Literature DB >> 28983650

[Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].

A Jerrentrup1, T Müller2, J Mariss3, S Dylla4.   

Abstract

This article presents the case of a patient with dyspnea, a history of poorly controlled asthma, sinonasal polyposis, blood eosinophilia and transient pulmonary infiltrates. The autoantibodies antinuclear antibodies, cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were negative. These symptoms are typical for eosinophilic granulomatosis with polyangiitis (EGPA, alternatively known as Churg-Strauss syndrome). The delay between onset and diagnosis in this case was several years. Although EGPA belongs to the spectrum of ANCA-associated vasculitis, less than 50% of EGPA patients are ANCA positive. Cardiac involvement (such as endomyocardial infiltration, arrhythmia and pericarditis) is the major cause of early death and a poor prognosis. In therapeutic regimens glucocorticoids and/or another immunosuppressant (e. g. cyclophosphamide, methotrexate or azathioprine) are used. The so-called five-factor score is a useful tool for assessment of prognosis.

Entities:  

Keywords:  Asthma; Autoantibodies; Eosinophilia; Immunosuppression; Vasculitis

Mesh:

Substances:

Year:  2018        PMID: 28983650     DOI: 10.1007/s00108-017-0303-3

Source DB:  PubMed          Journal:  Internist (Berl)        ISSN: 0020-9554            Impact factor:   0.743


  9 in total

1.  Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients.

Authors:  S A Worthy; N L Müller; D M Hansell; C D Flower
Journal:  AJR Am J Roentgenol       Date:  1998-02       Impact factor: 3.959

2.  Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management.

Authors:  Matthieu Groh; Christian Pagnoux; Chiara Baldini; Elisabeth Bel; Paolo Bottero; Vincent Cottin; Klaus Dalhoff; Bertrand Dunogué; Wolfgang Gross; Julia Holle; Marc Humbert; David Jayne; J Charles Jennette; Romain Lazor; Alfred Mahr; Peter A Merkel; Luc Mouthon; Renato Alberto Sinico; Ulrich Specks; Augusto Vaglio; Michael E Wechsler; Jean-François Cordier; Loïc Guillevin
Journal:  Eur J Intern Med       Date:  2015-05-09       Impact factor: 4.487

3.  The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort.

Authors:  Loïc Guillevin; Christian Pagnoux; Raphaele Seror; Alfred Mahr; Luc Mouthon; Philippe Le Toumelin
Journal:  Medicine (Baltimore)       Date:  2011-01       Impact factor: 1.889

4.  The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).

Authors:  A T Masi; G G Hunder; J T Lie; B A Michel; D A Bloch; W P Arend; L H Calabrese; S M Edworthy; A S Fauci; R Y Leavitt
Journal:  Arthritis Rheum       Date:  1990-08

5.  Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome.

Authors:  Sophia Kim; Gautham Marigowda; Eyal Oren; Elliot Israel; Michael E Wechsler
Journal:  J Allergy Clin Immunol       Date:  2010-06       Impact factor: 10.793

6.  Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis.

Authors:  Michael E Wechsler; Praveen Akuthota; David Jayne; Paneez Khoury; Amy Klion; Carol A Langford; Peter A Merkel; Frank Moosig; Ulrich Specks; Maria C Cid; Raashid Luqmani; Judith Brown; Stephen Mallett; Richard Philipson; Steve W Yancey; Jonathan Steinfeld; Peter F Weller; Gerald J Gleich
Journal:  N Engl J Med       Date:  2017-05-18       Impact factor: 91.245

7.  High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener's granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients.

Authors:  Y Allenbach; R Seror; C Pagnoux; L Teixeira; P Guilpain; L Guillevin
Journal:  Ann Rheum Dis       Date:  2008-11-17       Impact factor: 19.103

8.  Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

Authors:  Matthieu Groh; Gabriella Masciocco; Elizabeth Kirchner; Arnt Kristen; Carlo Pellegrini; Shaïda Varnous; Guillermo Bortman; Mark Rosenberg; Antonio Brucato; Paul Waterworth; Edgardo Bonacina; Fabio Facchetti; Leonard Calabrese; Gina Gregorini; Juan Jose Scali; Randall Starling; Maria Frigerio; Andrea Maria D'Armini; Loïc Guillevin
Journal:  J Heart Lung Transplant       Date:  2014-02-24       Impact factor: 10.247

9.  Survey of healthcare experiences of Australian adults living with rare diseases.

Authors:  Caron Molster; Debra Urwin; Louisa Di Pietro; Megan Fookes; Dianne Petrie; Sharon van der Laan; Hugh Dawkins
Journal:  Orphanet J Rare Dis       Date:  2016-03-24       Impact factor: 4.123

  9 in total
  1 in total

Review 1.  Cutaneous manifestations of acute kidney injury.

Authors:  Gavin A Esson; Amaani B Hussain; Simon J Meggitt; Nick J Reynolds; John A Sayer
Journal:  Clin Kidney J       Date:  2021-12-09
  1 in total

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