Plasma Exchanges to Treat Primary Systemic Necrotizing Vasculitides: Data from a French Nationwide Study

Gonzalo De Luna¹, Dominique Chauveau², Julien Aniort³, Pierre-Louis Carron⁴, Pierre Gobert⁵, Alexandre Karras⁶, Sylvain Adam-Marchand⁷, Francois Maurier⁸, Pierre-Yves Hatron⁹, Alexandre Mania¹⁰, Guillaume Le Guenno¹¹, Stéphane Ballly¹², Boris Bienvenu¹³, Eric Cardineau¹⁴, Tiphaine Goulenok¹⁵, Noémie Jourde-Chiche Sr.¹⁶, Maxime Samson¹⁷, Antoine Huart¹⁸, Jacques Pourrat¹⁹, Aurelien Tiple²⁰, Olivier Aumaître²¹, Xavier Puéchal²², Farhad Heshmati²³, Claire Le Jeunne²⁴, Luc Mouthon²⁵, Loïc Guillevin²⁶ and Benjamin Terrier²², ¹Medecine Interne, Cochin University Hospital, Paris, France, ²CH Toulouse, Toulouse, France, ³CHU, Clermont-Ferrand, France, ⁴Internal Medicine, Centre Hospitalier de Grenoble, Grenoble, France, ⁵Nephrology, Centre Hospitalier d'Avignon, Avignon, France, ⁶George Pompidou European Hospital, Paris, France, ⁷Pneumology, Centre Hospitalier Universitaire de Tours, Tours, France, ⁸HP Metz Belle Isle Hospital, Department of Internal Medicine, Metz, France, ⁹Service de Médecine Interne, Centre National de Référence des Maladies Systémiques Rares, Hôpital Claude Huriez, CHRU Lille, Lille, France, ¹⁰Hôpital Gabriel Montpied, Clermont-Ferrand, France, ¹¹Internal Medicine department, Clermont-Ferrand, France, ¹²CH, Chambéry, France, ¹³Internal Medicine, Hospital Caen, Caen, France, ¹⁴CH, Alencon, France, ¹⁵University Paris Diderot - APHP - Bichat Hospital, aris, France, ¹⁶Nephrology, Aix-Marseille Université - APHM, Marseille, France, ¹⁷Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, ¹⁸CHU, Toulouse, France, ¹⁹Nephrology, Rangeuil Hospital, Paris, France, ²⁰Nephrology, CHU, Clermont-Ferrand, France, ²¹Department of Internal Medicine 2. Referal center for SLE/APS, CHU Pitié-Salpêtrière, Paris, France, ²²Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Paris, France, ²³Cochin Hospital, Paris, France, ²⁴Department of Internal Medicine, Hotel-Dieu Hospital, AP-HP, Paris, Paris, France, ²⁵Department of Internal Medicine, Department of Internal Medicine, Cochin Hospital, Referent Center for Necrotizing Vasculitis and Systemic Sclerosis, Paris-Descartes University, AP-HP, Paris, France, ²⁶Internal Medicine, Hopital Cochin, Paris, France

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: ANCA, polyangiitis, treatment and vasculitis, Wegener's granulomatosis

Session Information

Date: Sunday, November 8, 2015

Title: Vasculitis Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

Plasma exchange (PE) is usually used to treat severe primary systemic necrotizing vasculitides (SNVs) and/or virus-induced vasculitides. Only severe renal insufficiency (serum creatinine (SCR) >500 µmol/l) was validated but long-term outcomes remain poor. In practice, PE may be used in clinical situations without literature input. This study aimed to identify practical PE indications for nonviral SNVs and evaluate short-term and long-term prognoses.

Methods:

This multicenter retrospective study (2005–2014) included PE-treated patients with AAV or nonviral polyarteritis nodosa (PAN) meeting ACR criteria, EMA algorithm and/or Chapel Hill nomenclature. For each indication, analysis of short- and long-term outcomes compared baseline (M0) vs post-PE parameter values.

