Prospective long-term follow-up of patients with localised Wegener's granulomatosis: does it occur as persistent disease stage?

Holle, Julia; Gross, Wolfgang L.; Holl‐Ulrich, Konstanze; Ambrosch, Petra; Noelle, Bernhard; Both, Marcus; Csernok, Elena; Moosig, Frank; Schinke, S.; Reinhold‐Keller, E.

doi:10.1136/ard.2010.130203

Cited by 195 publications

(181 citation statements)

References 31 publications

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“…4 AAV is categorized as microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA) based on the phenotypes of ANCA, the histopathological findings of granulomatosis and/or necrotizing vasculitis formation, and clinical findings such as prior asthma, existence of respiratory tract lesions, and/or urinary tract involvement. 4,5 AAV develops not only in a single organ, such as 'pulmonary-limited type' or 'renal-limited type', [6][7][8] but also into multi-organ disorders targeting the skin, respiratory tract, kidneys, cardiovascular and gastrointestinal systems, with general symptoms including fever, arthralgia or myalgia. The nervous system is also a common target of AAV, and furthermore, this is sometimes life-threatening and the cause of serious handicap.…”

Section: Introductionmentioning

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

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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.

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Section: Introductionmentioning

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

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“…Ein ANCA-Nachweis gelingt bei lokalisierter (auf die oberen Atemwege beschränkter) GPA (<50 %) und der EGPA (ca. 40 %) seltener [79,92,201]. Für die AAV bestehen bisher keine konsentierten Diagnosekriterien.…”

Section: Diagnostikunclassified

“…Eine Sonderstellung nimmt die lokalisierte Form der GPA ein [92]. In diesen Fällen ist der Nachweis der klassischen histomorphologischen Trias (granulomatöse Entzündung, landkartenartige Nekrose, Vaskulitis) selten.…”

Section: Diagnostikunclassified

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S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

Schirmer

Moosig²

2017

Z Rheumatol

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“…Patients with limited GPA do not have rapidly progressive glomerulonephritis or other organ-threatening disease manifestations. Limited GPA commonly presents with granulomatous inflammation and encompasses the 5-10% of patients with localized disease confined to the upper airway without other evidence of systemic vasculitic manifestations (5). The granulomatous lesions of GPA, such as those found in the ENT domain, may be accompanied by evidence of small vessel vasculitis in approximately onethird to one-half of biopsies.…”

Section: Introductionmentioning

confidence: 99%

Effectiveness of Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis (Wegener's)

Lally

Lebovics

Huang

et al. 2014

Arthritis Care & Research

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Objective. Ear, nose, and throat (ENT) involvement is the most prevalent manifestation of granulomatosis with polyangiitis (Wegener's) (GPA) and correlates with permanent damage and decreased quality of life. Although prior studies have evaluated the efficacy of rituximab (RTX) for granulomatous features of GPA, none have evaluated its efficacy solely for ENT manifestations. We compared the effectiveness of RTX to other therapies for the ENT manifestations of GPA in a large, well-characterized cohort. Methods. We performed a retrospective analysis of 975 visits from 99 GPA patients seen at a tertiary care ENT practice between 2003 and 2013. At each visit, subjects had a complete ENT examination, with ENT activity assessed by a single expert otolaryngologist. ENT disease activity during the observational period in subjects receiving RTX was compared to subjects receiving all other therapy. Results. In total, 48 subjects had never received RTX and 51 received RTX at least once. There was no active ENT disease during 92.4% of the observational period (days) for subjects receiving RTX, compared with 53.7% of the observational period for subjects not receiving RTX (odds ratio 11.0 [95% confidence interval 5.5-22.0], P < 0.0001). Subjects receiving RTX, compared with those receiving methotrexate, azathioprine, cyclophosphamide, or trimethoprim-sulfamethoxazole, were significantly more likely to have no active ENT disease (P < 0.0001 for each comparison). Conclusion. RTX is an effective treatment for ENT manifestations of GPA. Subjects treated with RTX were significantly less likely to have active ENT disease compared with those not receiving RTX. Patients being treated with RTX were 11 times less likely to have active ENT disease than patients being treated with other therapies.

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Prospective long-term follow-up of patients with localised Wegener's granulomatosis: does it occur as persistent disease stage?

Abstract: There is evidence that locWG is a long-term disease stage or phenotype (5% of all patients with WG), 46% of whom are ANCA-positive. LocWG is characterised by destructive and/or space-consuming lesions associated with high relapse rates (46%) and local damage.

Cited by 195 publications

References 31 publications

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

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

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

Effectiveness of Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis (Wegener's)

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