Acute Interstitial Pneumonia (Hamman-Rich Syndrome) in Lung Transplantation: A Case Series.

Abstract

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.

• Authors: Hailey M Shepherd, Yuriko Terada, Tsuyoshi Takahashi, Michael K Pasque, Hrishikesh S Kulkarni, Rodrigo Vazquez Guillamet, Chad A Witt, Ruben G Nava, Varun Puri, Daniel Kreisel, G Alexander Patterson, Ramsey R Hachem
• Journal: Transplantation proceedings (Transplant Proc) Vol. 54 Issue 8 Pg. 2313-2316 (Oct 2022) ISSN: 1873-2623 [Electronic] United States
• PMID: 36075742 (Publication Type: Journal Article)
• Copyright: Copyright © 2022 Elsevier Inc. All rights reserved.

Topics

• Male
• Female
• Humans
• Hamman-Rich Syndrome
• Retrospective Studies
• Lung Transplantation (adverse effects)
• Idiopathic Interstitial Pneumonias (diagnosis, surgery)
• Extracorporeal Membrane Oxygenation