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FRENVEX, the French VEXAS syndrome study group

VEXAS syndrome was individualised at the end of 2020. French cases began to be brought together in November of the same year, and the collection that came of it signs its publications under the name FRENVEX. That name appears in article titles and in the collective author field of MEDLINE records. It points to no website, to no declared status and to no published definition.

Published on 14 September 2026 · The network · about 13 minutes to read

In brief
  • FRENVEX designates the French multicentre collection of VEXAS syndrome cases. The publications call it sometimes a study group, sometimes a registry, and these two words do not describe the same object.
  • The reported inclusions begin in November 2020, a few weeks after the first description of the disease.
  • Sixteen original articles and one correction carry, as of 13 September 2026, either the acronym in their title or the name of the group in their collective author field.
  • Successive analyses have covered growing numbers of patients, from one hundred and sixteen in 2022 to two hundred and ninety-one in 2023, which do not add up and which do not give the size of the collection.
  • No public source gives the expansion of the acronym, the date the group was formed, its legal status, its governance or its current size.

What the name designates, and what it does not say

The acronym circulates in several forms, all of them found in article titles or in the collective author field of MEDLINE records. Three English designations coexist: French VEXAS study group, French VEXAS registry and FRENVEX group. A fourth appears once, National French VEXAS Study Group (NFVS), in the record of the 2023 dermatological study.

Only one sentence published in the body of a peer-reviewed article ties the acronym to a name in plain words. It appears in the abstract of the azacitidine study published in Blood in 2025: the study covers 88 patients "from FRENVEX (French VEXAS study group)". That is the gloss on which this page relies.

No source consulted spells out the acronym letter by letter. The spontaneous reading, which sees in it a contraction of FRENch and VEXas, is a reader's reconstruction. It is written nowhere and this page does not present it as the meaning of the name.

The coexistence of "study group" and "registry" is not a matter of style. A study group brings clinicians together around a question. A registry is a data collection system, with inclusion criteria, a data controller and regulatory formalities. The publications use both words for the same object, without ever separating the two functions. In French, the only expanded name that appears in an institutional source is "groupe français d'étude du syndrome VEXAS", used by the teaching staff profile of Sorbonne Université Formation Continue.

When the network was formed, and why

The date on which the group was formed is given by no primary source. What is established is the date on which inclusions begin: November 2020. Three independent publications carry it. The 2022 dermatological series writes that one hundred and sixteen patients were referred to a French multicentre registry between November 2020 and May 2021. The 2023 pulmonary study dates the same window in the same way. The 2025 neurological study writes that the collection held two hundred and ninety-one patients for the period from November 2020 to March 2023.

VEXAS syndrome had been described in 2020 through the identification of somatic variants of the UBA1 gene in adult men with severe inflammatory disease. The French inclusions therefore begin within the weeks following the original publication.

The reason for a national collection lies in the way these patients reach hospital. The disease combines multi-organ inflammatory manifestations with haematological involvement. A patient comes into internal medicine for unexplained fever, into rheumatology for chondritis, into dermatology for a rash, into haematology for macrocytic anaemia, into nephrology for renal failure. None of these entry points spontaneously sees the whole picture. The FAI²R rare disease network estimates, on its page devoted to VEXAS, a summary signed by Prof. Sophie Georgin-Lavialle and dated September 2024, the number of patients identified in France at about three hundred, and the frequency of the disease at one man in four thousand over the age of fifty. On that scale, a single centre taken alone does not gather enough cases to describe the disease, and no randomised controlled trial had been published in VEXAS syndrome at the date of the American College of Rheumatology guidance document.

How the trace of the network reads in the publications

The network having no website, its trace lies in the signature of its articles. It takes two forms, both of them verifiable on PubMed.

The first is the title. Three articles carry the acronym in their title: the 2024 study of targeted therapies, the 2025 study on erythroid-stimulating agents and luspatercept, and the 2025 azacitidine study. Two others carry the wording French VEXAS registry.

The second is the collective author field of the MEDLINE record, the CN field. It carries the name of the group, under one or another of its designations, for fifteen of the sixteen original articles listed here; the 2025 azacitidine study is the exception, carrying the acronym in its title and its abstract but no CN field.

