Reference center for rare systemic autoimmune and autoinflammatory diseases in adults in Île-de-France, East and West
The reference center for rare systemic autoimmune and autoinflammatory diseases in adults in Île-de-France, East and West of the Georges-Pompidou european hospital is specialised on RENAL DAMAGES CAUSED BY SYSTEMIC DISEASES AND COMPLEMENT DAMAGES (ARMAC). The center treats kidney diseases associated with rare autoimmune diseases (such as systemic lupus erythematosus, systemic vasculitis and scleroderma), as well as nephropathies secondary to defects in the complement system, which are involved in immune defences (membranoproliferative or C3 glomerulonephritis, atypical hemolytic uremic syndrome).
The HEGP site works in close collaboration with the coordinating reference center at Cochin hospital (rare systemic auto-immune and auto-inflammatory diseases in adults in Île-de-France, East and West).
This reference center is affiliated with the FAI²R rare diseases healthcare network.
Key words : systemic lupus erythematosus, lupus nephritis, systemic vasculitis, ANCA, systemic sclerosis, cryoglobulinemia, avacopan, rituximab, complement, C3 glomerulopathy, membranoproliferative glomerulonephritis (MPGN), hemolytic uremix syndrome, eculizumab.
Medical team
Pr Alexandre Karras
MD, PhD
Contact us
Phone. +33 1 56 09 57 00
or + 33 1 56 09 23 99
In case of emergency
The reference center for rare systemic autoimmune and autoinflammatory diseases in adults in Île-de-France, East and West aims to help diagnose and manage autoimmune kidney diseases in patients with systemic pathologies and/or acquired or inborn abnormalities of the complement system.
In order to achieve these objectives, our reference center offers exploration of these pathologies in consultation, day hospitalization or conventional hospitalization, in order to carry out the appropriate clinical, biological, radiological or histopathological investigations, and also to implement the treatment of these diseases.
Our diagnostic expertise is based on close collaboration with the HEGP immunology laboratory, a national reference center for complement and autoimmunity examination, a radiology platform of excellence for uronephrological examination, and our hospital group’s renal anatomopathology laboratory at the Necker site, which is the largest renal pathology center in France.
On the therapeutic level, our center handles all aspects of autoimmune disease therapy (immunosuppressive drugs, plasma exchange), as well as chronic kidney disease (hemodialysis and peritoneal dialysis, kidney transplant follow-up), while collaborating with the HEGP clinical investigation center (CIC) to take part in national and international clinical trials, evaluating innovative therapeutics in the field. We are also developing a therapeutic patient education program, to improve patients’ quality of life and encourage their involvement in the management of their pathology.
On the university level, our center is at the forefront of clinical and fundamental research aimed at understanding the pathophysiology of these diseases and developing new therapeutic approaches. At the same time, we contribute to the training of medical colleagues in these rare diseases, through specific courses (university diplomas, conferences, post-graduate training days) and the setting up and running of multidisciplinary consultation meetings on the themes of our reference center.
- Systemic lupus erythematosus
- Mixed connective tissue disease
- ANCA-associated vasculitis
- Microscopic polyangiitis
- Granulomatosis with polyangiitis
- Cryoglobulinemic vasculitis
- Immunoglobulin A vasculitis
- Anti-glomerular basement membrane vasculitis
- Polyarteritis nodosa
- Systemic sclerosis
- Primary Sjögren syndrome
- Anthiphospholipid syndrome
- C3 glomerulopathy
- Membranoproliferative glomerulonephritis
- Hemolytic uremic syndrome
- Pr Alexandre Karras, nephrologist, head of the reference center
- Dr Sophie Chauvet, nephrologist
- Pr Eric Thervet, nephrologist
A key element of comprehensive patient care, therapeutic patient education (TPE) is a multidisciplinary approach defined by the WHO: « TPE aims to help patients acquire or maintain the skills they need to manage their lives with chronic disease as effectively as possible ».
ETP thus gives patients the opportunity to take part in an individualized and controlled healthcare pathway, between a therapeutic standard proposed by the healthcare team and the patient’s own standard, based on his or her representations and projects.
Our ETP program is developed in collaboration with the coordinating reference center team at Cochin, organized by Dr Nathalie MOREL.
