Rituximab therapy for primary central nervous system vasculitis: A 6 patient experience and review of the literature

Carlo Salvarani, Robert D Jr. Brown, Francesco Muratore, Teresa J.H. Christianson, Elena Galli, Nicolò Pipitone, Giulia Cassone, John Huston, Caterina Giannini, Kenneth J Warrington, Gene G. Hunder

Research output: Contribution to journalReview article

1 Citation (Scopus)

Abstract

Objectives: To assess the efficacy and safety of Rituximab (RTX) in adult primary central nervous system vasculitis (PCNSV). Methods: We retrospectively assessed the effect of RTX in 6 patients with PCNSV. Five of the 6 were refractory to high dose glucocorticoids (GCs) and/or conventional immunosuppressants (IS). The sixth was newly diagnosed and received RTX in combination with GCs. Clinical evaluation, laboratory tests, and imaging modalities were performed at initial RTX administration and during the follow-up. Treatment response was assessed using the treating physician's global opinion regarding response and the degree of disability using the modified Rankin scale (mRS). We also performed a literature review for previous use of RTX in PCNSV using PubMed, Ovid Medline, and the Cochrane library. Results: The six patients (3 females) had a median age at diagnosis of 50.5 years (range 17–68 years). All had active disease when RTX was started. In 4 patients, RTX administration was associated with a marked reduction in the number of flares (from 18 before starting RTX to 3 after). One patient, after an initial improvement, had 2 flares when B cells were depleted and he was not able to reduce prednisone below 20 mg/day. A 6th patient had a flare when B cells recovered and retreatment with RTX re-induced and maintained remission. The median mRS score at last visit (median: 2; range 0–4) was lower than that prior to treatment (median 3; range 1–5). The median prednisone daily dose before RTX administration was significantly higher than that at last follow-up (p =.006). In the literature review, we identified 5 papers describing 7 patients treated with RTX. Six patients responded to RTX with clinical and MRI improvement with no reported flares after RTX treatment. Conclusions: Our data support a potential role for RTX treatment in selected patients with PCNSV.

Original languageEnglish (US)
JournalAutoimmunity Reviews
DOIs
StatePublished - Jan 1 2019

Fingerprint

Central Nervous System Vasculitis
Therapeutics
Prednisone
Rituximab
Glucocorticoids
B-Lymphocytes
Retreatment
Immunosuppressive Agents

Keywords

  • Disability
  • PCNSV
  • Rituximab

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Rituximab therapy for primary central nervous system vasculitis : A 6 patient experience and review of the literature. / Salvarani, Carlo; Brown, Robert D Jr.; Muratore, Francesco; Christianson, Teresa J.H.; Galli, Elena; Pipitone, Nicolò; Cassone, Giulia; Huston, John; Giannini, Caterina; Warrington, Kenneth J; Hunder, Gene G.

In: Autoimmunity Reviews, 01.01.2019.

Research output: Contribution to journalReview article

Salvarani, Carlo ; Brown, Robert D Jr. ; Muratore, Francesco ; Christianson, Teresa J.H. ; Galli, Elena ; Pipitone, Nicolò ; Cassone, Giulia ; Huston, John ; Giannini, Caterina ; Warrington, Kenneth J ; Hunder, Gene G. / Rituximab therapy for primary central nervous system vasculitis : A 6 patient experience and review of the literature. In: Autoimmunity Reviews. 2019.
@article{4c5524f0f83f445ea4a4054425593fc6,
title = "Rituximab therapy for primary central nervous system vasculitis: A 6 patient experience and review of the literature",
abstract = "Objectives: To assess the efficacy and safety of Rituximab (RTX) in adult primary central nervous system vasculitis (PCNSV). Methods: We retrospectively assessed the effect of RTX in 6 patients with PCNSV. Five of the 6 were refractory to high dose glucocorticoids (GCs) and/or conventional immunosuppressants (IS). The sixth was newly diagnosed and received RTX in combination with GCs. Clinical evaluation, laboratory tests, and imaging modalities were performed at initial RTX administration and during the follow-up. Treatment response was assessed using the treating physician's global opinion regarding response and the degree of disability using the modified Rankin scale (mRS). We also performed a literature review for previous use of RTX in PCNSV using PubMed, Ovid Medline, and the Cochrane library. Results: The six patients (3 females) had a median age at diagnosis of 50.5 years (range 17–68 years). All had active disease when RTX was started. In 4 patients, RTX administration was associated with a marked reduction in the number of flares (from 18 before starting RTX to 3 after). One patient, after an initial improvement, had 2 flares when B cells were depleted and he was not able to reduce prednisone below 20 mg/day. A 6th patient had a flare when B cells recovered and retreatment with RTX re-induced and maintained remission. The median mRS score at last visit (median: 2; range 0–4) was lower than that prior to treatment (median 3; range 1–5). The median prednisone daily dose before RTX administration was significantly higher than that at last follow-up (p =.006). In the literature review, we identified 5 papers describing 7 patients treated with RTX. Six patients responded to RTX with clinical and MRI improvement with no reported flares after RTX treatment. Conclusions: Our data support a potential role for RTX treatment in selected patients with PCNSV.",
keywords = "Disability, PCNSV, Rituximab",
author = "Carlo Salvarani and Brown, {Robert D Jr.} and Francesco Muratore and Christianson, {Teresa J.H.} and Elena Galli and Nicol{\`o} Pipitone and Giulia Cassone and John Huston and Caterina Giannini and Warrington, {Kenneth J} and Hunder, {Gene G.}",
year = "2019",
month = "1",
day = "1",
doi = "10.1016/j.autrev.2018.12.002",
language = "English (US)",
journal = "Autoimmunity Reviews",
issn = "1568-9972",
publisher = "Elsevier",

