Open AccessAutoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension
Author(s) -
Ellender Claire M.,
McLean Catriona,
Williams Trevor J.,
Snell Gregory I.,
Whitford Helen M.
Publication year - 2015
Publication title -
respirology case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.304
H-Index - 9
ISSN - 2051-3380
DOI - 10.1002/rcr2.104
Subject(s) - medicine , pulmonary hypertension , amyloidosis , lung transplantation , lung , pathology , histopathology , systemic disease , transplantation , disease
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.