Two Cases of Refractory Wegeners Granulomatosis Successfully Treated with Rituximab

Tamura, Naoto; Matsudaira, Ran; Hirashima, Mika; Ikeda, Makoto; Tajima, Masanori; Nawata, Masuyuki; Morimoto, Shinji; Kaneda, Kazuhiko; Kobayashi, Shigeto; Hashimoto, Hiroshi; Takasaki, Yoshinari

doi:10.2169/internalmedicine.46.6156

Cited by 73 publications

(34 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Multiple courses of rituximab effectively induced further remissions following relapses, and re-treatment on a preemptive basis resulted in sustained remissions. These results are consistent with those of previous studies (9,(12)(13)(14)(15)(16)(17)(18)(19)(20). This is the largest series reported to date, and it pools the results from 4 UK centers.…”

Section: Discussionsupporting

confidence: 92%

“…In studies of small numbers of patients, rituximab has been associated with high rates of remission, often Ͼ80%, in patients with vasculitis that had been refractory to standard therapies (9,(12)(13)(14)(15)(16)(17)(18)(19). Relapses of vasculitis following rituximab therapy have occurred, although efficacy with second and subsequent courses of rituximab has been reported (9,13,14).…”

mentioning

confidence: 99%

See 1 more Smart Citation

A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody–associated vasculitis

Jones¹,

Ferraro²,

Chaudhry³

et al. 2009

Arthritis & Rheumatism

327

214

View full text Add to dashboard Cite

Objective. B cell depletion with rituximab has allowed remissions in relapsing or refractory antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in small studies. The aim of this study was to determine the efficacy and safety of rituximab for ANCA-associated vasculitis in a larger multicenter cohort. This permitted comparison of rituximab dosing regimens, the value of continuing immunosuppression, and investigation of ANCA and B cell levels as retreatment biomarkers.Methods. Retrospective, standardized data collection from 65 sequential patients receiving rituximab for refractory ANCA-associated vasculitis at 4 centers in the UK was used.Results. All patients achieved B cell depletion. Complete remission occurred in 49 of the 65 patients (75%), partial remission in 15 (23%), and no response in 1 (2%). The prednisolone dosage was reduced from 12.5 mg/day (median) to 9.0 mg/day at 6 months (P ‫؍‬ 0.0006). Immunosuppressive therapy was withdrawn in 37 of 60 patients (62%). Twenty-eight of 49 patients who achieved full remission (57%) experienced relapse (median 11.5 months). B cell return preceded relapse in 14 of 27 patients (52%). Although ANCA levels fell after rituximab therapy, relapse was not associated with ANCA positivity or a rise in ANCA levels. Neither the initial rituximab regimen (4 infusions of 375 mg/m 2 each given 1 week apart or 2 infusions of 1 gm each given 2 weeks apart) nor withdrawal of immunosuppressive therapy (37 of 60 patients [62%]) influenced the timing of relapse. Thirty-eight patients received >2 courses of rituximab, and complete remission was induced or maintained in 32 of them (84%). IgM levels fell, although IgG levels remained stable. Forty-six serious adverse events occurred, including 2 episodes of lateonset neutropenia, which were attributed to rituximab.Conclusion. Rituximab was effective remission induction therapy for refractory ANCA-associated vasculitis in this study. There was no difference in efficacy between the 2 main treatment regimens. Continuing immunosuppression did not reduce relapses. Relapses occurred, but re-treatment was effective and safe. There was no clear influence of rituximab on the frequency of serious adverse events. ANCA and B cell levels lacked sufficient sensitivity to guide the timing of re-treatment.

show abstract

Section: Discussionsupporting

confidence: 92%

mentioning

confidence: 99%

A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody–associated vasculitis

Jones¹,

Ferraro²,

Chaudhry³

et al. 2009

Arthritis & Rheumatism

327

214

View full text Add to dashboard Cite

show abstract

“…29 RTX was actually effective for one patient who suffered from intractable HP which was resistant to other conventional immunosuppressive agents, and the efficacy of RTX has also been reported in some cases of refractory HP associated with AAV. [30][31][32] Additionally, favorable outcomes were achieved with the combination of MTX and PSL in patients who indicated HP as initial and/or limited involvement without another severe visceral disorder, suggesting the concomitant use of MTX may be useful for improving HP associated with AAV unless other life-threatening organ impairment is involved. Considering not only our favorable results but also the inferiority of PSL monotherapy as previously Figure 2 Representative findings of thickened dura mater with gadolinium (Gd)-enhancement on T1-weighted image, and histopathological examination.…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

