Literature DB >> 29189887

Eosinophilic granulomatosis with polyangiitis without respiratory symptoms or asthma in an adolescent: case report and literature review.

Gülçin Otar Yener1, Zahide Ekici Tekin1, Neşe Çallı Demirkan2, Selçuk Yüksel3.   

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain. He had eosinophilia, and electroneuromyography demonstrated sensorimotor polyneuropathy. His skin biopsy revealed necrotizing eosinophilic vasculitis and eosinophilic panniculitis. Although he had no respiratory symptoms or history of asthma, prominent pulmonary involvement was evident on thoracic MRI. After treatment, his complaints of pain improved but mild neuropathy persisted. After 4 years of follow-up, he had minimal hypoesthesia in his right hand but had not experienced any relapses. This case highlights the fact that in cases suspected of EGPA, even without respiratory symptoms or asthma, detailed imaging should be performed for a definitive diagnosis. In addition, mild neurological findings may persist despite treatment in EGPA. The relevant literature on EGPA, with specific reference to pediatric cases, is reviewed.

Entities:  

Keywords:  Asthma; Eosinophilia; Polyneuropathy; Vasculitis

Mesh:

Substances:

Year:  2017        PMID: 29189887     DOI: 10.1007/s00296-017-3884-1

Source DB:  PubMed          Journal:  Rheumatol Int        ISSN: 0172-8172            Impact factor:   2.631


  22 in total

1.  Allergic granulomatosis, allergic angiitis, and periarteritis nodosa.

Authors:  J CHURG; L STRAUSS
Journal:  Am J Pathol       Date:  1951 Mar-Apr       Impact factor: 4.307

2.  Churg-Strauss Syndrome in childhood: a clinical review.

Authors:  Tamihiro Kawakami; Yoshinao Soma
Journal:  J Rheumatol       Date:  2009-11       Impact factor: 4.666

Review 3.  Eosinophilia in Rheumatologic/Vascular Disorders.

Authors:  Hiromichi Tamaki; Soumya Chatterjee; Carol A Langford
Journal:  Immunol Allergy Clin North Am       Date:  2015-06-17       Impact factor: 3.479

4.  Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort.

Authors:  Cloé Comarmond; Christian Pagnoux; Mehdi Khellaf; Jean-François Cordier; Mohamed Hamidou; Jean-François Viallard; François Maurier; Stéphane Jouneau; Boris Bienvenu; Xavier Puéchal; Olivier Aumaître; Guillaume Le Guenno; Alain Le Quellec; Ramiro Cevallos; Olivier Fain; Bertrand Godeau; Raphaèle Seror; Bertrand Dunogué; Alfred Mahr; Philippe Guilpain; Pascal Cohen; Achille Aouba; Luc Mouthon; Loïc Guillevin
Journal:  Arthritis Rheum       Date:  2013-01

Review 5.  Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.

Authors:  J G Lanham; K B Elkon; C D Pusey; G R Hughes
Journal:  Medicine (Baltimore)       Date:  1984-03       Impact factor: 1.889

Review 6.  Eosinophilia-myalgia syndrome, toxic-oil syndrome, and diffuse fasciitis with eosinophilia.

Authors:  L D Kaufman; L B Krupp
Journal:  Curr Opin Rheumatol       Date:  1995-11       Impact factor: 5.006

Review 7.  Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients.

Authors:  Jochen Zwerina; Gerhard Eger; Matthias Englbrecht; Bernhard Manger; Georg Schett
Journal:  Semin Arthritis Rheum       Date:  2008-07-17       Impact factor: 5.532

8.  Churg-Strauss syndrome presenting as an abdominal mass in a non-asthmatic child.

Authors:  Mohamed A Hamdan; Sareea Al-Rumaithi; Naser Tolaymat; Gabriel Ionescu; Moueid Al-Ashari; Hassan El-Teraifi
Journal:  Ann Trop Paediatr       Date:  2007-12

9.  2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.

Authors:  J C Jennette; R J Falk; P A Bacon; N Basu; M C Cid; F Ferrario; L F Flores-Suarez; W L Gross; L Guillevin; E C Hagen; G S Hoffman; D R Jayne; C G M Kallenberg; P Lamprecht; C A Langford; R A Luqmani; A D Mahr; E L Matteson; P A Merkel; S Ozen; C D Pusey; N Rasmussen; A J Rees; D G I Scott; U Specks; J H Stone; K Takahashi; R A Watts
Journal:  Arthritis Rheum       Date:  2013-01

10.  Churg-strauss syndrome without respiratory symptoms in a child.

Authors:  R B Basak; H Narchi; M Bakir; S Joshi; W Conca
Journal:  Indian J Dermatol       Date:  2011-01       Impact factor: 1.494

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