Two points of method, which govern the reading of the author positions given below. Positions are counted on the FAU fields of the complete MEDLINE record alone, that is, on the named authors. The CN, FIR and IR fields, which carry the name of the group and the list of its collaborators, never enter the count. And the fact that a record comes up in a search does not prove that the study rests on the French collection: the record of the study on lysozyme nephropathy in chronic myelomonocytic leukaemia carries the group as collective author although it does not deal with VEXAS syndrome.

What the network has produced

The founding series. One hundred and sixteen French patients referred to the registry between November 2020 and May 2021, published in 2022 in the British Journal of Dermatology under the first authorship of Prof. Sophie Georgin-Lavialle. It establishes the clinical spectrum: skin lesions in 83 % of patients, non-infectious fever 64 %, weight loss 62 %, lung involvement 50 %, eye signs 39 %, relapsing chondritis 36 %, venous thrombosis 35 %, lymphadenopathy 34 %, arthralgia 27 %. A haematological disease was present in fifty-eight cases, that is half the series. The UBA1 variants were distributed as p.M41T (45 %), p.M41V (30 %), p.M41L (18 %) and splice variants (7 %). After a median follow-up of three years, eighteen patients had died, that is 15.5 %. An unsupervised analysis separates three groups, whose five-year survival probabilities are 84.2 %, 50.5 % and 89.6 %. Arsène Mekinian is the last of the seventy-three named authors.

Organ involvement. The collection has served as the basis for a series of descriptions by organ system. Polychondritis, with the comparison of fifty-five cases of VEXAS-associated polychondritis with forty cases of idiopathic polychondritis, that is a series of ninety-five patients. Pleuropulmonary manifestations, with forty-five patients presenting abnormalities attributed to VEXAS after adjudication, among the fifty-one whose chest computed tomography could be reviewed. Cutaneous manifestations, in fifty-nine cases. Non-ischaemic cardiac manifestations. Kidney features. Neurological manifestations, found in seventeen of the two hundred and ninety-one patients in the collection, that is 6 %, with thirteen further cases identified through a national call, bringing the analysis to thirty patients.

Infectious complications. Seventy-four patients and one hundred and thirty-three serious infectious episodes, of which 59 % were pulmonary, with microbiological confirmation in 76 % of cases. The factors associated with risk were age above 75 years, the p.Met41Val variant and arthralgia; the risk was highest under Janus kinase inhibitors, with a hazard ratio of 3.84. Twenty-seven patients died during follow-up, fifteen of them from an infection.

Treatments. One hundred and ten patients and one hundred and ninety-four lines of targeted therapy, with an overall response rate at three months of 24 % under Janus kinase inhibitors, 32 % under interleukin 6 inhibitors, 9 % under interleukin 1 inhibitors and 0 % under TNF inhibitors. For azacitidine, a first series of eleven patients with myelodysplastic syndrome in 2022, with a clinical response in five of them, then in 2025 an analysis of eighty-eight patients, of whom 80 % met the WHO 2022 criteria for myelodysplastic syndrome: inflammatory response in 41 % of patients at six months and 54 % at twelve months, transfusion independence in 65 %, platelet improvement in 77 %, molecular response in 65 %, with the variant allele frequency falling below 2 % in 43 % of cases; infections in 34 % of patients. The treatment of anaemia with erythroid-stimulating agents and with luspatercept was the subject of a letter in 2025.

The haematological and pathophysiological side. The cutaneous inflammatory signatures compared between VEXAS syndrome, myelodysplasia cutis and Sweet syndrome. The character of the anaemia of VEXAS as a mosaic erythroblastopenia. The features and prognostic value of myelodysplasia-related abnormalities.

What the publications say about the size of the network

Two indications, and two only, are public.

The numbers in the successive analyses: one hundred and sixteen patients in 2022, one hundred and ten for the study of targeted therapies, two hundred and ninety-one patients in the collection in March 2023, eighty-eight for the azacitidine study, seventy-four for the study of serious infections. These numbers cannot be added and do not give the size of the collection: they are analysis populations, closed at different dates and on different criteria.