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- RENATO trial – renal ANCA-associated vasculitis – National hospital clinical research program (PHRC) (Ongoing)
- OBILUP trial – lupus nephritis- National hospital clinical research program (PHRC) (Ongoing)
- RIGA trial – immunoglobulin A vasculitis – National hospital clinical research program (PHRC) (Ongoing)
- Iptacopan trial – C3G/MPGN – Novartis (Ongoing)
- ICE trial – cryoglobulinemic vasculitis – National hospital clinical research program (PHRC) (Ongoing)
- MASTER trial- ANCA – ANCA-associated vasculitis dialysis – National hospital clinical research program (PHRC) (Ongoing)
- MAINRITSAN trial – ANCA-associated vasculitis – National hospital clinical research program (PHRC) (Finalized)
- MAINRITSAN 2 and 3 trials – ANCA-associated vasculitis – National hospital clinical research program (PHRC) (Finalized)
- WIN-lupus trial – lupus nephritis – National hospital clinical research program (PHRC) (Finalized)
- PNEUMOVAS trial – vaccination – vasculitis – National hospital clinical research program (PHRC) (Finalized)
- PEXIVAS trial – ANCA-associated vasculitis – International trial (Finalized)
- CLEAR trial – Avacopan and ANCA-associated vasculitis – Chemocentryx (Finalized)
- ADVOCATE trial – Avacopan and ANCA-associated vasculitis – Chemocentryx (Finalized)
- C3G_LNP023 trial – Iptacopan and C3G – Novartis (Finalized)
French vasculitis study group (GFEV) cohort
- Systemic diseases UD
Paris Cité university - Kidneys and systemic diseases IUD
Strasbourg and Paris Cité universities - Rare kidney diseases UD
Paris-Saclay university
2024
– Rituximab as maintenance therapy for ANCA- associated vasculitides: pooled analysis and long-term outcome of 277 patients included in the MAINRITSAN trials.
Delestre F, Charles P, Karras A, Pagnoux C, Néel A, Cohen P, Aumaître O, Faguer S, Gobert P, Maurier F, Samson M, Godmer P, Bonnotte B, Cottin V, Hanrotel-Saliou C, Le Gallou T, Carron PL, Desmurs-Clavel H, Direz G, Jourde- Chiche N, Lifermann F, Martin-Silva N, Pugnet G, Quéméneur T, Matignon M, Benhamou Y, Daugas E, Lazaro E, Limal N, Ducret M, Huart A, Viallard JF, Hachulla E, Perrodeau E, Puechal X, Guillevin L, Porcher R, Terrier B; French Vasculitis Study Group (FVSG).
Ann Rheum Dis. 2024 Jan 11;83(2):233-241. doi: 10.1136/ard-2023-224623. PMID: 37918894.
2023
– Proteinuria and hematuria after remission induction are associated with outcome in ANCA- associated vasculitis.
Benichou N, Charles P, Terrier B, Jones RB, Hiemstra T, Mouthon L, Bajema I, Berden A, Thervet E, Guillevin L, Jayne D, Karras A; French Vasculitis Study Group (FVSG) and European Vasculitis Society (EUVAS) investigators.
Kidney Int. 2023 Jun;103(6):1144-1155. doi: 10.1016/j.kint.2023.02.029. Epub 2023 Mar 20. PMID: 36940799.
– Rare Variants in Complement Gene in C3 Glomerulopathy and Immunoglobulin-Mediated Membranoproliferative GN.
Meuleman MS, Vieira-Martins P, El Sissy C, Audard V, Baudouin V, Bertrand D, Bridoux F, Louillet F, Dossier C, Esnault V, Jourde-Chiche N, Karras A, Morin MP, Provot F, Remy P, Ribes D, Rousset-Rouviere C, Servais A, Thervet E, Tricot L, Zaidan M, Wynckel A, Zuber J, Le Quintrec M, Frémeaux-Bacchi V, Chauvet S.
Clin J Am Soc Nephrol. 2023 Nov 1;18(11):1435-1445. doi: 10.2215/CJN.0000000000000252. Epub 2023 Aug 24. PMID: 37615951; PMCID: PMC10637453.
– C3 Glomerulopathy With Concurrent Thrombotic Microangiopathy: Clinical and Immunological Features.
Chabannes M, Rabant M, El Sissy C, Dragon-Durey MA, Vieira Martins P, Meuleman MS, Karras A, Buob D, Bridoux F, Daugas E, Audard V, Caillard S, Olagne J, Kandel C, Ferlicot S, Philipponnet C, Crepin T, Thervet E, Ducloux D, Frémeaux-Bacchi V, Chauvet S.
Am J Kidney Dis. 2023 Sep;82(3):279-289. doi: 10.1053/j.ajkd.2022.12.020. Epub 2023 Apr 14. PMID: 37061020.
2022
– Rituximab vs Cyclophosphamide Induction Therapy for Patients With Granulomatosis With Polyangiitis.
Puéchal X, Iudici M, Perrodeau E, Bonnotte B, Lifermann F, Le Gallou T, Karras A, Blanchard-Delaunay C, Quéméneur T, Aouba A, Aumaître O, Cottin V, Hamidou M, Ruivard M, Cohen P, Mouthon L, Guillevin L, Ravaud P, Porcher R, Terrier B; French Vasculitis Study Group.
