Effectiveness of Rituximab in Severe Wegener`s Granulomatosis: Report of Two Cases and Review of the Literature

Oristrell, J; Bejarano, Guillermina; Jordana, R.; Monteagudo, Manuel; Marí, Begoña; Casanovas, A.; Tolosa, Carles

doi:10.2174/1874306400903010094

Cited by 12 publications

(9 citation statements)

References 23 publications

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“…Simultaneously, partial response regarding changes in the sinonasal granulomas was seen in three patients and no improvement in one patient. A beneficial effect of RTX for lung granulomas has been reported in several case series [23][24][25].…”

Section: Discussionmentioning

confidence: 86%

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

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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.

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Section: Discussionmentioning

confidence: 86%

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

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“…Granulomatosis with polyangiitisis (GPA) is an idiopathic systemic form of vasculitis characterized by involvement of the upper and lower airways and the kidneys. The exact cause of GPA is unknown, but it seems to have characteristics of an autoimmune disease [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness

2016

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Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.

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“…These patients were treated with rituximab and achieved sustainable remission due to limited response or adverse events with immunosuppressive drugs (table 1). 4 9–11 In our case, the patient had intestinal perforation and multiple systemic involvements under initial classical treatment and initiation of rituximab led to a valuable response, and the patient's BVAS/WG score diminished significantly. While rituximab was used 375 mg/m 2 in four cures in previously reported cases, we planned 1000 mg/intravenously in four cures to achieve sustainable remission.…”

Section: Discussionmentioning

confidence: 66%

Rituximab seems a promising therapeutic option in granulomatosis with polyangiitis with intestinal perforation: a case report and literature review

et al. 2013

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SUMMARYGranulomatosis with polyangiitis (Wegener's) (GPA) is a chronic disease of unknown aetiology that leads to necrotising vasculitis in small and medium-sized vessels characterised by respiratory system and kidney involvement. Intestinal involvement is rare and perforation is even rarer in GPA. In this study, we are presenting a literature review of related cases, and a 29-year-old man referred from the emergency department with a multiple distal ileal perforation that was diagnosed with GPA, and successfully treated with rituximab.

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Effectiveness of Rituximab in Severe Wegener`s Granulomatosis: Report of Two Cases and Review of the Literature

Cited by 12 publications

References 23 publications

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

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

Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness

Rituximab seems a promising therapeutic option in granulomatosis with polyangiitis with intestinal perforation: a case report and literature review

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