Results:

Diagnoses of the 152 patients [94 men, 58 women; median age 64 (range 17–89) yr] were: 87 granulomatosis with polyangiitis (GPA), 56 microscopic polyangiitis (MPA), 5 PAN and 4 eosinophilic granulomatosis with polyangiitis (EGPA). ANCA were positive in 142/147 (97%, never PAN): 55% PR3-ANCA+ and 45% MPO-ANCA+.

PE was used for rapidly progressive glomerulonephritis (RPGN) in 126 (83%) [mean SCR 465±257 µmol/l; including <250, 250–500 and >500 µmol/l in one-third each], 64 (42%) alveolar hemorrhage most often RPGN-associated, 23 (15%) with extensive and severe multiple mononeuropathy, usually of acute onset (<4 weeks) and severe motor weakness, and 7 (5%) with extensive skin necrosis. M0 median BVAS was 18. Median (range) PE was 7 (1–12) sessions over a median of 11 (1–43) days.

After median follow-up of 22 (range 1–125) months post-PE onset, 18 (12%) had died, including 11 within M1–6. Renal function of 126 PE-treated RPGN patients improved significantly, as assessed by estimated glomerular filtration rate (eGFR) using MDRD, reaching a plateau between M3 and M6 post-PE onset, and maintaining eGFR through follow-up M24. According to M0-SCR (µmol/l) subgroup, M0-to-M6 eGFR (ml/min), respectively, rose from 33.3 to 47.3 (P<0.0001) for <250, from 13.5 to 34.7 (P<0.0001) for 250–500, and from 6.9 to 32.9 (P<0.0001) for >500. PE-session numbers were similar for the 3 M0-SCR subgroups, with eGFR improving as that number rose, suggesting a PE dose-dependent effect on eGFR recovery.

PE resolved alveolar hemorrhages in all 64 patients, enabling O_2-therapy or MV discontinuation, after a median of 15 days.

Motor weakness regressed markedly in 23 PE-treated extensive mononeuritis patients. Severe motor-weakness (MRC <3/5) declined from M0 52% to 23%, 19% and 12.5% at M3, M6 and M12 post-PE onset.

End-stage renal disease and/or mortality rates were similar among M0-SCR groups but higher for MPO- than PR3-ANCA+ patients. PE-attributable adverse events occurred in 63%. No one died during PE.

Conclusion:

Our results highlight PE indications for SNVs. Different organ involvements seem to benefit from PE. For RPGN patients, the PE number seemed to correspond to the degree of eGFR recovery. These findings support using PE in conditions less severe than previously validated.

Disclosure: G. De Luna, None; D. Chauveau, None; J. Aniort, None; P. L. Carron, None; P. Gobert, None; A. Karras, None; S. Adam-Marchand, None; F. Maurier, None; P. Y. Hatron, None; A. Mania, None; G. Le Guenno, None; S. Ballly, None; B. Bienvenu, None; E. Cardineau, None; T. Goulenok, None; N. Jourde-Chiche Sr., None; M. Samson, None; A. Huart, None; J. Pourrat, None; A. Tiple, None; O. Aumaître, None; X. Puéchal, None; F. Heshmati, None; C. Le Jeunne, None; L. Mouthon, None; L. Guillevin, None; B. Terrier, None.

To cite this abstract in AMA style:

De Luna G, Chauveau D, Aniort J, Carron PL, Gobert P, Karras A, Adam-Marchand S, Maurier F, Hatron PY, Mania A, Le Guenno G, Ballly S, Bienvenu B, Cardineau E, Goulenok T, Jourde-Chiche N Sr., Samson M, Huart A, Pourrat J, Tiple A, Aumaître O, Puéchal X, Heshmati F, Le Jeunne C, Mouthon L, Guillevin L, Terrier B. Plasma Exchanges to Treat Primary Systemic Necrotizing Vasculitides: Data from a French Nationwide Study [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/plasma-exchanges-to-treat-primary-systemic-necrotizing-vasculitides-data-from-a-french-nationwide-study/. Accessed .

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