Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension
AuthorEllender, CM; McLean, C; Williams, TJ; Snell, GI; Whitford, HM
Source TitleRespirology Case Reports
University of Melbourne Author/sMcLean, Catriona
AffiliationFlorey Department of Neuroscience and Mental Health
Document TypeJournal Article
CitationsEllender, 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 StatusOpen Access
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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