Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension
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Date
2015-06-01Source Title
Respirology Case ReportsPublisher
WILEYAffiliation
Florey Department of Neuroscience and Mental HealthMetadata
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Journal ArticleCitations
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 AccessDOI
10.1002/rcr2.104Abstract
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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