

Rejoignez le registre en remplissant ce formulaire : urls.fr/oKTWdH

📩 +d'infos : urls.fr/oneLY-
April 30, 2025 at 2:03 PM
ahmad-yatim.bsky.social
3/ Chilblains can occur secondary to chronic type I interferon (I-IFN)-driven conditions such as monogenic interferonopathies and #lupus, or primary, when striking otherwise healthy individuals
April 18, 2025 at 9:54 PM
castltrastondrs.bsky.social
#Medsky🧪 #IDsky #immunosky #Publichealth understanding of #covidtoes Outbreaks of #chilblains were reported during the #COVID19pandemic. Given the essential role of type I-IFN in protective immunity against #SARSCoV2 & the association of chilblains with inherited type I interferonopathies…
jem.org Journal of Experimental Medicine @jem.org · Apr 14
Chilblains were reported in #SARSCoV2–exposed individuals without PCR- or serologically defined #COVID19. Saidoune, Gilliet , @ahmad-yatim.bsky.social et al. show that excessive TLR7 responses to SARS-CoV-2 by pDCs are linked to I-IFN–driven chilblain development. rupress.org/jem/article/...
April 14, 2025 at 4:36 PM
angelopilotti.bsky.social
🔍My work is investigating the Integrated Stress Response (ISR) in dendritic cells and interferonopathies.
The ISR is a cellular pathway activated in response to stress, reducing global protein synthesis and promoting cell survival. (2/7)
March 13, 2025 at 2:38 PM
angelopilotti.bsky.social
🔬 Exploring the role of the Integrated Stress Response in dendritic cells and interferonopathies 🔬

📊Last week, I had the opportunity of presenting my research project during the PhD workshop at @ciml.bsky.social. (1/7)
March 13, 2025 at 2:38 PM
angelopilotti.bsky.social
Honored to be working on the Characterization of rare gene variants affecting the Integrated Stress Response in dendritic cells and interferonopathies at @ciml.bsky.social in P. Pierre’s lab.
And grateful to be part of the @immerge.bsky.social program!

#ISR #IntegratedStressResponse #SAVI #SLE
immerge.bsky.social IMMERGE MSCA DN @immerge.bsky.social · Mar 4
🔬 Meet the Researchers Behind IMMERGE! 🔬

This week: @angelopilotti.bsky.social & Dr. Pierre from @cnrs.fr!
Angelo will study the functional impact of EIF2AK mutants in dendritic cells & interferonopathies🧬👨‍🔬

Stay tuned for more IMMERGE scientists! 🚀

#IMMERGE #MSCA #IEI #DCs #Interferonopathies
March 4, 2025 at 8:23 PM
achillebro.bsky.social
just this morning @angelopilotti.bsky.social presented his poster at the @ciml.bsky.social #PhD workshop! Super interesting project linking stress responses with interferonopathies! very curious to follow up this project!
immerge.bsky.social IMMERGE MSCA DN @immerge.bsky.social · Mar 4
🔬 Meet the Researchers Behind IMMERGE! 🔬

This week: @angelopilotti.bsky.social & Dr. Pierre from @cnrs.fr!
Angelo will study the functional impact of EIF2AK mutants in dendritic cells & interferonopathies🧬👨‍🔬

Stay tuned for more IMMERGE scientists! 🚀

#IMMERGE #MSCA #IEI #DCs #Interferonopathies
March 4, 2025 at 10:02 AM
immerge.bsky.social
🔬 Meet the Researchers Behind IMMERGE! 🔬

This week: @angelopilotti.bsky.social & Dr. Pierre from @cnrs.fr!
Angelo will study the functional impact of EIF2AK mutants in dendritic cells & interferonopathies🧬👨‍🔬

Stay tuned for more IMMERGE scientists! 🚀

#IMMERGE #MSCA #IEI #DCs #Interferonopathies
March 4, 2025 at 7:58 AM
bioconf.bsky.social
Upcoming seminar by Jean-Philippe Herbeuval: Control of type I interferon production by CXCR4 minor pocket agonists (MiPAs): from HIV sexual transmission to novel therapeutic strategies for interferonopathies at Institut Necker Enfants Malades, Friday 14 February 2025 at 12:00. More info
January 22, 2025 at 12:42 AM
respifil.bsky.social
📰 La newsletter de janvier est sortie ! 🎉 Découvrez ses temps forts :
🫁 Pr V. Cottin prendra la coordination médicale de #RespiFIL.
🙌 à nos 2 chargées de mission
🫶🏻 prolongation du partenariat avec l’Alliance Sociale 🤝
🆕 protocole sur les #interféronopathies et bien plus encore ! 🔗 shorturl.at/W6MQx
January 21, 2025 at 3:01 PM
natrevrheum.nature.com
#JanuaryIssue | Emerging concepts and treatments in autoinflammatory interferonopathies and monogenic systemic lupus erythematosus

Goldbach-Mansky and colleagues propose a pathogenesis-based classification of #autoinflammatory interferonopathies in this #Review

bit.ly/4gLCpOx
Emerging concepts and treatments in autoinflammatory interferonopathies and monogenic systemic lupus erythematosus - Nature Reviews Rheumatology
This Review provides a comprehensive update on dysregulated type I interferon production and signalling in autoinflammatory interferonopathies, monogenic systemic lupus erythematosus and conditio...
bit.ly
December 20, 2024 at 9:22 AM
natrevrheum.nature.com
Our #JanuaryIssue is out now! Featuring Reviews on the ‘Treg paradox’ in #inflammatory #arthritis, classification of the #autoinflammatory interferonopathies and an update on #autoantibodies in the idiopathic inflammatory myopathies.

bit.ly/41LgnqU
Red and blue swirls of T cells with details about the content of the January Issue
December 20, 2024 at 9:15 AM
ndimmuno.bsky.social
Interesting read. I wonder if we see more Mtb infections or more severe/disseminated infections in patients with monogenic interferonopathies? Esp. from Mtb endemic regions? #autoinflammation #IDsky #immunosky
rodrahimi.bsky.social Rod Rahimi @rodrahimi.bsky.social · Dec 16
Interesting review by Russell Vance

"The major difference, for which we lack a satisfying explanation, is that innate immunity is able to sterilize a dose of 10^6 Legionella within a few days, whereas innate immunity fails to eliminate even a single bacillus of Mtb" #ImmunoSky
Tuberculosis as an unconventional interferonopathy
Tuberculosis is caused by Mycobacterium tuberculosis, a bacterium that accounts for more human mortality than any other. Evidence is accumulating for …
www.sciencedirect.com
December 18, 2024 at 1:31 PM