Myeloperoxidase–Antineutrophil Cytoplasmic Antibody (ANCA)–Positive Granulomatosis With Polyangiitis (Wegener's) Is a Clinically Distinct Subset of ANCA‐Associated Vasculitis: A Retrospective Analysis of 315 Patients From a German Vasculitis Referral Center

Schirmer, Jan Henrik; Wright, Marvin N.; Herrmann, Kristine; Laudien, Martin; Nölle, Bernhard; Reinhold‐Keller, E.; Bremer, Jan Phillip; Moosig, Frank; Holle, Julia

doi:10.1002/art.39786

Cited by 98 publications

(96 citation statements)

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“…Recently, GPA patients with MPO-ANCA were reported to have different clinical features from those with PR3-ANCA or those without. 30 To identify the influence of ANCA types on the disease course of GPA, we divided 30 patients into MPO-ANCA (eight patients), PR3-ANCA (17 patients) and ANCA negative (five patients) groups according to ANCA types, and we compared variables between the two groups as described in Table S2. We found that gender and alveolar hemorrhage showed significant differences between patients with MPO-ANCA and PR3-ANCA.…”

mentioning

confidence: 99%

Birmingham vasculitis activity score of more than 9.5 at diagnosis is an independent predictor of refractory disease in granulomatosis with polyangiitis

et al. 2017

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mentioning

confidence: 99%

Birmingham vasculitis activity score of more than 9.5 at diagnosis is an independent predictor of refractory disease in granulomatosis with polyangiitis

et al. 2017

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“…Patients with MPO-ANCApositive GPA are predominantly females. They had limited disease, high incidence of subglottic stenosis, less need for immunosuppressive therapy, and lower relapse rates in comparison to patients with PR3-ANCA [63].…”

Section: Clinical Presentationmentioning

confidence: 98%

“…With the introduction of glucocorticoids and cyclophosphamide, the clinical course has become a chronic relapsing and remitting disease with remission noted in 90% of the patients. Increased risk for relapse is associated with PR3-ANCA positivity and lung or ENT involvement [63]. The ANCA titer often rises in active disease.…”

Section: Prognosis or Clinical Coursementioning

confidence: 99%

Vasculitis

Hoang

Park

2020

Hospital-Based Dermatopathology

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“…Spinal cord involvement represents a rare complication of granulomatosis with polyangiitis (GPA). [1][2][3] Hypertrophic pachymeningitis, as a possible manifestation, can arise in proteinase-3 antibody (anti-PR3) positive cases as well as in myeloperoxidase antibody (anti-MPO) associated GPA, whereas primary spinal angiitis seems to be a unique complication of anti-PR3 positive GPA. 4,5 Here, we report a case of long-term immunosuppressive treatment in a patient with anti-PR3 positive GPA, who developed an acute paraplegia caused by extensive pachymeningitis which despite treatment escalation only partially responded.…”

Section: Dear Editormentioning

confidence: 99%

Aggressive spinal cord involvement in granulomatosis with polyangiitis

et al. 2019

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Spinal cord involvement represents a rare complication of granulomatosis with polyangiitis (GPA). 1-3 Hypertrophic pachymeningitis, as a possible manifestation, can arise in proteinase-3 antibody (anti-PR3) positive cases as well as in myeloperoxidase antibody (anti-MPO) associated GPA, whereas primary spinal angiitis seems to be a unique complication of anti-PR3 positive GPA. 4,5 Here, we report a case of long-term immunosuppressive treatment in a patient with anti-PR3 positive GPA, who developed an acute paraplegia caused by extensive pachymeningitis which despite treatment escalation only partially responded.A 54-year-old Caucasian woman was diagnosed with anti-PR3 positive GPA in 2014 after presenting with bilateral conductive hearing loss and lower respiratory tract involvement (asymptomatic pulmonary nodules). After initiating an immunosuppressive treatment with prednisolone (10 mg/day) and methotrexate (15 mg/week), disease activity was sufficiently controlled for 2 years. In December 2016, the patient noticed slowly progressive neck and upper back pain, slight gait instability and intermittent numbness in both legs. In February 2017, she was admitted to our neurological ward due to acute urinary retention. Clinical examination showed nuchal rigidity, positive Kernig's and Brudzinski's signs and sensory ataxic gait disturbance in absence of leg paresis. Magnetic resonance imaging (MRI) showed a long contrast-enhanced swelling of the spinal meninges (C5-T8) combined with extensive spinal edema (Figure 1). Cerebrospinal fluid analysis showed elevated protein levels and an increased leukocyte count consistent with a spinal block. Further microbiological analyses did not show any pathological findings (Figure S1), but anti-PR3 antibodies were elevated. Histological examination of a meningeal biopsy (T3) revealed chronic granulating inflammation (Figure 2). We diagnosed a chronic hypertrophic spinal cord pachymeningitis and started intravenous steroid pulse therapy (1000 mg/day for 5 days) followed by initiation of intravenous cyclophosphamide treatment (800 mg) and an increase of oral prednisolone (50 mg/day). As a result, neurological deficits partly receded. At discharge the patient was able to walk small stretches without help. Urinary dysfunction as well as neck and upper back pain fully recovered. In April 2017, the patient was readmitted due to reoccurrence of gait instability and urinary dysfunction and severe neck and upper back pain. At hospitalization she presented with subtle paraparesis, which showed a significant deterioration within 1 day with painless leg paraplegia and sensory loss below T8. MRI showed a slightly progressive spinal edema (Figure 1) but did not reveal a definite cause for the acute deterioration. Immediate steroid pulse therapy (1000 mg/day for 5 days) and surgical spinal decompression were attempted without clinical improvement. Simultaneously, the patient developed sudden hemoptysis. A computed tomography scan revealed pulmonary granulomas with one large cavern, and the anti-P...

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Myeloperoxidase–Antineutrophil Cytoplasmic Antibody (ANCA)–Positive Granulomatosis With Polyangiitis (Wegener's) Is a Clinically Distinct Subset of ANCA‐Associated Vasculitis: A Retrospective Analysis of 315 Patients From a German Vasculitis Referral Center

Cited by 98 publications

References 45 publications

Birmingham vasculitis activity score of more than 9.5 at diagnosis is an independent predictor of refractory disease in granulomatosis with polyangiitis

Birmingham vasculitis activity score of more than 9.5 at diagnosis is an independent predictor of refractory disease in granulomatosis with polyangiitis

Vasculitis

Aggressive spinal cord involvement in granulomatosis with polyangiitis

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