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Eosinophilic granulomatosis with polyangiitis presenting with myositis: case based review

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Abstract

Eosinophilic granulomatosis with polyangitis (EGPA) is a systemic necrotizing small-vessel vasculitis that presents heterogeneously as a multi-organ disease. EGPA evolves through three phases: (1) prodromic phase with asthma, atopy and sinusitis, (2) eosinophilic phase characterized by peripheral eosinophilia and eosinophilic infiltration without necrosis, and (3) vasculitic phase involving organ damage. EGPA often presents with asthma, mononeuritis multiplex, lung infiltrates, sinusitis and constitutional symptoms. Although myalgias are common, EGPA rarely presents with true weakness with elevated creatinine kinase (CK). We describe a rare case of a patient presenting with eosinophilic myositis, who subsequently developed fulminant EGPA. The patient’s diagnosis was supported by an initial clinical presentation of weakness and elevated CK, followed by fleeting pulmonary infiltrates and mononeuritis multiplex, peripheral eosinophilia, and strongly positive myeloperoxidase anti-cytoplasmic antibody (MPO-ANCA). Muscle biopsy revealed eosinophilic myositis. The patient responded well to high-dose glucocorticoids and cyclophosphamide with improved symptoms and biochemical markers. Based on our literature review, there are only seven similar cases reported of EGPA presenting with myositis and confirmatory muscle biopsies. There is significant heterogeneity in their clinical findings, histopathology and treatments that were used. Our case report and literature review highlights the importance of recognizing myositis as an initial presenting symptom of EGPA, providing an opportunity for early diagnosis and treatment to reduce risk of further disease progression and morbidity.

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Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

  1. Division of Rheumatology, University of Toronto, Toronto, ON, Canada

    Sahil Koppikar, Ghaydaa Al-dabie, Dana Jerome & Ophir Vinik

  2. Division of Rheumatology, Women’s College Hospital, University of Toronto, 76 Grenville Street, Toronto, ON, M5S 1B2, Canada

    Sahil Koppikar & Dana Jerome

  3. Division of Rheumatology, St. Michael’s Hospital, Toronto, ON, Canada

    Ophir Vinik

Authors
  1. Sahil Koppikar
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  3. Dana Jerome
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Contributions

SK: writing of manuscript including editing and revisions, literature review at all stages of its production, and clinical management of patient. GA: writing of manuscript including editing and revisions, literature review at all stages of its production, and clinical management of patient. DJ: manuscript editing and revision at all stages of production, final approval of manuscript, and clinical management of patient. OV: manuscript editing and revision at all stages of production, final approval of manuscript, and clinical management of patient. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

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Correspondence to Sahil Koppikar.

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All the procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Written informed consent was obtained from this patient prior to submission of this article for consideration as a case-based review.

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Koppikar, S., Al-dabie, G., Jerome, D. et al. Eosinophilic granulomatosis with polyangiitis presenting with myositis: case based review. Rheumatol Int 40, 1163–1170 (2020). https://doi.org/10.1007/s00296-020-04567-8

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  • DOI: https://doi.org/10.1007/s00296-020-04567-8

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