Wegener?s Granulomatosis

Lamprecht, Peter; Gross, Wolfgang L.

doi:10.1007/s00059-004-2525-0

Cited by 79 publications

(68 citation statements)

References 35 publications

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“…Upper respiratory tract (URT) disease occurs in 95% of patients with GPA [19]. URT was involved in 77.9% of patients in the present study, which was less than its rate as observed in other studies.…”

Section: Discussion ____________________________contrasting

confidence: 78%

Granulomatosis with polyangiitis (Wegener's granulomatosis): An analysis of 59 patients

Jokar

Mirfeizi

2017

Rheum Res

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Granulomatosis with polyangiitis (Wegener's granulomatosis) is a type of vasculitis which can be categorized as a subbranch of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. This study examined the demographic, clinical, radiographic and laboratory characteristics of patients with granulomatosis with polyangiitis (GPA) in Imam Reza Hospital, Mahshad, Iran. The medical records of patients admitted to the Department and Clinic of Rheumatology who were diagnosed with granulomatosis with polyangiitis between January 1, 2006 and December 31, 2016 were retrospectively studied. Patients were classified according to the American College of Rheumatology (ACR) 1990 classification criteria for Wegener's granulomatosis. A total of 59 GPA-diagnosed patients (55.9% male, 44.1% female) were identified. The male-tofemale ratio of the patients was 1.32:1. The mean age was 35.58±15.09 years, with the youngest and oldest patients being 10 and 71 years old, respectively. The mean disease duration was 47.5±32.88 months. The most common involved organs were the upper respiratory tract in 77.9%, lower respiratory tract in 62.7%, kidney in 59.3%, joint/muscle in 49.15%, and eye in 28.8% of patients. Four (6.7%) patients died during the follow-up which had a mean of 3.9 years. In the studied region, GPA inflicts the younger population, and it is more common in males. Instances of upper respiratory tract, lower respiratory tract, and kidney involvement are less common in the present study than in most other studies. GPA might have less severe manifestations in the studied area than in other regions.

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Section: Discussion ____________________________contrasting

confidence: 78%

Granulomatosis with polyangiitis (Wegener's granulomatosis): An analysis of 59 patients

Jokar

Mirfeizi

2017

Rheum Res

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“…3 Regarding the pathological description of GPA, the predominance of granulomatosis is obvious in the respiratory tract lesions, while small-vessel vasculitis principally concerns the pathogenesis of renal involvement. 8,25,26 Considering these indications, existence of granulomatous lesions may be required for the formation of HP in AAV. In fact, all patients with HP in the present study significantly indicated paranasal sinus involvement since the early phases of the illness, and a pulmonary nodular lesion was detected in one patient without paranasal sinus involvement.…”

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

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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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“…PR3 exists in both soluble and membrane-associated forms and is the principal autoantigen in the systemic vasculitic disease, Wegener's granulomatosis (6,7). After limited cleavage by PR3, IL-32α evidences enhanced biological activity, as evaluated via mouse macrophage inflammatory protein-2 (MIP-2) and human IL-8 induction (5).…”

Section: Introductionmentioning

confidence: 99%