Therapeutic Effect of Sirolimus for Lymphangioleiomyomatosis Remaining in the Abdominopelvic Region After Lung Transplantation: A Case Report

Itö, Tatsuo; Suno, Manabu; Sakamoto, Kana; Yoshizaki, Yusuke; Yamamoto, Kenyu; Nakanishi, R.; Hirano, Yutaka; Irie, Masahiro; Kurosaki, T.; Otani, Shinji; Yamane, Masaomi; Sugimoto, Seiichiro; Miyoshi, Kentaroh; Oto, Takahiro

doi:10.1016/j.transproceed.2015.12.021

Cited by 5 publications

(5 citation statements)

References 15 publications

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“…Although the role of mTOR inhibitors has not been proven by randomised or large scale trials in before and after LT, possible contribution to better outcome in the management for LAM‐related complications, such as chylothorax, enlargement of renal AML, and recurrence of LAM, before and after LT is indicated . Wojarski et al reported that sirolimus‐based immunosuppression is safe and beneficial in selected LT recipients .…”

Section: Discussionmentioning

confidence: 99%

“…Moreover, recurrence of pulmonary LAM and progression of angiomyolipoma (AML) occasionally occur . Whereas mTOR inhibitors could ameliorate these LAM‐related problems resulting in longer survival of LAM recipients, details of long‐term outcome, especially over 10 years, have rarely been reported. The objective of this study was to describe our single‐institutional experience with long‐term outcomes of LT for LAM.…”

Section: Introductionmentioning

confidence: 99%

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Favorable survival even with high disease‐specific complication rates in lymphangioleiomyomatosis after lung transplantation—long‐term follow‐up of a Japanese center

Kurosaki

Otani

Miyoshi

et al. 2019

Clinical Respiratory J

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Background: Lung transplantation (LT) is a reliable therapeutic option for end-stage pulmonary lymphangioleiomyomatosis (LAM). Long-term outcome of LAM recipients after LT remains unknown. The aim of this study was to describe the outcomes of LT for LAM with a long-term follow-up, comparing those for other diseases in the same period. Methods: We retrospectively reviewed consecutive 145 LT recipients between 1998 and 2015 at Okayama University Hospital with minimum 3-year follow-up. Results: Twelve LAM recipients including nine sporadic-LAM and three tuberous sclerosis complex -LAM were identified. Nine of 12 underwent bilateral LT including four living-donor lobar LT. There was no significant difference in overall survival between the two groups. (P = 0.15). Chronic lung allograft dysfunction free survival rate in LAM compared with other diseases tended to be better (P = 0.058). However, the rate of requiring hemodialysis was significantly higher in LAM recipients than in the recipients of other diseases (P = 0.047). Notably, 8 of 12 (67%) LAM patients encountered LAM-related complication including chylothorax and pneumothorax, seven (58%) had proliferative diseases consisting of renal angiomyolipoma and recurrent LAM. Nine patients required mTOR inhibitors for LAM-related problems, contributing to improved control of LAM-related problems. While all nine recipients of bilateral LT have still survived, two patients died of diseases in their native lungs and one required re-LT among three recipients of single LT. Conclusion: Although the rates of LAM-related complications were unexpectedly high in the long term, LT is a feasible therapeutic option for patients with advanced pulmonary LAM. K E Y W O R D S lung transplantation, lymphangioleiomyomatosis, mTOR inhibitor | 117 KUROSAKI et Al.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Favorable survival even with high disease‐specific complication rates in lymphangioleiomyomatosis after lung transplantation—long‐term follow‐up of a Japanese center

Kurosaki

Otani

Miyoshi

et al. 2019

Clinical Respiratory J

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“…More recently, medical treatment with sirolimus has surfaced as beneficial due to its favorable effect on lymphangioleiomyoma size. [410111213141516171819]…”

Section: Discussionmentioning

confidence: 99%

“…In contrast, the effect of sirolimus also remained significant in several cases with serum trough level <5 ng/mL as was the case in our patient. [1112]…”

Section: Discussionmentioning

confidence: 99%

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas

Ussavarungsi¹,

Laroia²,

Burger³

2019

Lung India

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Lymphangioleiomyomatosis (LAM) is a rare disease associated with cystic lung destruction and abdominal tumors, including lymphangioleiomyomas, which frequently occur in the retroperitoneal region. Sirolimus therapy is currently recommended for LAM patients with abnormal or declining lung function with an adjusted dose to maintain a serum trough level of 5–15 ng/mL. We describe a significant reduction of retroperitoneal lymphangioleiomyomas after treatment with low-dose sirolimus therapy (serum trough level <5 ng/mL) in a patient with sporadic LAM.

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“…As comorbidities occur in TSC, patients with TSC-LAM should have a careful multidisciplinary pretransplant evaluation, but TSC is not a contraindication to transplantation. Sirolimus is contraindicated immediately after transplantation as it may induce dehiscence of bronchial sutures [112, 113], but it has been used at a later stage to manage LAM-specific complications [114-117]. Whether sirolimus should be stopped when patients are listed for transplantation to avoid being on therapy during the perioperative period is currently unclear.…”

Section: Lam and Tuberous Sclerosismentioning

confidence: 99%

Cystic Lung Disease in Genetic Syndromes with Deficient Tumor Suppressor Gene Function

Daccord¹,

Nicod²,

Lazor³

2017

Respiration

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Cystic lung diseases constitute a distinct group of rare lung disorders, among which two result from monogenic defects affecting tumor suppressor genes: lymphangioleiomyomatosis, either sporadic or associated with tuberous sclerosis complex, and Birt-Hogg-Dubé syndrome. These disorders have similarities in their clinical expression, including occurrence in young adults, multiple pulmonary cysts, recurrent pneumothorax, skin hamartomas, and renal tumors. However, they markedly differ in their gender distribution, pathogenesis, disease course, and prognosis. Our knowledge on these two rare conditions is rapidly expanding. Management of lymphangioleiomyomatosis has substantially improved in the past decade with the understanding of its pathogenic mechanisms, the discovery of an effective therapy, and development of large cohorts and international guidelines. Birt-Hogg-Dubé syndrome has been described more recently and still awaits deeper understanding of its pathophysiology.

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Therapeutic Effect of Sirolimus for Lymphangioleiomyomatosis Remaining in the Abdominopelvic Region After Lung Transplantation: A Case Report

Cited by 5 publications

References 15 publications

Favorable survival even with high disease‐specific complication rates in lymphangioleiomyomatosis after lung transplantation—long‐term follow‐up of a Japanese center

Favorable survival even with high disease‐specific complication rates in lymphangioleiomyomatosis after lung transplantation—long‐term follow‐up of a Japanese center

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas

Cystic Lung Disease in Genetic Syndromes with Deficient Tumor Suppressor Gene Function

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