Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension

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    Author
    Ellender, CM; McLean, C; Williams, TJ; Snell, GI; Whitford, HM
    Date
    2015-06-01
    Source Title
    Respirology Case Reports
    Publisher
    WILEY
    University of Melbourne Author/s
    McLean, Catriona
    Affiliation
    Florey Department of Neuroscience and Mental Health
    Metadata
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    Document Type
    Journal Article
    Citations
    Ellender, C. M., McLean, C., Williams, T. J., Snell, G. I. & Whitford, H. M. (2015). Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension. RESPIROLOGY CASE REPORTS, 3 (2), pp.78-81. https://doi.org/10.1002/rcr2.104.
    Access Status
    Open Access
    URI
    http://hdl.handle.net/11343/257369
    DOI
    10.1002/rcr2.104
    Abstract
    A 33-year-old woman with past history of Sjögren's syndrome and systemic lupus erythematosus presented with dyspnea and syncope secondary to pulmonary hypertension. After progressive symptoms over 4 years, she received bilateral lung transplantation. Histopathology of the explanted lungs showed isolated pulmonary amyloid light-chain amyloidosis and pulmonary cysts. No evidence of systemic amyloidosis was found at the time of transplantation. Seven years post lung transplantation, she remains well with no evidence of systemic amyloidosis recurrence.

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