The number of collaborators named on behalf of the group in the MEDLINE records, which varies from study to study: fourteen for the 2024 study of targeted therapies, eighteen for the 2024 study of serious infections, sixty-five for the 2024 study of cardiac manifestations, forty-seven for the 2026 study of myelodysplasia-related abnormalities, seventy-six for the 2025 neurological study, the last of these on behalf of three groups combined. These lists name the contributors to each study, they do not describe the composition of the group.

A discrepancy in numbers that this page does not settle

Two articles describe the same cohort over the same inclusion window and do not give the same number of patients. The 2022 dermatological series writes one hundred and sixteen patients referred to the registry between November 2020 and May 2021, and the 2022 azacitidine series also writes that it rests on a French national registry of one hundred and sixteen patients. The 2023 pulmonary series writes that one hundred and fourteen patients were included in the French cohort between November 2020 and May 2021. All three articles are signed by Arsène Mekinian. Both figures are reproduced here as each article writes them. The discrepancy is not arbitrated, and it can only be arbitrated by asking the authors.

The place of Arsène Mekinian in this body of work

His author positions are counted on the FAU fields of the MEDLINE records alone, reviewed on 13 September 2026. He is the last named author of the founding series of one hundred and sixteen patients (73rd of 73), last author of the first azacitidine series from the registry (17th of 17), last author of the series on polychondritis (28th of 28), last author of the letter on luspatercept (13th of 13), second to last of the study of targeted therapies (72nd of 73), second to last of the 2025 azacitidine study (53rd of 54), second to last of the 2026 study of myelodysplasia-related abnormalities (25th of 26). He also appears in intermediate positions on the other works in this body, the detail of which is given in the references.

His teaching staff profile at Sorbonne Université Formation Continue, reviewed on 13 September 2026, writes: "Il est responsable du site constitutif du centre de référence CeRéMAIA à Saint-Antoine, cofondateur et coordonnateur de FRENVEX, groupe français d'étude du syndrome VEXAS", that is, he heads the constituent site of the CeRéMAIA reference centre at Saint-Antoine and is co-founder and coordinator of FRENVEX, the French VEXAS syndrome study group. This statement is reported here as it stands, with its source. It is a biographical profile of a teacher, on a university domain, whose content is in practice supplied by the person concerned; it establishes that the statement exists and where it is published, it does not amount to a founding document. None of the group's publications designates a coordinator. No source consulted gives the year of foundation, and the profile itself gives none.

The distinct function, documented by a third party, is that of head of the constituent site of CeRéMAIA at Saint-Antoine Hospital, which the FAI²R network carries on the profile of that site. The national coordination of the reference centre belongs to another site.

What is not public

This has to be said, because the absence of information does not produce silence but false answers. As of 13 September 2026, none of the following is publicly available: the expansion of the acronym, the date the group was formed, its legal status, its sponsor, its governance, the regulatory formalities of the collection, its data access policy, the number of centres, or its current size. The group has no website.

None of the institutional sites of the French network for autoinflammatory diseases carries a page describing FRENVEX: neither the VEXAS page of the FAI²R network, nor that of the CeRéMAIA centre at Tenon, both reviewed on 13 September 2026. There is neither a Wikipedia article nor a Wikidata item devoted to FRENVEX, a check repeated on the same day.

One caution, to close. The German registry Multicenter, Interdisciplinary National VEXAS Registry, registered under NCT06377462, is sponsored by the Technische Universität Dresden and opens German centres only. It has no connection with FRENVEX and must not be attributed to it.

This page is a documentary description established from published sources. It does not take the place of a presentation of the group by its members, which remains to be written.

