Wegener's granulomatosis

Wegener, F

doi:10.1007/bf00175962

Cited by 44 publications

(7 citation statements)

References 36 publications

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“…1, could explain the clearly dissociated therapeutic responses to it according to constitutional and vasculitis-or granulomatous-related manifestations. The poorer and later responses of granulomatous lesions could be attributed to the initiation of B-and T-cell autoreactivities in the initial inflammatory granuloma [7,12,14]. That lesion might be considered a potential protective sanctuary for autoimmune B cells in which marked rituximab-induced B-cell depletion occurs late and is only partial, as suggested for other "closed" body compartments (e.g., bone marrow, lymph nodes) [49,50], in contrast to the circulation, where B-cell depletion is fully and rapidly achieved.…”

Section: Do Rituximab Pharmacodynamics Concord With Its Observed Clinmentioning

confidence: 99%

“…Considering the natural history of this entity, several data support a localized granulomatous onset of the disease in connective tissue. The accompanying systemic ANCA-associated vasculitis mediated by B-lymphocytes is a secondary feature that represents a later stage of the disease [12][13][14]. The complete clinical triad [ear, nose, and throat (ENT), lung, and kidney] of WG is not always observed at the time of disease onset, with only 40% of the patients having renal disease, which is a vasculitis-related manifestation, although 80-90% of WG patients will ultimately go on to develop such involvement.…”

Section: Histopathological Features Of Wgmentioning

confidence: 99%

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Analysis of Wegener’s Granulomatosis Responses to Rituximab: Current Evidence and Therapeutic Prospects

Aouba

Pagnoux

Bienvenu

et al. 2007

Clinic Rev Allerg Immunol

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Available data on small numbers of patients with refractory and/or relapsing Wegener's granulomatosis support very good overall outcomes with rituximab, an anti-CD20 biotherapy targeting B cells, in combination with ongoing immunosuppressants, apparently independently of antineutrophil cytoplasm antibody (ANCA) status. However, clearly dissociated responses were observed, with constitutional and vasculitis-related symptoms often achieving complete responses within days or weeks, and granulomatous-related manifestations regressing more slowly over a few weeks to several months or not at all, particularly for orbital pseudotumors. Tolerance was good with few side effects, and when relapses occurred, more often after 12 months, a second or even a third rituximab cycle was almost always able to obtain another complete remission. Randomized trials are needed to define rituximab's best place, compared to cyclophosphamide, in initial and maintenance regimens; to determine optimally combined drugs, considering the various responses of granulomatous-related manifestations; and to delineate the role of circulating B-cell monitoring for pre-emptive management decisions. This promising biotherapy opens the way for other therapeutic agents targeting B lymphocytes and to improve our understanding of the pathophysiology of ANCA-associated vasculitis.

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Section: Do Rituximab Pharmacodynamics Concord With Its Observed Clinmentioning

confidence: 99%

Section: Histopathological Features Of Wgmentioning

confidence: 99%

Analysis of Wegener’s Granulomatosis Responses to Rituximab: Current Evidence and Therapeutic Prospects

Aouba

Pagnoux

Bienvenu

et al. 2007

Clinic Rev Allerg Immunol

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“…The primary granulomas form and develop in connective tissue, but without vascular involvement (5,6). In his last paper on this topic, Friedrich Wegener wrote "the vasculitis that accompanies the granulomatous disease is a secondary feature that represents a later stage" (7). Thus, insights into the leading immunopathogenic mechanisms in WG should come from studies concentrating on cells from the "pathergic granuloma" or its surroundings (e.g., bronchoalveolar lavage [BAL] fluid).…”

mentioning

confidence: 99%

Cytokine profiles in Wegener's granulomatosis: Predominance of type 1 (Th1) in the granulomatous inflammation

Csernok

Trabandt

Müller

et al. 1999

Arthritis & Rheumatism

192

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Methods. Competitive reverse transcriptasepolymerase chain reaction (RT-PCR) and enzymelinked immunosorbent assay were used to assess IFN␥ and IL-4 expression in T cell clones (TCC), T cell lines (TCL), and polyclonal CD4؉ and CD8؉ cells derived from NBS, BAL, and PB.Results. Patients with WG and chronic rhinitis were found to share in situ production of messenger RNA (mRNA) specific for IFN␥ (Th1). Only 2 patients with WG expressed IL-4, whereas IL-4 mRNA PCR products were found in inflamed tissues of the disease control patients. The granuloma-derived T cells of WG patients produced only IFN␥, while TCC, TCL, and CD4؉ and CD8؉ T cells from BAL and PB produced mainly IFN␥.Conclusion. Our data indicate that a Th1 cytokine pattern predominates in the granulomatous inflammation in patients with WG.

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“…The clinical features in correlation with histopathology were initially described by Wegener in 1936 1 . The disease is associated with granuloma formation and necrosis of the upper airway, lungs, and kidneys 2 , although other organs can also be affected.…”

Section: To the Editormentioning

confidence: 99%

Hydrocephalus in Wegener’s Granulomatosis: Neuroendoscopic Findings and Management

Rangel-Castilla

Barber

Zhang³

2011

J Rheumatol

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Wegener's granulomatosis

Cited by 44 publications

References 36 publications

Analysis of Wegener’s Granulomatosis Responses to Rituximab: Current Evidence and Therapeutic Prospects

Analysis of Wegener’s Granulomatosis Responses to Rituximab: Current Evidence and Therapeutic Prospects

Cytokine profiles in Wegener's granulomatosis: Predominance of type 1 (Th1) in the granulomatous inflammation

Hydrocephalus in Wegener’s Granulomatosis: Neuroendoscopic Findings and Management

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