Management of Pneumothorax in Lymphangioleiomyomatosis

Almoosa, Khalid F.; Ryu, Jay H.; Méndez, José L.; Huggins, John T.; Young, Lisa R.; Sullivan, Eugene J.; Maurer, J.R.; McCormack, Francis X.; Sahn, Steven A.

doi:10.1378/chest.129.5.1274

Cited by 136 publications

(54 citation statements)

References 21 publications

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“…Dyspnea and spontaneous pneumothorax are the typical presenting symptoms and findings, as in our case. Pneumothorax occurs during application in almost 40% of patients and in 66% of patients at any time in the disease process (3,4). Pneumothorax recurrences are identified in 75% of patients, as in our case (5).…”

Section: Discussionsupporting

confidence: 48%

Progesterone Treatment Experience in a Patient with Lymphangioleiomyomatosis Presenting with Recurrent Pneumothorax

Tülek¹,

Kanat²,

Süerdem³

2015

Eurasian J Pulmonol

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Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease. Patients with LAM generally present with dyspnea and spontaneous pneumothorax. Today, while there is no proven medical treatment method for LAM, progesterone is among the widely used drugs. In this report, the effect of approximately 1 year of progesterone treatment in a patient with LAM, presenting with recurrent pneumothorax, was evaluated.

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Section: Discussionsupporting

confidence: 48%

Progesterone Treatment Experience in a Patient with Lymphangioleiomyomatosis Presenting with Recurrent Pneumothorax

Tülek¹,

Kanat²,

Süerdem³

2015

Eurasian J Pulmonol

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“…The treatment of SP in BHDS does not differ from the treatment of pneumothorax of other etiologies. [49] Almoosa et al [50] found that chemical and surgical pleurodesis in patients with LAM decreased the pneumothorax recurrence rate, and pleurodesis after the first SP in BHDS has been suggested. Thorascopic pleural covering technique has also been suggested as a way of preventing recurrence of pneumothorax in patients with BHDS, but further studies are needed.…”

Section: Resultsmentioning

confidence: 99%

Birt–Hogg–Dubé syndrome: a case report and a review of the literature

Jensen

Villumsen

Skytte

et al. 2017

European Clinical Respiratory Journal

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Background: Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant inherited syndrome caused by mutations in the folliculin coding gene (FLCN). The clinical manifestations of the syndrome involve the skin, lungs, and kidneys. Because of the rarity of the syndrome, guidelines for diagnosis and management of the patients with BHDS are lacking. Objective: To present a case story and a review of the literature on BHDS in order to give an update on genetics, clinical manifestations, diagnosis, treatment, prognosis and follow-up strategies. Design: Literature review and case story. Results: A PubMed and Embase search identified 330 papers. BHDS is characterized by small benign tumors in the skin, spontaneous pneumothoraces caused by cysts in the lungs and a seven-fold increased risk of renal cancer. A case story of a young female patient presenting with pneumothorax and a family history of recurrent pneumothoraces in many relatives illustrates how the history and the diagnostic work up resulted in a diagnosis of BHDS. Conclusion: BHDS is a rare inherited disorder. In patients with spontaneous pneumothorax or cystic lung disease without any obvious explanation, BHDS should be considered. Concomitant skin manifestations, a family history of familiar pneumothorax, renal cancers and skin manifestations supports the suspicion of BHDS. Early diagnosis is important in order to subject patients to systematic screening for renal cancers. A radiological surveillance strategy for renal cancer is proposed.

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“…Interestingly, in an off-label study, sirolimus treatment completely resolved or reduced lymphangioleiomyomas and effusions in patients with LAM [76]. Chemical pleurodesis has been advised for the management of pneumothorax as the best choice in preventing recurrence of pneumothorax, although it is associated with perioperative bleeding [78, 79]. …”

Section: Lymphangioleiomyomatosismentioning

confidence: 99%

Lymphatics in lymphangioleiomyomatosis and idiopathic pulmonary fibrosis

Glasgow¹,

El–Chemaly²,

Moss³

2012

European Respiratory Review

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The primary function of the lymphatic system is absorbing and transporting macromolecules and immune cells to the general circulation, thereby regulating fluid, nutrient absorption and immune cell trafficking. Lymphangiogenesis plays an important role in tissue inflammation and tumour cell dissemination. Lymphatic involvement is seen in lymphangioleiomyomatosis (LAM) and idiopathic pulmonary fibrosis (IPF). LAM, a disease primarily affecting females, involves the lung (cystic destruction), kidney (angiomyolipoma) and axial lymphatics (adenopathy and lymphangioleiomyoma). LAM occurs sporadically or in association with tuberous sclerosis complex (TSC). Cystic lung destruction results from proliferation of LAM cells, which are abnormal smooth muscle-like cells with mutations in the TSC1 or TSC2 gene. Lymphatic abnormalities arise from infiltration of LAM cells into the lymphatic wall, leading to damage or obstruction of lymphatic vessels. Benign appearing LAM cells possess metastatic properties and are found in the blood and other body fluids. IPF is a progressive lung disease resulting from fibroblast proliferation and collagen deposition. Lymphangiogenesis is associated with pulmonary destruction and disease severity. A macrophage subset isolated from IPF bronchoalveolar lavage fluid (BALF) express lymphatic endothelial cell markers in vitro, in contrast to the same macrophage subset from normal BALF. Herein, we review lymphatic involvement in LAM and IPF.

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Management of Pneumothorax in Lymphangioleiomyomatosis

Cited by 136 publications

References 21 publications

Progesterone Treatment Experience in a Patient with Lymphangioleiomyomatosis Presenting with Recurrent Pneumothorax

Progesterone Treatment Experience in a Patient with Lymphangioleiomyomatosis Presenting with Recurrent Pneumothorax

Birt–Hogg–Dubé syndrome: a case report and a review of the literature

Lymphatics in lymphangioleiomyomatosis and idiopathic pulmonary fibrosis

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