Acute Interstitial Pneumonia (Hamman-Rich Syndrome) in Lung Transplantation : A Case Series
Copyright © 2022 Elsevier Inc. All rights reserved..
BACKGROUND: Acute interstitial pneumonia (AIP), also known as Hamman-Rich syndrome, is a rare and rapidly progressive idiopathic interstitial lung disease with a high mortality rate. Treatment is limited to supportive care and empirical high-dose steroids; however, outcomes are generally poor. There are few reports of lung transplantation (LTx) in patients with AIP.
METHODS: We retrospectively identified patients with AIP among those who underwent LTx at our center between January 2008 and December 2020.
RESULTS: During the study period, 4 patients with AIP underwent bilateral LTx: 3 men and 1 woman, between 30 and 57 years of age. The lung allocation score ranged between 71 and 89. Of the 4 patients, 2 needed extracorporeal membrane oxygenation and mechanical ventilation (MV) and 1 needed MV preoperatively. Time of onset to transplant ranged from 1 to 3 months. None of the patients had primary graft dysfunction after LTx; 2 had acute cellular rejection and 1 had chronic lung allograft dysfunction. The 4 patients are alive with survival ranging between 1 and 12 years after LTx.
CONCLUSION: AIP should be considered in patients with acute respiratory failure without a clear etiology. Our study showed that LTx led to good outcomes and should be considered as a treatment option in appropriate candidates.
Medienart: |
E-Artikel |
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Erscheinungsjahr: |
2022 |
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Erschienen: |
2022 |
Enthalten in: |
Zur Gesamtaufnahme - volume:54 |
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Enthalten in: |
Transplantation proceedings - 54(2022), 8 vom: 07. Okt., Seite 2313-2316 |
Sprache: |
Englisch |
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Beteiligte Personen: |
Shepherd, Hailey M [VerfasserIn] |
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Anmerkungen: |
Date Completed 06.12.2022 Date Revised 06.12.2022 published: Print-Electronic Citation Status MEDLINE |
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doi: |
10.1016/j.transproceed.2022.07.010 |
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funding: |
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Förderinstitution / Projekttitel: |
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PPN (Katalog-ID): |
NLM345928237 |
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500 | |a published: Print-Electronic | ||
500 | |a Citation Status MEDLINE | ||
520 | |a Copyright © 2022 Elsevier Inc. All rights reserved. | ||
520 | |a BACKGROUND: Acute interstitial pneumonia (AIP), also known as Hamman-Rich syndrome, is a rare and rapidly progressive idiopathic interstitial lung disease with a high mortality rate. Treatment is limited to supportive care and empirical high-dose steroids; however, outcomes are generally poor. There are few reports of lung transplantation (LTx) in patients with AIP | ||
520 | |a METHODS: We retrospectively identified patients with AIP among those who underwent LTx at our center between January 2008 and December 2020 | ||
520 | |a RESULTS: During the study period, 4 patients with AIP underwent bilateral LTx: 3 men and 1 woman, between 30 and 57 years of age. The lung allocation score ranged between 71 and 89. Of the 4 patients, 2 needed extracorporeal membrane oxygenation and mechanical ventilation (MV) and 1 needed MV preoperatively. Time of onset to transplant ranged from 1 to 3 months. None of the patients had primary graft dysfunction after LTx; 2 had acute cellular rejection and 1 had chronic lung allograft dysfunction. The 4 patients are alive with survival ranging between 1 and 12 years after LTx | ||
520 | |a CONCLUSION: AIP should be considered in patients with acute respiratory failure without a clear etiology. Our study showed that LTx led to good outcomes and should be considered as a treatment option in appropriate candidates | ||
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700 | 1 | |a Pasque, Michael K |e verfasserin |4 aut | |
700 | 1 | |a Kulkarni, Hrishikesh S |e verfasserin |4 aut | |
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