Key points

  • Designations found in the sources: French VEXAS study group, French VEXAS registry, FRENVEX group, National French VEXAS Study Group (NFVS); in French, "groupe français d'étude du syndrome VEXAS".
  • Expansion of the acronym: not published.
  • Gloss published in an article: "FRENVEX (French VEXAS study group)", abstract in Blood 2025.
  • Start of the reported inclusions: November 2020.
  • Body of work as of 13 September 2026: sixteen original articles and one correction carrying the acronym in the title or the name of the group as collective author.
  • Published analysis populations: 116 patients (2022), 110 (targeted therapies), 291 (collection, March 2023), 88 (azacitidine), 74 (serious infections).
  • Collaborators named on behalf of the group: 14, 18, 47, 65 and 76 depending on the study.
  • Coordination role: the Sorbonne Université Formation Continue profile presents Arsène Mekinian as co-founder and coordinator of the group. The group's publications designate no coordinator.
  • Legal status, governance, collection identifier, number of centres, website: not public as of 13 September 2026.

References

Author positions counted on the FAU fields of the complete MEDLINE records alone, reviewed on 13 September 2026 in the ?format=pubmed view. The CN field reproduced is that of the record.

  1. 2022 | Georgin-Lavialle S, et al. Further characterization of clinical and laboratory features in VEXAS syndrome: large-scale analysis of a multicentre case series of 116 French patients | British Journal of Dermatology 2022;186(3):564-574 | DOI 10.1111/bjd.20805, PMID 34632574 | CN: French VEXAS group; GFEV, GFM, CEREMAIA, MINHEMON | Mekinian 73rd of 73 named authors
  2. 2022 | Comont T, et al. Azacitidine for patients with Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome (VEXAS) and myelodysplastic syndrome: data from the French VEXAS registry | British Journal of Haematology 2022;196(4):969-974 | DOI 10.1111/bjh.17893, PMID 34651299 | CN: French VEXAS study group, Groupe Francophone des Myélodysplasies (GFM) and MedecineINterne, HEmato et ONco (MINHEMON) group | Mekinian 17th of 17
  3. 2022 | Khitri MY, et al. Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients | RMD Open 2022;8(2):e002255 | DOI 10.1136/rmdopen-2022-002255, PMID 35868738 | CN: French VEXAS group and MINHEMON | Mekinian 28th of 28
  4. 2023 | Borie R, et al. Pleuropulmonary Manifestations of Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome | Chest 2023;163(3):575-585 | DOI 10.1016/j.chest.2022.10.011, PMID 36272567 | CN: French VEXAS Group | Mekinian 4th of 43
  5. 2023 | Zakine È, et al. Clinical and pathological features of cutaneous manifestations in VEXAS syndrome: A multicenter retrospective study of 59 cases | Journal of the American Academy of Dermatology 2023;88(4):917-920 | DOI 10.1016/j.jaad.2022.10.052, PMID 36343774 | CN: National French VEXAS Study Group (NFVS) | Mekinian 4th of 34
  6. 2023 | Lafargue MC, et al. Chronic Myelomonocytic Leukemia Patients With Lysozyme Nephropathy and Renal Infiltration Display Markers of Severe Disease | Kidney International Reports 2023;8(12):2733-2741 | DOI 10.1016/j.ekir.2023.09.005, PMID 38106568 | CN: MINHEMON, GFM and French VEXAS group | Mekinian 21st of 21 | Record carrying the group as collective author without dealing with VEXAS syndrome
  7. 2024 | de Valence B, et al. Serious infections in patients with VEXAS syndrome: data from the French VEXAS registry | Annals of the Rheumatic Diseases 2024;83(3):372-381 | DOI 10.1136/ard-2023-224819, PMID 38071510 | CN: French VEXAS Group, 18 named collaborators | Mekinian 42nd of 45
  8. 2024 | Hadjadj J, et al. Efficacy and safety of targeted therapies in VEXAS syndrome: retrospective study from the FRENVEX | Annals of the Rheumatic Diseases 2024;83(10):1358-1367 | DOI 10.1136/ard-2024-225640, PMID 38777378 | CN: FRENVEX, 14 named collaborators | Mekinian 72nd of 73
