Giant-cell Granuloma of the Respiratory Tract (Wegener's Granulomatosis)

Walton, E. W.

doi:10.1136/bmj.2.5091.265

Cited by 829 publications

(316 citation statements)

References 33 publications

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“…Untreated, the disease usually progresses from a limited necrotizing granulomatous process to a generalized vasculitis and leads to fatal outcome in >90% of patients in 2 years with mean survival time of 5 months [5]. The advent of cyclophosphamide (CYC) therapy together with corticosteroids for the induction of remission has reduced the mortality greatly and has become a conventional treatment option of GPA.…”

Section: Introductionmentioning

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

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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: Introductionmentioning

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

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“…However, since the report of aneurysm formation and free-vessel rupture of medium and large vessels in a patient with ANCA disease, the possibility that medium-sized vessels might be ruptured cannot be ruled out. 47 In our case, due to the absence of thrombocytopenia, disseminated intravascular coagulation, and trauma in the present case, GPA was considered to be the cause of the femoral hemorrhage. Although heparin was administered (5000 m for plasmapheresis), the last dose was given 3 h before the occurrence of rectus hemorrhage.…”

Section: -31mentioning

confidence: 52%

Hemorrhagic complications associated with PR3-ANCA crescentic glomerulonephritis

Cao

Tian

et al. 2015

Renal Failure

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“…This compared with a mean survival of 5 months for untreated Wegener's (Walton, 1958) and 12.5 months with corticosteroid therapy (Hollander & Manning, 1967) in earlier studies. In the NIH series 23 of their 85 cyclophosphamide treated patients were in remission off all treatment for a mean 35.3 months (Fauci et al, 1983).…”

Section: Cyclophosphamide and Chlorambucilmentioning

confidence: 67%