Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension

Claire M. Ellender; Catriona McLean; Trevor J. Williams; Gregory I. Snell; Helen M. Whitford

doi:10.1002/rcr2.104

Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension

Claire M. Ellender, Catriona McLean, Trevor J. Williams, Gregory I. Snell, Helen M. Whitford

Medicine Alfred Hospital

Research output: Contribution to journal › Article › Other › peer-review

4 Citations (Scopus)

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.

Original language	English
Pages (from-to)	78-81
Number of pages	4
Journal	Respirology Case Reports
Volume	3
Issue number	2
DOIs	https://doi.org/10.1002/rcr2.104
Publication status	Published - 2015

Keywords

Amyloidosis
Pulmonary hypertension
Sjögren's syndrome

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10.1002/rcr2.104

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title = "Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension",

abstract = "A 33-year-old woman with past history of Sj{\"o}gren'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.",

keywords = "Amyloidosis, Pulmonary hypertension, Sj{\"o}gren's syndrome",

author = "Ellender, {Claire M.} and Catriona McLean and Williams, {Trevor J.} and Snell, {Gregory I.} and Whitford, {Helen M.}",

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AU - Ellender, Claire M.

AU - McLean, Catriona

AU - Williams, Trevor J.

AU - Snell, Gregory I.

AU - Whitford, Helen M.

PY - 2015

Y1 - 2015

N2 - 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.

AB - 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.

KW - Amyloidosis

KW - Pulmonary hypertension

KW - Sjögren's syndrome

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ER -

Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension

Abstract

Keywords

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