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Hypereosinophilic Syndrome - A Case with Churg Strauss Vasculitis

Received: 31 January 2017     Accepted: 21 February 2017     Published: 23 October 2017
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Abstract

The hypereosinophilic syndromes represent a group of disorders marked by the sustained overproduction of eosinophils, with eosinophilic infiltration of tissues and mediator release, leading to multiple organic damages. Churg-Strauss syndrome is a rare disorder characterized by systemic small vessel vasculitis, extra vascular granulomas and hypereosinophilia, characteristically occurring in people with background late-onset asthma and allergic rhinitis. Its annual incidence is between 0 and 4 per million of population. The authors present the case of a 72 year old Caucasian female patient, admitted at the Hygeia Hospital Tirana due to a hypereosinophilic syndrome. The thorax CT scan verified bilateral pseudo nodular pulmonary infiltrates; the head MRI showed vascular bilateral lesions in periventricular white matter and centrum semiovale, right internal capsule and left caudate nucleus, and the nerve conduction study (NCS) revealed a distal mixed polyneuropathy. A fibro-gastroscopy with biopsy suggested an atrophic gastritis. The patient underwent a course of treatment with intravenous methylprednisolone, tapered thereafter to oral prednisone together with cyclophosphamide, with a prompt improvement of the clinical picture.

Published in American Journal of Internal Medicine (Volume 5, Issue 5)
DOI 10.11648/j.ajim.20170505.14
Page(s) 83-85
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2017. Published by Science Publishing Group

Keywords

Hypereosinophilia, Churg-Strauss Syndrome, Vasculitis, Systemic Changes

References
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[2] Galli SJ, Goetzl EJ. Eosinophils, basophils, and mast cells. In: Handin RI, Stossel TP, Lux SE, Stossel TP, eds. Blood: Principles and Practice of Hematology. Baltimore, Md: Lippincott Williams & Wilkins; 1995, pp. 621-640.
[3] Klion AD, Bochner BS, Gleich GJ, Nutman TB, Rothenberg ME, Simon HU, et al. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol. 2006 Jun;117 (6): 1292-302.
[4] Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975 Jan; 54 (1): 1-27.
[5] Simon HU, Rothenberg ME, Bochner BS, Weller PF, Wardlaw AJ, Wechsler ME, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol. 2010 Jul. 126 (1): 45-9.
[6] Klion AD. Eosinophilic myeloproliferative disorders. Hematology Am Soc Hematol Educ Program. 2011; 2011: 257-63.
[7] Liao W, Long H, Chang CC, Lu Q. The Eosinophil in Health and Disease: from Bench to Bedside and Back. Clin Rev Allergy Immunol. 2016 Apr; 50 (2): 125-39.
[8] Valent P, Klion AD, Rosenwasser LJ, Arock M, Bochner BS, Butterfield JH, et al. ICON: Eosinophil Disorders. World Allergy Organ J. 2012 Dec; 5 (12): 174-81.
[9] Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis. 2006 May; 47 (5): 770-9.
[10] Pagnoux C. Churg-Strauss syndrome: evolving concepts. Discov Med. 2010 Mar; 9 (46): 243-52.
[11] Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990 Aug; 33 (8): 1094-100.
[12] Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M. Incidence and survival rates in Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford). 2009 Dec; 48 (12): 1560-5.
[13] Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005 Nov 1; 143 (9): 632-8.
[14] Gross WL, Reinhold-Keller E. Churg-Strauss Syndrome. Orphanet Encyclopedia. 2002, 1-7.
[15] Fallah-Tafti S, Mansouri D, Masjedi MR, Marashian M, Faridian D. Churg Strauss syndrome after introducing oral steroid to inhaler: a report of three cases. Iran J Allergy Asthma Immunol. 2006 Jun; 5 (2): 89-94.
[16] Semple D, Keogh J, Forni L, Venn R. Clinical review: Vasculitis on the intensive care unit -- part 2: treatment and prognosis. Crit Care. 2005 Apr; 9 (2): 193-7.
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  • APA Style

    Blerina Dhamo, Arben Pilaca, Gentian Vyshka. (2017). Hypereosinophilic Syndrome - A Case with Churg Strauss Vasculitis. American Journal of Internal Medicine, 5(5), 83-85. https://doi.org/10.11648/j.ajim.20170505.14

