Churg–Strauss Syndrome in Childhood: A Systematic Literature Review and Clinical Comparison with Adult Patients

Zwerina, Jochen; Eger, G.; Englbrecht, Matthias; Manger, Bernhard; Schett, Georg

doi:10.1016/j.semarthrit.2008.05.004

Cited by 125 publications

(113 citation statements)

References 33 publications

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“…Regarding serum ANCA positivity, relatively low frequency in children with CSS, about 25%, were reported to be positive for serum ANCA (Zwerina et al 2008), which is different from that of adults with CSS. This discrepancy suggests that the possible pathogenetic heterogeneity of CSS may exist between children and adults, although this remains to be examined in future studies.…”

Section: Discussionmentioning

confidence: 77%

“…It has been reported that childhood CSS is rare, and the features of the disease in children have not been fully understood (Ikemoto et al 2001;Kobayashi et al 2003;Boyer et al 2006;Zwerina et al 2008;Kawakami and Soma 2009;Mutsaers et al 2009;Domircin et al 2010;Salerno et al 2010). Regarding serum ANCA positivity, relatively low frequency in children with CSS, about 25%, were reported to be positive for serum ANCA (Zwerina et al 2008), which is different from that of adults with CSS.…”

Section: Discussionmentioning

confidence: 95%

mentioning

confidence: 99%

“…It has been reported that pulmonary, cardiac, and gastrointestinal involvements are predominant in pediatric CSS cases compared to that seen in adult cases, and these clinical signs are hallmarks of a worse prognosis (Boyer et al 2006;Zwerina et al 2008). Since CSS is an uncommon disease entity in children, the treatment of pediatric patients with CSS, especially in severe cases, remains challenging (Ikemoto et al 2001;Boyer et al 2006;Zwerina et al 2008;Domircin et al 2010). Regarding the etiology of CSS, some case reports have suggested an association between the onset of CSS and the use of leukotriene modifiers for the treatment of pre-existing asthma (Kobayashi et al 2003;Boyer et al 2006;Salerno et al 2010), although this remains speculative (Harrold 2007).…”

mentioning

confidence: 99%

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Successful Multidrug Treatment of a Pediatric Patient with Severe Churg-Strauss Syndrome Refractory to Prednisolone

Watanabe

Aizawa-Yashiro

Tsuruga

et al. 2011

Tohoku J. Exp. Med.

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Section: Discussionmentioning

confidence: 77%

Section: Discussionmentioning

confidence: 95%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Successful Multidrug Treatment of a Pediatric Patient with Severe Churg-Strauss Syndrome Refractory to Prednisolone

Watanabe

Aizawa-Yashiro

Tsuruga

et al. 2011

Tohoku J. Exp. Med.

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“…It usually presents between 40 and 60 years of age, with a mean age onset of 49 years [19]. Although pediatric cases have also been described, no clear gender predominance, family clustering, or ethnic predisposition has been clearly found [20,21].…”

Section: Epidemiologymentioning

confidence: 99%

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

et al. 2015

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Eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA) is a systemic small-to-medium-sized vasculitis associated with asthma and eosinophilia. Histologically EGPA presents tissue eosinophilia, necrotizing vasculitis, and granulomatous inflammation with eosinophil tissue infiltration. EGPA commonly involves the upper airway and lung parenchyma, peripheral neuropathy, cardiac disorders, and skin lesions. The anti-neutrophil cytoplasmic antibodies (ANCA) are positive in 40% of cases, especially in those patients with clinical signs of vasculitis. The pathogenesis of EGPA is multifactorial. The disease can be triggered by exposure to a variety of allergens and drugs, but a genetic background has also been described, particularly an association with HLA-DRB4. Th2 response is of special importance in the upregulation of different interleukins such as IL-4, IL-13, and IL-5. Th1 and Th17 responses are also of significance. Activated eosinophils have a prolonged survival and probably cause tissue damage by releasing eosinophil granule proteins, while their tissue recruitment can be regulated by chemokines such as eotaxin-3 and CCL17. Humoral immunity is also OPEN ACCESSSinusitis 2016, 1 25 abnormally regulated, as demonstrated by excessive responses of IgG4 and IgE. EGPA has a good respond to glucocorticoids, although the combination of glucocorticoids and immunosuppressants (e.g., cyclophosphamide, azathioprine) is needed in most of cases. Newer treatment options include anti-IL-5 antibodies (mepolizumab), whose efficacy has been described in clinical trials, and anti-CD-20, a B cell-depleting agent (rituximab), reported in several case series.

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Mepolizumab Treatment of Pediatric Eosinophilic Granulomatosis With Polyangiitis

et al. 2018

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Churg–Strauss Syndrome in Childhood: A Systematic Literature Review and Clinical Comparison with Adult Patients

Cited by 125 publications

References 33 publications

Successful Multidrug Treatment of a Pediatric Patient with Severe Churg-Strauss Syndrome Refractory to Prednisolone

Successful Multidrug Treatment of a Pediatric Patient with Severe Churg-Strauss Syndrome Refractory to Prednisolone

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

Mepolizumab Treatment of Pediatric Eosinophilic Granulomatosis With Polyangiitis

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