et al. 2017

View full text Add to dashboard Cite

Aim: To elucidate the characteristics of patients with hypertrophic pachymeningitis (HP) in a population with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods:We retrospectively investigated the clinical records of 39 patients who were diagnosed with AAV. To determine the characteristics of HP in AAV, the epidemiological and clinical data from patients with HP were statistically compared with those from patients without HP.Results: Of 39 patients with AAV, seven (17.9%) had associated HP. All patients with HP were classified as having granulomatosis with polyangiitis (GPA), whereas only five of 32 patients without HP were diagnosed as having GPA (P < 0.0001). The frequencies of myeloperoxidase (MPO)-ANCA and proteinase 3-ANCA positivity in patients with HP were equivalent, while MPO-ANCA positivity was obviously dominant in patients without HP. HP occurred as the initial clinical episode of AAV in three patients (7.7% of all AAV). Frequent significant characteristics of patients with HP were headache, cranial neuropathy and paranasal involvement (P < 0.05), and histopathological findings from paranasal involvement were useful for the diagnosis of GPA in some patients with HP. Combination therapy of corticosteroid and an immunosuppressant, such as methotrexate, cyclophosphamide or rituximab, was effective for achieving remission and improving radiographic findings of HP.Conclusion: AAV is a common cause of HP; epidemiological features of AAV patients with HP are different from those of patients without HP. Additionally, HP impacts diagnosis because it may be an initial clinical sign of AAV.

show abstract

“…Several case reports suggest a beneficial effect of anti-B cell treatment in refractory orbital granulomas [18][19][20]. Taylor et al [21] recently reported beneficial effect of RTX treatment in seven patients with granulomatous orbital disease who all entered remission within 2-7 months without relapse.…”

Section: Discussionmentioning

confidence: 99%

Off‐Trial Evaluation of the B cell–Targeting Treatment in the Refractory Cases of Antineutrophil Cytoplasmic Antibodies (ANCA)‐Associated Vasculitis: Long‐Term Follow‐Up from a Single Centre

et al. 2012

View full text Add to dashboard Cite

The aim of the study was to evaluate long‐term clinical and immunological effects of anti‐B cell treatment in patients with antineutrophil cytoplasmic antibodies (ANCA)‐associated vasculitis refractory to conventional immunosuppressive treatment. Rituximab (RTX) was added to the ongoing immunosuppressive treatment in 29 patients with refractory ANCA‐associated vasculitis. The disease activity was measured using Birmingham Vasculitis Activity Score/Wegener’s granulomatosis (BVAS/WG score), and clinical laboratory variables were recorded. The median BVAS/WG score before treatment was 6 (IQR 3–8), and 28 patients (97%) had disease flare classified either severe (62%) or limited (34%). Six of 29 patients (21%) achieved a complete remission, and 12 (41%) had a treatment response with ≥50% decrease in BVAS/WG score at 6 months. Fourteen patients (64%) with kidney involvement achieved remission, and in seven patients (50%), no flare was seen during the follow‐up period. Three patients had renal flare and were successfully re‐treated with RTX. Seventeen patients had disease symptoms from airways and eyes at RTX initiation, whereas only 29% displayed ≥50% treatment response. Limited clinical improvement was seen in patients with endobronchial lesions and trachea‐subglottic granulomatous disease. RTX is a potent therapeutic option for ANCA‐associated vasculitis refractory to conventional treatment. Best response may be expected in patients with vasculitic manifestations.

show abstract

Two Cases of Refractory Wegeners Granulomatosis Successfully Treated with Rituximab

Abstract: Abstract

Cited by 73 publications

References 20 publications

A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody–associated vasculitis

A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody–associated vasculitis

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

Off‐Trial Evaluation of the B cell–Targeting Treatment in the Refractory Cases of Antineutrophil Cytoplasmic Antibodies (ANCA)‐Associated Vasculitis: Long‐Term Follow‐Up from a Single Centre

Contact Info

Product

Resources

About