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A 42-Year-Old Woman with Progressive Limb Weakness and Breathing Difficulty

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A Case-Based Guide to Neuromuscular Pathology

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Abstract

Hydroxychloroquine, first introduced as an antimalarial drug in 1955, has been widely used along with corticosteroids and other immunomodulatory agents to treat a variety of connective tissue diseases. However, it affects lysosomal function which can result in the development of a vacuolar myopathy, characterized by the presence of autophagic rimmed vacuoles and distinctive curvilinear bodies and myeloid profiles. Patients with hydroxychloroquine myopathy often present with proximal limb weakness which can progress into diffuse weakness and respiratory failure. Muscle biopsy is essential to differentiate hydroxychloroquine myopathy from steroid myopathy and inflammatory myopathies associated with connective tissue diseases. Here we present a case with clinical presentation and muscle biopsy features consistent with hydroxychloroquine myopathy. The patient had a near-complete recovery 2 months after discontinuing hydroxychloroquine.

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Authors and Affiliations

  1. Departments of Neurology and Pathology, Boston University Medical Center, Boston, MA, USA

    Lan Zhou

  2. Department of Pathology, Neuropathology Section, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Dennis K. Burns

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  1. Lan Zhou
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  2. Dennis K. Burns
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Correspondence to Lan Zhou .

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Editors and Affiliations

  1. Departments of Neurology and Pathology, Boston University Medical Center, Boston, MA, USA

    Lan Zhou

  2. Department of Pathology, Neuropathology Section, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Dennis K. Burns

  3. Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Chunyu Cai

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Zhou, L., Burns, D.K. (2020). A 42-Year-Old Woman with Progressive Limb Weakness and Breathing Difficulty. In: Zhou, L., Burns, D., Cai, C. (eds) A Case-Based Guide to Neuromuscular Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-25682-1_19

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  • DOI: https://doi.org/10.1007/978-3-030-25682-1_19

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-25681-4

  • Online ISBN: 978-3-030-25682-1

  • eBook Packages: MedicineMedicine (R0)

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