}

TY - JOUR

T1 - Rituximab therapy for primary central nervous system vasculitis

T2 - A 6 patient experience and review of the literature

AU - Salvarani, Carlo

AU - Brown, Robert D Jr.

AU - Muratore, Francesco

AU - Christianson, Teresa J.H.

AU - Galli, Elena

AU - Pipitone, Nicolò

AU - Cassone, Giulia

AU - Huston, John

AU - Giannini, Caterina

AU - Warrington, Kenneth J

AU - Hunder, Gene G.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Objectives: To assess the efficacy and safety of Rituximab (RTX) in adult primary central nervous system vasculitis (PCNSV). Methods: We retrospectively assessed the effect of RTX in 6 patients with PCNSV. Five of the 6 were refractory to high dose glucocorticoids (GCs) and/or conventional immunosuppressants (IS). The sixth was newly diagnosed and received RTX in combination with GCs. Clinical evaluation, laboratory tests, and imaging modalities were performed at initial RTX administration and during the follow-up. Treatment response was assessed using the treating physician's global opinion regarding response and the degree of disability using the modified Rankin scale (mRS). We also performed a literature review for previous use of RTX in PCNSV using PubMed, Ovid Medline, and the Cochrane library. Results: The six patients (3 females) had a median age at diagnosis of 50.5 years (range 17–68 years). All had active disease when RTX was started. In 4 patients, RTX administration was associated with a marked reduction in the number of flares (from 18 before starting RTX to 3 after). One patient, after an initial improvement, had 2 flares when B cells were depleted and he was not able to reduce prednisone below 20 mg/day. A 6th patient had a flare when B cells recovered and retreatment with RTX re-induced and maintained remission. The median mRS score at last visit (median: 2; range 0–4) was lower than that prior to treatment (median 3; range 1–5). The median prednisone daily dose before RTX administration was significantly higher than that at last follow-up (p =.006). In the literature review, we identified 5 papers describing 7 patients treated with RTX. Six patients responded to RTX with clinical and MRI improvement with no reported flares after RTX treatment. Conclusions: Our data support a potential role for RTX treatment in selected patients with PCNSV.

AB - Objectives: To assess the efficacy and safety of Rituximab (RTX) in adult primary central nervous system vasculitis (PCNSV). Methods: We retrospectively assessed the effect of RTX in 6 patients with PCNSV. Five of the 6 were refractory to high dose glucocorticoids (GCs) and/or conventional immunosuppressants (IS). The sixth was newly diagnosed and received RTX in combination with GCs. Clinical evaluation, laboratory tests, and imaging modalities were performed at initial RTX administration and during the follow-up. Treatment response was assessed using the treating physician's global opinion regarding response and the degree of disability using the modified Rankin scale (mRS). We also performed a literature review for previous use of RTX in PCNSV using PubMed, Ovid Medline, and the Cochrane library. Results: The six patients (3 females) had a median age at diagnosis of 50.5 years (range 17–68 years). All had active disease when RTX was started. In 4 patients, RTX administration was associated with a marked reduction in the number of flares (from 18 before starting RTX to 3 after). One patient, after an initial improvement, had 2 flares when B cells were depleted and he was not able to reduce prednisone below 20 mg/day. A 6th patient had a flare when B cells recovered and retreatment with RTX re-induced and maintained remission. The median mRS score at last visit (median: 2; range 0–4) was lower than that prior to treatment (median 3; range 1–5). The median prednisone daily dose before RTX administration was significantly higher than that at last follow-up (p =.006). In the literature review, we identified 5 papers describing 7 patients treated with RTX. Six patients responded to RTX with clinical and MRI improvement with no reported flares after RTX treatment. Conclusions: Our data support a potential role for RTX treatment in selected patients with PCNSV.

KW - Disability

KW - PCNSV

KW - Rituximab

UR - http://www.scopus.com/inward/record.url?scp=85062240089&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85062240089&partnerID=8YFLogxK

U2 - 10.1016/j.autrev.2018.12.002

DO - 10.1016/j.autrev.2018.12.002

M3 - Review article

C2 - 30743080

AN - SCOPUS:85062240089

JO - Autoimmunity Reviews

JF - Autoimmunity Reviews

SN - 1568-9972

ER -