JAMA Netw Open. 2022 Nov 1;5(11):e2243799. doi: 10.1001/jamanetworkopen.2022.43799. PMID: 36441554; PMCID: PMC9706346.
– Evaluation of Rituximab for Induction and Maintenance Therapy in Patients 75 Years and Older With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Thietart S, Karras A, Augusto JF, Philipponnet C, Carron PL, Delbrel X, Mesbah R, Blaison G, Duffau P, El Karoui K, Smets P, London J, Mouthon L, Guillevin L, Terrier B, Puéchal X; French Vasculitis Study Group.
JAMA Netw Open. 2022 Jul 1;5(7):e2220925. doi: 10.1001/jamanetworkopen.2022.20925. PMID: 35802372; PMCID: PMC9270693.
– Management of severe renal disease in anti-neutrophil-cytoplasmic-antibody-associated vasculitis: the place of rituximab and plasma exchange?
Morel P, Karras A, Porcher R, Belenfant X, Audard V, Rafat C, Hanouna G, Beaudreuil S, Vilain C, Hummel A, Terrier B, Pillebout E, Groh M, Jouenne R, Dhote R, Fain O, Ponsoye M, Noel N, Limal N, Puéchal X, Le Jeunne C, Guillevin L, Mouthon L, Régent A.
Rheumatology (Oxford). 2022 Oct 6;61(10):4056-4064. doi: 10.1093/rheumatology/keac046. PMID: 35108368.
– Kidney Histopathology Can Predict Kidney Function in ANCA-Associated Vasculitides with Acute Kidney Injury Treated with Plasma Exchanges.
Nezam D, Porcher R, Grolleau F, Morel P, Titeca-Beauport D, Faguer S, Karras A, Solignac J, Jourde-Chiche N, Maurier F, Sakhi H, El Karoui K, Mesbah R, Carron PL, Audard V, Ducloux D, Paule R, Augusto JF, Aniort J, Tiple A, Rafat C, Beaudreuil S, Puéchal X, Gobert P, Massy Z, Hanrotel C, Bally S, Martis N, Durel CA, Desbuissons G, Godmer P, Hummel A, Perrin F, Néel A, De Moreuil C, Goulenok T, Guerrot D, Grange S, Foucher A, Deroux A, Cordonnier C, Guilbeau-Frugier C, Modesto-Segonds A, Nochy D, Daniel L, Moktefi A, Rabant M, Guillevin L, Régent A, Terrier B; on behalf of the French Vasculitis Study Group.
J Am Soc Nephrol. 2022 Mar;33(3):628-637. doi: 10.1681/ASN.2021060771. Epub 2022 Jan 24. PMID: 35074934; PMCID: PMC8975074.
– Results from a nationwide retrospective cohort measure the impact of C3 and soluble C5b-9 levels on kidney outcomes in C3 glomerulopathy.
Chauvet S, Hauer JJ, Petitprez F, Rabant M, Martins PV, Baudouin V, Delmas Y, Jourde-Chiche N, Cez A, Ribes D, Cloarec S, Servais A, Zaidan M, Daugas E, Delahousse M, Wynckel A, Ryckewaert A, Sellier-Leclerc AL, Boyer O, Thervet E, Karras A, Smith RJH, Frémeaux-Bacchi V.
Kidney Int. 2022 Oct;102(4):904-916. doi: 10.1016/j.kint.2022.05.027. Epub 2022 Jun 22. PMID: 35752323; PMCID: PMC10588728.
– Complement Activation and Thrombotic Microangiopathy Associated With Monoclonal Gammopathy: A National French Case Series.
Martins M, Bridoux F, Goujon JM, Meuleman MS, Ribes D, Rondeau E, Guerry MJ, Delmas Y, Levy B, Ducloux D, Kandel-Aznar C, Le Fur A, Garrouste C, Provot F, Gibier JB, Thervet E, Bruneval P, Rabant M, Karras A, Dragon Durey MA, Fremeaux-Bacchi V, Chauvet S.
Am J Kidney Dis. 2022 Sep;80(3):341-352. doi: 10.1053/j.ajkd.2021.12.014. Epub 2022 Feb 22. PMID: 35217094.
2021
– Disease Activity and Adverse Events in Patients with ANCA-Associated Vasculitides Undergoing Long-Term Dialysis.
Kauffmann M, Bobot M, Robert T, Burtey S, Couvrat-Desvergnes G, Lavainne F, Puéchal X, Terrier B, Quéméneur T, Faguer S, Karras A, Brunet P, Couchoud C, Jourde-Chiche N; REIN registry and the French Vasculitis Study Group.
