Vasculitis mimics need to be differentiated from primary and secondary vasculitides as described in the Chapel Hill nomenclature. The clinical symptomatology resembles that of vasculitis of small and medium, rarely also large vessels and hence imitates the classical vasculitic disorders. Pathogenetically, the causes are partly genetic mutations, embolization syndromes, infections and substance abuse.
View Article and Find Full Text PDFObjective: 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.
Objective: To evaluate the clinical presentation and long-term outcome of a vasculitis centre cohort of patients with microscopic polyangiitis (MPA) with respect to organ manifestations, treatment, chronic damage and mortality.
Methods: We performed a retrospective chart review at our vasculitis referral centre. MPA patients admitted between 1991 and 2013 classified by a modified European Medicines Agency algorithm were diagnosed and treated according to a standardized interdisciplinary approach.
Z Rheumatol
November 2012
Granulomatosis with polyangitis (GPA, Wegener's granulomatosis) is characterized by a granulomatous inflammation of the respiratory tract and a necrotizing ANCA-associated small to medium-size vessel vasculitis with a predilection for the lungs (pulmonary capillaritis) and kidneys (necrotizing glomerulonephritis). The disease evolves stage-wise and typically starts as inflammation of the respiratory tract followed by development of systemic vasculitis manifestations. Today, treatment is evidence-based and adapted according to activity and disease stage which has resulted in a significant improvement in long-term outcome.
View Article and Find Full Text PDFCurr Pharm Des
February 2013
Granulumatosis with polyangiitis (wegener's)/GPA microscopic polyangiitis (MPA) and Churg Strauss syndrome (CSS) are primary systemic vasculitides which predominantly affect small vessels, showing a high association with a positive C/PR3-ANCA in GPA and P/MPO-ANCA in MPA, so called ANCA-associated vasculitides (AAV). The diagnostic work-up relies on an interdisciplinary approach including imaging techniques and laboratory tests in order to assess disease stage and extent. The golden standard remains the histological proof of a necrotizing, pauci-immune small vessel vasculitis, in GPA additionally non-caseating granuloma is found mainly in the respiratory tract.
View Article and Find Full Text PDFObjective: First, to investigate the overall efficacy and safety of rituximab (RTX) in refractory granulomatosis with polyangiitis (GPA) in a tertiary referral centre. Second, to compare the efficacy of RTX in granulomatous and vasculitic manifestations in GPA.
Patients And Methods: This study comprised a retrospective, standardised data collection from all patients who received RTX for refractory Wegener's granulomatosis from 2002 to 2010.
The outcome of ANCA (antineutrophil cytoplasmic antibody)-associated vasculitis (AAV) has been significantly improved due to the combined use of cyclophosphamide (CYC) and glucocorticosteroids. Recent studies demonstrated a normalization of life expectancy for several subgroups of AAV patients. Mortality is highest in the first year after diagnosis and infections are the most frequent cause of death.
View Article and Find Full Text PDFSince the introduction of combined immunosuppressive therapy consisting of oral cyclophosphamide (CYC) and glucocorticosteroids (GC) in the 1970s, the outcome of antineutrophil cystoplasmic antibodies (ANCA)-associated vasculitides has improved dramatically over the last decades. However, the long-term follow-up of patients treated with CYC plus GC has revealed a high treatment-related morbidity and mortality and a high proportion of patients suffering from relapses (up to 50%), requiring CYC and GC again. Methotrexate (MTX) can replace CYC for induction of remission in patients with a non life-threatening disease course of ANCA associated vasculitides ('early systemic').
View Article and Find Full Text PDFObjective: To determine the long-term outcome in patients with Wegener's granulomatosis (WG) over 4 decades in an academic hospital unit specializing in rheumatology.
Methods: We included 290 patients, divided them into 2 cohorts, and compared them with the historical cohort of 155 patients. Comparisons were retrospective regarding disease manifestations, therapy, mortality, and incidence of malignancies.
Objectives: Nasal colonisation with Staphylococcus aureus (S. aureus) has been implicated in Wegener's granulomatosis (WG) disease activity. In this study, the frequency of nasal colonisation with S.
View Article and Find Full Text PDFObjective: To identify patients with localised Wegener's granulomatosis (locWG) to assess whether it occurs as a long-term disease stage or phenotype and to characterise its outcome.
Methods: Patients in a 'localised stage' with histological criteria compatible with WG and a follow-up period of ≥1 year were included. They were prospectively followed at the Vasculitis Center Schleswig-Holstein from 1989 to 2009 and the clinical manifestations, antineutrophil cytoplasmic autoantibodies (ANCA) status and damage were evaluated.
Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are primary systemic small vessel vasculitides, associated with a positive C/PR3-ANCA in WG and P/MPO-ANCA in MPA. The most prominently involved organs are the upper (only in WG) and lower respiratory tract and the kidneys. The diagnostic work-up is an interdisciplinary approach assessing disease stage and extent.
View Article and Find Full Text PDFCyclophosphamide remains the therapy of choice in severe ANCA positive vasculitis. In order to avoid the considerable side effects of high cumulative doses sometimes manifesting themselves years later, induction therapy with a low cyclophosphamide dose and an early switch to cyclophosphamide-free maintenance regimens should be aimed for. This can be achieved by an induction therapy of 3--6 months of intravenous pulse therapy (pCYC) or oral therapy (oCYC).
View Article and Find Full Text PDFIntroduction: The authors present data on the incidence of primary systemic vasculitides (PSV) in the northern German state of Schleswig-Holstein from 1998 to 2005.
Methods: Population-based study of all new cases of PSV from 1 January 1998 onward in a region with a population of 2.83 million.
According to the literature it is known that structured standardized patient education represents an effective additional treatment in patients with chronic diseases. Positive effects in the reduction of disease activity and depression have been shown for rheumatoid arthritis, systemic lupus erythematodes, and diabetes mellitus. An interdisciplinary approach for providing information was developed for patients with primary systemic vasculitides (PSV) in the vasculitis center in Bad Bramstedt.
View Article and Find Full Text PDFObjectives: Results from open-label trials suggest that methotrexate (MTX) and leflunomide (LEF) are effective for maintenance of remission in Wegener's granulomatosis (WG), but data from randomized controlled clinical trails are not yet available.
Methods: In this multicentre, prospective randomized controlled clinical trial, patients with generalized WG were treated either with oral LEF 30 mg/day or oral MTX (starting with 7.5 mg/week reaching 20 mg/week after 8 weeks) for 2 yrs following induction of remission with cyclophosphamide.
A 53 year old male patient suffering from painful and swollen ankles showed a large enhancement on skeletal scintigraphy. The DXA bone density values for the neck of the femur and the lumbar vertebrae indicated severe osteoporosis. The conventional diagnostic x-ray revealed stress fractures of both distal tibiae (Looser's transformation zones).
View Article and Find Full Text PDFIn small vessel vasculitides, imaging studies aid in determining disease extent and activity, localization for biopsy, and for disease monitoring. They do not directly delineate the vasculitic lesion. Imaging studies focus on the upper and lower respiratory tract.
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