Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg Strauss syndrome within a defined population in southern Sweden

Mohammad, Aladdin J; Jacobsson, Lennart; Mahr, Alfred; Sturfelt, Gunnar; Segelmark, Mårten

doi:10.1093/rheumatology/kem107

Cited by 236 publications

(127 citation statements)

References 22 publications

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“…Furthermore, the interval from first symptoms to diagnosis declined from 8 months in cohort 1 to 4 months in cohort 3, which resulted in a reduced extent of disease at diagnosis. With regard to organ manifestations at diagnosis, the present study showed frequencies of organ involvement similar to those in the 2 population-based studies from Scandinavia (37,38) and confirmed the findings of Takala et al (38) regarding (nearly) unchanged organ manifestations at diagnosis and a rise in mean age at diagnosis. Furthermore, a declining interval from occurrence of symptoms to diagnosis has also been found by Eriksson et al (7) and Takala et al (38).…”

Section: Discussionsupporting

confidence: 91%

Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades

Holle

Gross

Latza

et al. 2010

Arthritis & Rheumatism

250

143

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Objective. 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. The historical cohort (cohort 1) included 155 patients diagnosed between 1966 and 1993, cohort 2 included 123 patients diagnosed between 1994 and 1998, and cohort 3 included 167 patients diagnosed between 1999 and 2002.Results. Over time, the interval between first symptoms and diagnosis was reduced by half (from 8 months to 4 months). Organ manifestations were similar in the 3 cohorts, and more than 80% of patients still required cyclophosphamide (CYC); however, the median cumulative dose was reduced significantly (from 67 gm in cohort 1 to 36 gm in cohort 2 and to 24 gm in cohort 3). The standardized mortality ratios ( Conclusion. Mortality of WG patients declined over the last 4 decades, probably due to improved diagnostic and therapeutic procedures and increased awareness of WG, which led to earlier diagnosis and therapy, reduction in relapse rates, and lower cumulative CYC dose with fewer deaths related to therapy.

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Section: Discussionsupporting

confidence: 91%

Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades

Holle

Gross

Latza

et al. 2010

Arthritis & Rheumatism

250

143

View full text Add to dashboard Cite

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“…The incidence of MPA is 2,7 to 94 per 1 million with a slight male predominance [14]. However, other data do not correlate the incidence of MPA with the latitude [1,15,16]. The average age of onset is between 50 and 60 years [13,17,18].…”

Section: Historymentioning

confidence: 99%

Microscopic Polyangiitis: from Pathogenesis to Treatment

Dousdampanis¹,

Assimakopoulos²,

Trigka³

2017

UNOAJ

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Microscopic Polyangiitis (MPA) is a systemic pauci-immune necrotizing vasculitis of small-calibre vessels characterized by the absence of granulomas. MPA is an autoimmune disease but its aetiology remains obscure. MPA is associated with presence of antineutrophil cytoplasmatic autoantibodies (ANCAs) but not all patients with MPA have ANCAs. There is strong evidence that not all ANCAs are pathogenetic. It seems that specific epitopes determine ANCAs pathogenicity. Given that MPA is a systemic vasculitis, multiple organs could be affected resulting in a wide spectrum of signs and symptoms. The kidneys and lungs are the most typical organs involved in MPA. Notably, MPA is the major cause of pulmo-renal syndrome. MPA has a poor prognosis if not treated but the use of aggressive immunosuppressive treatment has improved the prognosis and the patient's survival. Rituximab could be considered as an alternative treatment for severe disease, for patients who do not respond adequately to the immunosuppressive treatment and for patients with relapses. However, the long-term safety of Rituximab in MPA is unknown and it should be elucidated by further studies. Interestingly, kidney transplantation is safe and effective and it has a good prognosis in MPA patients with ESRD.

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“…The incidence of WG is approximately 8 per million of the population and the prevalence 160 per million [139,141,142]. Caucasians are more frequently affected than other race/ethnicities, and men are affected more often than women.…”

Section: Wegener's Granulomatosismentioning

confidence: 99%

“…The ANCAs in MPA are usually directed toward MPO [140]. The incidence of MPA is approximately 4 per million of the population and the prevalence is 94 per million [139,142]. As in WG, neurological involvement in MPA usually manifests as peripheral neuropathy and mononeuritis multiplex [153].…”

Section: Microscopic Polyangiitismentioning

confidence: 99%

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Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Geldern¹,

McPharlin

Becker

2012

Neurotherapeutics

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This chapter will review the spectrum of immunemediated diseases that affect the nervous system and may result in an admission to the neurological intensive care unit. Immunomodulatory strategies to treat acute exacerbations of neurological diseases caused by aberrant immune responses are discussed, but strategies for long-term immunosuppression are not presented. The recommendations for therapeutic intervention are based on a synthesis of the literature, and include recommendations by the Cochrane Collaborative, the American Academy of Neurology, and other key organizations. References from recent publications are provided for the disorders and therapies in which randomized clinical trials and large evidenced-based reviews do not exist. The chapter concludes with a brief review of the mechanisms of action, dosing, and side effects of commonly used immunosuppressive strategies in the neurocritical care unit.

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Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg Strauss syndrome within a defined population in southern Sweden

Abstract: The prevalence of WG, MPA, PAN and CSS in our district is the highest figure reported so far. Explanations for this finding may include high incidence, extensive ANCA-testing, good survival as well as sensitive search methods for case identification.

Cited by 236 publications

References 22 publications

Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades

Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades

Microscopic Polyangiitis: from Pathogenesis to Treatment

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

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