Clin J Am Soc Nephrol. 2021 Nov;16(11):1665-1675. doi: 10.2215/CJN.03190321. PMID: 34750159; PMCID: PMC8729406.
– Reappraisal of Renal Arteritis in ANCA-associated Vasculitis: Clinical Characteristics, Pathology, and Outcome.
Boudhabhay I, Delestre F, Coutance G, Gnemmi V, Quemeneur T, Vandenbussche C, Lazareth H, Canaud G, Tricot L, Gosset C, Hummel A, Terrier B, Rabant M, van Daalen EE, Wester Trejo MAC, Bajema IM, Karras A, Duong Van Huyen JP.
J Am Soc Nephrol. 2021 Sep;32(9):2362-2374. doi: 10.1681/ASN.2020071074. Epub 2021 Jun 21. PMID: 34155059; PMCID: PMC8729836.
– Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases.
Durel CA, Sinico RA, Teixeira V, Jayne D, Belenfant X, Marchand-Adam S, Pugnet G, Gaultier J, Le Gallou T, Titeca-Beauport D, Agard C, Barbet C, Bardy A, Blockmans D, Boffa JJ, Bouet J, Cottin V, Crabol Y, Deligny C, Essig M, Godmer P, Guilpain P, Hirschi-Santelmo S, Rafat C, Puéchal X, Taillé C, Karras A; French Vasculitis Study Group (FVSG).
Rheumatology (Oxford). 2021 Jan 5;60(1):359-365. doi: 10.1093/rheumatology/keaa416. PMID: 32856066.
2020
– Microscopic polyangiitis: Clinical characteristics and long-term outcomes of 378 patients from the French Vasculitis Study Group Registry.
Nguyen Y, Pagnoux C, Karras A, Quéméneur T, Maurier F, Hamidou M, Le Quellec A, Chiche NJ, Cohen P, Régent A, Lifermann F, Mékinian A, Khouatra C, Hachulla E, Pourrat J, Ruivard M, Godmer P, Viallard JF, Terrier B, Mouthon L, Guillevin L, Puéchal X; French Vasculitis Study Group.
J Autoimmun. 2020 Aug;112:102467. doi: 10.1016/j.jaut.2020.102467. Epub 2020 Apr 25. PMID: 32340774.
– Anti-Factor B Antibodies and Acute Postinfectious GN in Children.
Chauvet S, Berthaud R, Devriese M, Mignotet M, Vieira Martins P, Robe-Rybkine T, Miteva MA, Gyulkhandanyan A, Ryckewaert A, Louillet F, Merieau E, Mestrallet G, Rousset-Rouvière C, Thervet E, Hogan J, Ulinski T, Villoutreix BO, Roumenina L, Boyer O, Frémeaux-Bacchi V.
J Am Soc Nephrol. 2020 Apr;31(4):829-840. doi: 10.1681/ASN.2019080851. Epub 2020 Feb 7. PMID: 32034108; PMCID: PMC7191928.
A.Karras :
- American Society of Néphrology congress (2020) : prognostic value of persistent hematuria and proteinuria in ANCA associated vasculitis (oral presentation)
- Congress of the French-speaking Society of Nephrology Dialysis and Transplantation (2021) : therapeutic advances in the treatment of ANCA-associated vasculitis
- University Seminars in Nephrology (2022) : complement and ANCA-associated vasculitis
- Pitié immunopathology days ( 2022) : lupus nephritis and new therapeutic combinations
- Viggo Petersen Rheumatology Days (2023) : therapeutic advances in lupus nephritis
- EULAR Congress (European Society of Rheumatology) ( 2023) : ANCA vasculitis and the Kidney
- University Seminars in Nephrology (2024) : How to individualize treatment in ANCA-associated vasculitis
S.Chauvet :
- International Conference on Complement Therapeutics (Greece 2023) : Impact of C3 and sC5b-9 on renal outcomes of C3 glomerulopathy: results from a national-wide retrospective cohort
- Podocyte Meeting, USA. (2023) : C3 glomerulopathy, mechanisms of complement activation and correlation with disease phenotype
- University Seminars in Nephrology (2023) : C3 glomerulopathy : pathophysiology and therapeutic perspectives.
- Congress of the French-speaking Society of Nephrology Dialysis and Transplantation (2023) : C3 glomerulopathy : current therapies and prospects,
- Pierre Royer Nephropediatrics Seminar (2023) : Pediatric forms of C3 glomerulopathy: what are the particularities?
- International Society of Nephrology Frontiers. Bergame (2023) Complement-related kidney diseases: classification, genetics and treatment
Contact information
At the Georges-Pompidou european hospital, the reference center for rare systemic autoimmune and autoinflammatory diseases in adults in Île-de-France, East and West in brief …
* data valid for 2022