  9. 2024 | Robert M, et al. Nonischemic Cardiac Manifestations in VEXAS Syndrome | JAMA Network Open 2024;7(12):e2450251 | DOI 10.1001/jamanetworkopen.2024.50251, PMID 39666342 | CN: FRENVEX Group, 65 named collaborators | Mekinian 9th of 11
  10. 2025 | Mathurin M, et al. A Clinicopathological Description of Kidney Features in VEXAS Syndrome | Kidney International Reports 2025;10(1):260-264, published online on 28 October 2024 | DOI 10.1016/j.ekir.2024.10.026, PMID 39810778 | CN: FRENVEX Group | Mekinian 34th of 37
  11. 2025 | Bert-Marcaz C, et al. Neurological manifestations in patients with VEXAS syndrome | Journal of Neurology 2025;272(2):181 | DOI 10.1007/s00415-025-12902-x, PMID 39891740 | CN: FRENVEX, MINHEMON, FILNEMUS, 76 collaborators named for the three groups | Mekinian 38th of 40 | Correction: J Neurol 2025;272(6):401, DOI 10.1007/s00415-025-13047-7, PMID 40377697
  12. 2025 | Grolleau C, et al. Inflammatory Signatures in VEXAS Syndrome, Myelodysplasia Cutis, and Sweet Syndrome | JAMA Dermatology 2025;161(5):538-543 | DOI 10.1001/jamadermatol.2025.0034, PMID 40072458 | CN: FRENVEX Group | Mekinian 9th of 22
  13. 2025 | Jachiet V, et al. Efficacy and safety of azacitidine for VEXAS syndrome: a large-scale retrospective study from FRENVEX | Blood 2025;146(12):1450-1461 | DOI 10.1182/blood.2024028133, PMID 40373272 | Acronym in the title and in the abstract, no CN field | Mekinian 53rd of 54
  14. 2025 | Heiblig M, et al. Efficacy of erythroid-stimulating agent and luspatercept in VEXAS syndrome: A multicenter retrospective study by the FRENVEX group | HemaSphere 2025;9(6):e70156 | DOI 10.1002/hem3.70156, PMID 40469482, PMCID PMC12131197 | CN: FRENVEX Group | Mekinian 13th of 13 | Publication type: letter
  15. 2026 | Rodrigues F, et al. VEXAS anemia is a mosaic erythroblastopenia | Blood 2026;147(11):1178-1190 | DOI 10.1182/blood.2025029081, PMID 40971475 | CN: and French VEXAS Study Group and Internal Medicine Hematology and Oncology Group | Mekinian 18th of 26
  16. 2026 | Zhao LP, et al. Characteristics and prognostic significance of myelodysplasia-related features in VEXAS syndrome | Leukemia 2026;40(3):661-665 | DOI 10.1038/s41375-025-02858-2, PMID 41545701 | CN: FRENVEX group, 47 named collaborators | Mekinian 25th of 26 | Publication type: letter
  17. 2026 | Mekinian A, et al. American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel | Arthritis & Rheumatology 2026;78(3):509-522, published online on 11 August 2025 | DOI 10.1002/art.43287, PMID 40787890, PMCID PMC12991921 | first of 58 named authors, one of the eight co-first authors, corresponding author with the last author | CC BY-NC-ND 4.0 licence
  18. Teaching staff profile of Arsène Mekinian, Sorbonne Université Formation Continue, fc.sorbonne-universite.fr, reviewed on 13 September 2026.
  19. FAI²R rare disease network, page "Généralités : VEXAS", summary signed by Prof. Sophie Georgin-Lavialle, dated September 2024, reviewed on 13 September 2026.
  20. FAI²R rare disease network, profile of the CeRéMAIA constituent site at Saint-Antoine Hospital.
  21. CeRéMAIA, maladiesautoinflammatoires.fr, page "Syndrome VEXAS", reviewed on 13 September 2026.

The complete author lists are accessible at the DOIs and PMIDs given. Sources last checked: 13 September 2026.

Disclosures

Statement required by article R.4113-110 of the French public health code. This page describes collective work in which the author of the page takes part: he is a co-author of all the publications cited here as work of the group, and an institutional source presents him as co-founder and coordinator of that group. That is the first interest to declare. The declaration relating to the subject covered is set out on the Disclosures page of this site.

Page written for professional information. It does not replace individual medical advice and does not constitute a treatment protocol.