Churg–Strauss syndrome: evidence for disease subtypes?

Pagnoux, Christian; Guillevin, Loı̈c

doi:10.1097/bor.0b013e328333390b

Cited by 115 publications

(69 citation statements)

References 107 publications

(88 reference statements)

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“…These ANCA-negative GPA patients most often have limited disease, although it may progress later on to a more severe and diffused form and sometimes become ANCA-positive. The ANCA-negative EGPA patients may more frequently have cardiac manifestations but less renal or peripheral nerve involvement (34)(35)(36)(37)(38).…”

Section: Clinical and Biological Findings Diagnosismentioning

confidence: 99%

Updates in ANCA-associated vasculitis

2016

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Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited ANCA-associated vasculitides can be considered the fourth entity. Despite their rarity and still unknown cause(s), research pertaining to ANCA-associated vasculitides has been very active over the past decades. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) has been supported using several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) has not been as strongly demonstrated. Moreover, some MPO-ANCA subsets, which are directed against a few specific MPO epitopes, have recently been found to be better associated with disease activity, but a different method than the one presently used in routine detection is required to detect them. B cells possibly play a major role in the pathogenesis because they produce ANCAs, as well as neutrophil abnormalities and imbalances in different T-cell subtypes [T helper (Th)1, Th2, Th17, regulatory cluster of differentiation (CD)4+ CD25+ forkhead box P3 (FoxP3)+ T cells] and/or cytokine-chemokine networks. The alternative complement pathway is also involved, and its blockade has been shown to prevent renal disease in an MPO-ANCA murine model. Other recent studies suggested strongest genetic associations by ANCA type rather than by clinical diagnosis. The induction treatment for severe granulomatosis with polyangiitis and microscopic polyangiitis is relatively well codified but does not (yet) really differ by precise diagnosis or ANCA type. It comprises glucocorticoids combined with another immunosuppressant, cyclophosphamide or rituximab. The choice between the two immunosuppressants must consider the comorbidities, past exposure to cyclophosphamide for relapsers, plans for pregnancy, and also the cost of rituximab. Once remission is achieved, maintenance strategy following cyclophosphamide-based induction relies on less toxic agents such as azathioprine or methotrexate. The optimal maintenance strategy following rituximab-based induction therapy remains to be determined. Preliminary results on rituximab for maintenance therapy appear promising. Efforts are still under way to determine the optimal duration of maintenance therapy, ideally tailored according to the characteristics of each patient and the previous treatment received.Keywords: Antineutrophil cytoplasmic antibody-associated vasculitis, granulomatosis with polyangiitis (Wegener's granulomatosis), microscopic polyangiitis, Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis), cyclophosphamide, rituximab IntroductionAntineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (GPA; formerly Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; al...

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Section: Clinical and Biological Findings Diagnosismentioning

confidence: 99%

Updates in ANCA-associated vasculitis

2016

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“…Endomyocardial involvement and lung infiltrates are more common in the ANCAnegative subset [20]. ANCA, with an immunofluorescence pattern usually consisting of P-ANCA and anti-MPO specificity, is present in up to 40% of EGPA patients, but only a minority of patients have cytoplasmic ANCA with antibodies to proteinase 3 [21][22][23]. In our study, only one of the 15 patients (6.6%) was positive for ANCA, and this patient was in the vasculitic phase.…”

Section: Discussionmentioning

confidence: 65%

“…Glucocorticoids and immunosuppressive treatments form the cornerstone of therapy for improved prognosis and survival rates of EGPA patients if they are given early [22,23]. The Five-Factor Score (FFS) is usually used to determine prognosis in EGPA, and patients with FFS ≥1 have a worse prognosis and higher mortality [17,24].…”

Section: Discussionmentioning

confidence: 99%

Clinical and Serological Features of Eosinophilic and Vasculitic Phases of Eosinophilic Granulomatosis with Poliangiitis: a Case Series of 15 Patients

Yılmaz¹,

Tutar²,

Şimşek³

et al. 2017

Turk Thorac J

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OBJECTIVES:Eosinophilic granulomatosis with poliangiitis (EGPA) which was previously called Churg-Strauss Syndrome, is classified into eosinophilic and vasculitic phases. To characterize the eosinophilic and vasculitic phases of the disease in terms of clinical findings, serology, and treatment. MATERIALS AND METHODS:We included 15 EGPA patients in the study. The clinical, serological, and therapeutic characteristics and the treatment responses of the patients were recorded. RESULTS:Thirteen patients were classified as being in the eosinophilic phase and two were classified as being in the vasculitic phase of EGPA. Initial symptoms were worsening asthma in all patients (n=15; 100%). All patients had rhinosinusitis, and 66.6% had hypersensitivity to nonsteroidal anti-inflammatory drugs. The two patients in the vasculitic phase did not have nasal polyposis. Pulmonary and nervous system involvement were the most common symptoms. The erythrocyte sedimentation rates (ESRs) of the two patients in the vasculitic phase were 65 mm/h and 55 mm/h, while ESR was normal in eosinophilic-phase patients. Antineutrophil cytoplasmic antibodies (ANCA) was detected in one patient (6.6%) who was in the vasculitic phase (Case 15). The disease was under control with higher doses of methylprednisolone in the vasculitic phase (Case 14: 12 mg/day, Case 15: 10 mg/day) than in the eosinophilic phase. Relapse was detected in the two patients in the vasculitic phase. Oral corticosteroid was not discontinued in any case, and no mortality was reported. CONCLUSION:Patients with eosinophilic phase or vasculitic phase EGPA had similar clinical onset. However, higher ESR, ANCA positivity, and extrapulmonary organ involvement were only found in patients in the vasculitic phase. Corticosteroid responsiveness was very good in all patients in the eosinophilic phase, and the disease could be controlled with a very low maintenance dose of a corticosteroid.

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“…Patients positive for ANCA are more likely to have systemic vasculitis affecting several organs, so it is not surprising that our patient was ANCA negative. 9 Ocular involvement in CSS is unusual; there have been reported cases with varied ocular manifestations. To the best of our knowledge, following a review of the published literature using key databases that include Medline and PubMed, this is the first reported case where there is bilateral simultaneous nodular involvement of the bulbar conjunctiva as part of a complex in the initial presentation of CSS.…”

Section: To the Editormentioning

confidence: 99%