Plasma exchanges for the treatment of severe systemic necrotizing vasculitides in clinical daily practice: Data from the French Vasculitis Study Group.
The use of plasma exchanges (PLEX) in systemic necrotizing vasculitides (SNV) still need to be codified. To describe indications, efficacy and safety of PLEX for the treatment of SNV, we conducted a multicenter retrospective study on patients with ANCA-associated vasculitis (AAV) or non-viral polyarteritis nodosa (PAN) treated with PLEX. One hundred and fifty-two patients were included: GPA (n = 87), MPA (n = 56), EGPA (n = 4) and PAN (n = 5). PLEX were used for rapidly progressive glomerulonephritis (RPGN) in 126 cases (86%), alveolar hemorrhage in 64 cases (42%), and severe mononeuritis multiplex in 23 cases (15%). In patients with RPGN, there was a significant improvement in renal function compared to baseline value (P < 0.0001), the plateau being reached at month 3 after PLEX initiation, and estimated glomerular filtration rate improved especially as the number of PLEX increased. In patients with alveolar hemorrhage, mechanical ventilation was discontinued in all patients after a median time of 15 days. Patients treated for mononeuritis multiplex showed improvement of severe motor weakness. After a median follow of 22 months, 18 deaths (12%) were recorded, mainly in patients with RPGN and within the first 6 months. Incidence of end-stage renal disease and/or death was similar between groups of different baseline renal function, but was increased in MPO-ANCA compared to PR3-ANCA. Adverse events attributable to PLEX were recorded in 63%. No death occurred during PLEX. This large series describes indications, efficacy and safety of PLEX in daily practice. Randomized controlled studies are ongoing to define optimal indications, PLEX regimen and concomitant medications.
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
Gonzalo
G
de Luna
Department of Nephrology, Centre Hospitalier Universitaire Toulouse, France.
Dominique
D
Chauveau
Department of Nephrology, Centre Hospitalier Universitaire Clermont-Ferrand, France.
Julien
J
Aniort
Department of Nephrology, Centre Hospitalier Universitaire, Grenoble, France.
Pierre-Louis
PL
Carron
Department of Nephrology, Centre Hospitalier, Avignon, France.
Pierre
P
Gobert
Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP, Université Paris Descartes, Paris, France.
Alexandre
A
Karras
Department of Pulmonology, Centre Hospitalier Universitaire, Tours, France.
Sylvain
S
Marchand-Adam
Department of Internal Medicine, Centre Hospitalier, Metz, France.
François
F
Maurier
Department of Internal Medicine, Hôpital Claude Huriez, Université Lille 2, Lille, France.
Pierre-Yves
PY
Hatron
Department of Internal Medicine, Hôpital Gabriel Montpied, Clermont-Ferrand, France.
Alexandre
A
Mania
Department of Internal Medicine, Hôpital Estaing, Clermont-Ferrand, France.
Guillaume
G
le Guenno
Department of Nephrology, Centre Hospitalier, Chambéry, France.
Stéphane
S
Bally
Department of Internal Medicine, Centre Hospitalier Universitaire, Caen, France.
Boris
B
Bienvenu
Department of Nephrology, Centre Hospitalier, Alençon, France.
Eric
E
Cardineau
Department of Internal Medicine, Hôpital Beaujon, AP-HP, Clichy, France.
Tiphaine
T
Goulenok
Department of Nephrology, Aix-Marseille Universite, AP-HM Hôpital de la Conception, Marseille, France.
Noémie
N
Jourde-Chiche
Department of Internal Medicine and Clinical Immunology, Centre Hospitalier Universitaire, Dijon, France.
Maxime
M
Samson
Department of Nephrology, Centre Hospitalier Universitaire Toulouse, France.
Antoine
A
Huart
Department of Nephrology, Centre Hospitalier Universitaire Toulouse, France.
Jacques
J
Pourrat
Department of Nephrology, Centre Hospitalier Universitaire Clermont-Ferrand, France.
Aurélien
A
Tiple
Department of Internal Medicine, Hôpital Gabriel Montpied, Clermont-Ferrand, France.
Olivier
O
Aumaitre
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
Xavier
X
Puéchal
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
Farhad
F
Heshmati
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
Claire
C
le Jeunne
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
Luc
L
Mouthon
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
Loïc
L
Guillevin
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France. Electronic address: benjamin.terrier@cch.aphp.fr.
Benjamin
B
Terrier
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000401:mortality / Q000628:therapy*; D005260:Female; D005602:France / Q000453:epidemiology; D005919:Glomerular Filtration Rate; D005921:Glomerulonephritis / Q000401:mortality / Q000628:therapy*; D006470:Hemorrhage / Q000401:mortality / Q000628:therapy*; D006801:Humans; D015994:Incidence; D007676:Kidney Failure, Chronic / Q000453:epidemiology; D008171:Lung Diseases / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D020422:Mononeuropathies / Q000628:therapy*; D010951:Plasma Exchange; D010488:Polyarteritis Nodosa / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
ANCA-Associated vasculitides; Eosinophilic granulomatosis with polyangiitis; Granulomatosis with polyangiitis; Microscopic polyangiitis; Plasma exchanges; Polyarteritis nodosa