Renal Involvement in Churg-Strauss Syndrome

Sinico, Renato Alberto; Toma, Lucafrancesco Di; Maggiore, Umberto; Tosoni, Cinzia; Bottero, Paolo; Sabadini, Ettore; Giammarresi, Gaia; Tumiati, B; Gregorini, Gina; Pesci, Alberto; Monti, Stefano; Balestrieri, G.; Garini, G; Vecchio, F.; Buzio, Carlo

doi:10.1053/j.ajkd.2006.01.026

Cited by 170 publications

(112 citation statements)

References 34 publications

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“…It usually occurs in patients with asthma and eosinophilia, and has a heterogeneous clinical spectrum that includes constitutional symptoms, sinusitis, pulmonary infiltration, peripheral neuropathy, and skin (e.g., purpura, nodules), renal (e.g., isolated urinary abnormalities, rapidly progressive glomerulonephritis [RPGN]), and gastrointestinal manifestations (3)(4)(5)(6). Antineutrophil cytoplasmic antibodies (ANCAs) are present in ϳ40% of patients, usually in those developing clinical features resulting from active small-vessel vasculitis (e.g., RPGN, purpura) (7,8).…”

mentioning

confidence: 99%

HLA–DRB4 as a genetic risk factor for Churg‐Strauss syndrome

Vaglio

Martorana

Maggiore

et al. 2007

Arthritis & Rheumatism

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175

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Objective. To explore the association between HLA alleles and Churg-Strauss syndrome (CSS), and to investigate the potential influence of HLA alleles on the clinical spectrum of the disease.Methods. Low-resolution genotyping of HLA-A, HLA-B, and HLA-DR loci and genotyping of TNFA ؊238A/G and TNFA ؊308A/G single-nucleotide polymorphisms were performed in 48 consecutive CSS patients and 350 healthy controls.Results.

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mentioning

confidence: 99%

HLA–DRB4 as a genetic risk factor for Churg‐Strauss syndrome

Vaglio

Martorana

Maggiore

et al. 2007

Arthritis & Rheumatism

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175

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“…Early recognition and treatment of this disease may be important for preventing the development of life-threatening organ damage including end stage renal failure, thereby protecting quality of life and survival of patients with MPA. The presence of ANCA was associated with renal involvement and pulmonary haemorrhage, and to a lesser extent with vasculitis of the skin and mononeuritis multiplex [6]. Although rare, proteinuria may indicate the presence of MPA, hence early testing for ANCA may be warranted to exclude or support this possibility.…”

Section: Chance Finding Of Proteinuria Leadsmentioning

confidence: 99%

Chance Finding of Proteinuria Leads to the Diagnosis of ANCA Associated Vasculitis

Okamoto¹,

Sakurai²,

Kitahama³

et al. 2009

TOGMJ

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Microscopic polyangiitis (MPA) was recognised with a high early mortality rate and the renal involvement was characterised by segmental necrotizing glomerulonephritis with high positivity of antineutrophil cytoplasmic antibody (ANCA) and a broad spectrum of signs and symptoms including fever and malaise. We reported here a woman with ANCA associated with MPA diagnosed by a chance finding of proteinuria. To the best of our knowledge, early diagnosis of this disorder only by the presence of proteinuria has not been reported previously in the English literature.

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“…Purpura occurs in 25% of cases and usually involves the lower limbs. Nodules, urticaria, livedo, and skin ulcers [3,55] may also appear. Cardiac involvement frequently occurs at the vasculitic or generalized phase of the disease.…”

Section: Other Organs and Systemsmentioning

confidence: 99%

“…Glucocorticoids have dramatically changed the prognosis of the EGPA. Patients usually receive glucocorticoids before diagnosis, which may change the severity and histopathological lesions observed today [1,3].…”

Section: Introductionmentioning

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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Renal Involvement in Churg-Strauss Syndrome

Cited by 170 publications

References 34 publications

HLA–DRB4 as a genetic risk factor for Churg‐Strauss syndrome

HLA–DRB4 as a genetic risk factor for Churg‐Strauss syndrome

Chance Finding of Proteinuria Leads to the Diagnosis of ANCA Associated Vasculitis

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

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