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    ACS Style

    Blerina Dhamo; Arben Pilaca; Gentian Vyshka. Hypereosinophilic Syndrome - A Case with Churg Strauss Vasculitis. Am. J. Intern. Med. 2017, 5(5), 83-85. doi: 10.11648/j.ajim.20170505.14

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    AMA Style

    Blerina Dhamo, Arben Pilaca, Gentian Vyshka. Hypereosinophilic Syndrome - A Case with Churg Strauss Vasculitis. Am J Intern Med. 2017;5(5):83-85. doi: 10.11648/j.ajim.20170505.14

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  • @article{10.11648/j.ajim.20170505.14,
      author = {Blerina Dhamo and Arben Pilaca and Gentian Vyshka},
      title = {Hypereosinophilic Syndrome - A Case with Churg Strauss Vasculitis},
      journal = {American Journal of Internal Medicine},
      volume = {5},
      number = {5},
      pages = {83-85},
      doi = {10.11648/j.ajim.20170505.14},
      url = {https://doi.org/10.11648/j.ajim.20170505.14},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajim.20170505.14},
      abstract = {The hypereosinophilic syndromes represent a group of disorders marked by the sustained overproduction of eosinophils, with eosinophilic infiltration of tissues and mediator release, leading to multiple organic damages. Churg-Strauss syndrome is a rare disorder characterized by systemic small vessel vasculitis, extra vascular granulomas and hypereosinophilia, characteristically occurring in people with background late-onset asthma and allergic rhinitis. Its annual incidence is between 0 and 4 per million of population. The authors present the case of a 72 year old Caucasian female patient, admitted at the Hygeia Hospital Tirana due to a hypereosinophilic syndrome. The thorax CT scan verified bilateral pseudo nodular pulmonary infiltrates; the head MRI showed vascular bilateral lesions in periventricular white matter and centrum semiovale, right internal capsule and left caudate nucleus, and the nerve conduction study (NCS) revealed a distal mixed polyneuropathy. A fibro-gastroscopy with biopsy suggested an atrophic gastritis. The patient underwent a course of treatment with intravenous methylprednisolone, tapered thereafter to oral prednisone together with cyclophosphamide, with a prompt improvement of the clinical picture.},
     year = {2017}
    }
    

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  • TY  - JOUR
    T1  - Hypereosinophilic Syndrome - A Case with Churg Strauss Vasculitis
    AU  - Blerina Dhamo
    AU  - Arben Pilaca
    AU  - Gentian Vyshka
    Y1  - 2017/10/23
    PY  - 2017
    N1  - https://doi.org/10.11648/j.ajim.20170505.14
    DO  - 10.11648/j.ajim.20170505.14
    T2  - American Journal of Internal Medicine
    JF  - American Journal of Internal Medicine
    JO  - American Journal of Internal Medicine
    SP  - 83
    EP  - 85
    PB  - Science Publishing Group
    SN  - 2330-4324
    UR  - https://doi.org/10.11648/j.ajim.20170505.14
    AB  - The hypereosinophilic syndromes represent a group of disorders marked by the sustained overproduction of eosinophils, with eosinophilic infiltration of tissues and mediator release, leading to multiple organic damages. Churg-Strauss syndrome is a rare disorder characterized by systemic small vessel vasculitis, extra vascular granulomas and hypereosinophilia, characteristically occurring in people with background late-onset asthma and allergic rhinitis. Its annual incidence is between 0 and 4 per million of population. The authors present the case of a 72 year old Caucasian female patient, admitted at the Hygeia Hospital Tirana due to a hypereosinophilic syndrome. The thorax CT scan verified bilateral pseudo nodular pulmonary infiltrates; the head MRI showed vascular bilateral lesions in periventricular white matter and centrum semiovale, right internal capsule and left caudate nucleus, and the nerve conduction study (NCS) revealed a distal mixed polyneuropathy. A fibro-gastroscopy with biopsy suggested an atrophic gastritis. The patient underwent a course of treatment with intravenous methylprednisolone, tapered thereafter to oral prednisone together with cyclophosphamide, with a prompt improvement of the clinical picture.
    VL  - 5
    IS  - 5
    ER  - 

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Author Information
  • Internal and Emergency Medicine Service, Hygeia Hospital, Tirana, Albania

  • Internal and Emergency Medicine Service, Hygeia Hospital, Tirana, Albania

  • Faculty of Medicine, University of Medicine, Tirana, Albania

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