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

Autor: Frank Moosig, Julia U Holle, Eva Reinhold-Keller, Kristine Herrmann, Jan P Bremer, Martin Laudien, Jan H. Schirmer, Marvin N. Wright, Bernhard Nölle
Rok vydání: 2016
Předmět:
Male
Eye Diseases
Gastroenterology
0302 clinical medicine
immune system diseases
Proteinase 3
Recurrence
Germany
Immunology and Allergy
030212 general & internal medicine
skin and connective tissue diseases
Peripheral Nervous System Diseases
Middle Aged
Survival Rate
Otorhinolaryngologic Diseases
Cohort
Female
Kidney Diseases
Microscopic polyangiitis
Vasculitis
Granulomatosis with polyangiitis
Rituximab
Immunosuppressive Agents
Adult
medicine.medical_specialty
Adolescent
Myeloblastin
Immunology
Antibodies
Antineutrophil Cytoplasmic
03 medical and health sciences
Young Adult
Age Distribution
stomatognathic system
Rheumatology
Internal medicine
medicine
Humans
Immunologic Factors
cardiovascular diseases
Survival rate
Cyclophosphamide
Anti-neutrophil cytoplasmic antibody
Aged
Peroxidase
Proportional Hazards Models
Retrospective Studies
030203 arthritis & rheumatology
business.industry
Granulomatosis with Polyangiitis
Retrospective cohort study
Laryngostenosis
medicine.disease
respiratory tract diseases
Case-Control Studies
business
Zdroj: Arthritisrheumatology (Hoboken, N.J.). 68(12)
ISSN: 2326-5205
Popis: Objective To compare the phenotype, clinical course, and outcome of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA)-positive granulomatosis with polyangiitis (Wegener's) (GPA) to proteinase 3 (PR3)-ANCA-positive GPA and to MPO-ANCA-positive microscopic polyangiitis (MPA). Methods We characterized all MPO-ANCA-positive patients classified as having GPA by the European Medicines Agency algorithm who attended our center, in a retrospective chart review. A second cohort of patients with PR3-ANCA-positive GPA matched for age and sex was characterized. Patients with MPO-ANCA-positive MPA from a recently published cohort were also included in the analysis. All patients were diagnosed and treated according to a standardized interdisciplinary approach at a vasculitis referral center. Results Comprehensive data were available for 59 patients with MPO-ANCA-positive GPA, and they were compared to 118 patients with PR3-ANCA-positive GPA and 138 patients with MPO-ANCA-positive MPA. We observed a distinct phenotype in MPO-ANCA-positive GPA as compared to the other 2 cohorts. Patients with MPO-ANCA-positive GPA frequently had limited disease without severe organ involvement, had a high prevalence of subglottic stenosis, and had less need for aggressive immunosuppressive therapy (cyclophosphamide/rituximab). The patients with MPO-ANCA-positive GPA were also younger than the MPA patients and were predominantly female (significantly different than the MPA cohort). While GPA patients had higher survival rates compared to MPA patients (due to a high prevalence of pulmonary fibrosis in MPA), patients with MPO-ANCA had significantly lower relapse rates than those with PR3-ANCA. Conclusion Patients with MPO-ANCA-positive GPA show significantly different clinical courses compared to those with PR3-ANCA-positive GPA or MPO-ANCA-positive MPA, which should be considered in their clinical management. Classification according to ANCA specificity may improve the evaluation of relapse risk.
Databáze: OpenAIRE
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