Churg–Strauss syndrome: retrospective study in Burgundian population in France in past 10 years

Vinit, J.; Müller, G.; Bielefeld, P.; Pfitzenmeyer, P.; Bonniaud, Philippe; Lorcerie, B.; Besancenot, J.-F.

doi:10.1007/s00296-009-1275-y

Cited by 17 publications

(4 citation statements)

References 22 publications

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“…This trend of increasing EGPA prevalence in Japan has continued between 2017 and 2020 [ 34 ]. This is consistent with previous studies in Australia and France, which showed two- to three-fold increases in EGPA prevalence over 8–10 years from the late-1990s to mid-2000s, but with little change in incidence, although both studies were small with only eight and 31 EGPA cases identified, respectively [ 35 , 36 ]. However, the previously mentioned systematic review and meta-analysis study reported no strong trends for increasing EGPA prevalence over time [ 14 ].…”

Section: Discussionsupporting

confidence: 92%

Prevalence, incidence and healthcare burden of eosinophilic granulomatosis with polyangiitis in the UK

Hwee,

Harper,

et al. 2024

ERJ Open Res

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BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is a rare but serious disease characterised by the combination of small-to-medium vessel vasculitis, blood and tissue eosinophilia, and asthma and/or sino-nasal disease. This study estimated the prevalence and incidence of diagnosed EGPA in the UK, and described the demographics, clinical characteristics, and healthcare resource utilisation (HCRU) of this population.MethodsThis retrospective longitudinal study of patients with newly diagnosed EGPA (index) (2005–2019) used the Clinical Practice Research Datalink AURUM and Hospital Episode Statistics databases. The primary outcomes were the annual prevalence (2005–2019) and incidence (2006–2019) of EGPA, and secondary outcomes included patient demographics and clinical characteristics, and HCRU in the year pre- and post-index (diagnosis).ResultsPopulations of patients with EGPA comprised 940 prevalent cases and 502 incident cases of which 377 were linked to Hospital Episode Statistics. EGPA prevalence increased from 22.7 to 45.6 cases per 1 000 000 (2005–2019), driven by patients aged ≥18 years. Incidence ranged from 2.3 to 4.0 per 1 000 000 person-years (2006–2019). Pre-index, the most common clinical symptoms were respiratory related, and the most common comorbidities were asthma (80.6%) and nasal polyps (32.1%). Post-index, 19.1% had an EGPA-related inpatient stay (median length of stay: 11.0 days) and 38.7% had ≥5 oral corticosteroid (OCS) prescriptions with a mean OCS possession ratio per patient of 47.0%.ConclusionsAlthough EGPA incidence in the UK remains relatively stable, prevalence is increasing, and HCRU and OCS use remain frequent, suggesting considerable healthcare burden for patients with EGPA.

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Section: Discussionsupporting

confidence: 92%

Prevalence, incidence and healthcare burden of eosinophilic granulomatosis with polyangiitis in the UK

Hwee,

Harper,

et al. 2024

ERJ Open Res

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“…In addition, most patients will have elevated titers of perinuclear (p-) ANCA (5). Anti-myeloperoxidase activity (MPO-ANCA) level may also be elevated (6). Furthermore, ANCA, specifically MPO-ANCA, is a valuable adjunct in supporting the diagnosis of CSS yet is not useful for monitoring disease activity (7).…”

Section: Discussionmentioning

confidence: 99%

Differential Diagnosis of Churg-Strauss Syndrome with Tingling Sensation and Weakness of the Lower Extremities

2018

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IntroductionChurg-Strauss syndrome (CSS; or eosinophilic granulomatosis with polyangiitis) is an extremely rare autoimmune condition characterized by hypereosinophilia and vasculitis of small or medium-sized blood vessels, occurring in patients with asthma and allergic rhinitis.Case PresentationA 47-year-old man (height, 175.8 cm and weight, 74.8 kg) complained of a tingling sensation in his leg. He had a history of hypertension and asthma in the previous two years, and history of sinus surgery for sinusitis in the previous one year. He showed weight loss of 6 kg during the last month, and the signal intensity of the bone marrow on magnetic resonance imaging was diffusively reduced to be less than or equal to the disc. These findings indicated a hematologic disease and the possibility of malignancy. However, a nerve conduction study suggested multiple mononeuropathies. In addition, his laboratory findings showed peripheral eosinophilia and positivity for myeloperoxidase anti-neutrophil cytoplasmic autoantibody. Therefore, CSS was strongly suspected.ConclusionsChurg-Strauss syndrome is considered to be a condition with variable presentation and is difficult to diagnose because it has various clinical symptoms and disease progression. This study reports on a case with CSS, who underwent nerve biopsy and cholecystectomy for pathological confirmation because of various symptoms, and early diagnosis was difficult.

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“…The incidence has been reported to be 0.9 to 2 per million person-years and the prevalence 11.3 per million personyears. [37][38][39][40][41] In those with asthma, the incidence is estimated to be 35 to 67 cases per million person-years. 42,43 There is also an increased incidence associated with age greater than 50 years.…”

Section: Epidemiologymentioning

confidence: 99%

Current Approach to Diagnosis and Management of Pulmonary Eosinophilic Syndromes: Eosinophilic Pneumonias, Eosinophilic Granulomatosis with Polyangiitis, and Hypereosinophilic Syndrome

Sergew

Pérez

2016

Semin Respir Crit Care Med

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Eosinophils play a key role in orchestrating the complex clinicopathological pulmonary and extrapulmonary disease features in patients with eosinophilic syndromes. Eosinophilic pulmonary syndromes consist of a heterogeneous group of disorders characterized by the presence of peripheral blood eosinophilia and/or eosinophilic-related pulmonary impairment. These disorders can present with varying degrees of organ involvement, and while their presentation may be similar, it is important to define the disease state, as management and prognosis differ. In this article, we discuss acute and chronic eosinophilic pneumonias, eosinophilic granulomatosis with polyangiitis, and the hypereosinophilic syndromes. The mainstay of therapy for these disorders has been corticosteroids; however, recent and ongoing studies have provided strong grounds for optimism for specific targeted treatment approaches.

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Churg–Strauss syndrome: retrospective study in Burgundian population in France in past 10 years

Cited by 17 publications

References 22 publications

Prevalence, incidence and healthcare burden of eosinophilic granulomatosis with polyangiitis in the UK

Prevalence, incidence and healthcare burden of eosinophilic granulomatosis with polyangiitis in the UK

Differential Diagnosis of Churg-Strauss Syndrome with Tingling Sensation and Weakness of the Lower Extremities

Current Approach to Diagnosis and Management of Pulmonary Eosinophilic Syndromes: Eosinophilic Pneumonias, Eosinophilic Granulomatosis with Polyangiitis, and Hypereosinophilic Syndrome

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