Diffuse lung disease due to lymphangiomyoma

Bush, Joseph K.; McLean, Ross L.; Sieker, Herbert O.

doi:10.1016/0002-9343(69)90084-9

Cited by 53 publications

(17 citation statements)

References 14 publications

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“…The patients had a mean age of 34 (9) years at onset of symptoms and a median (range) time of 2 (0-29) years from onset of symptoms to diagnosis. Compared with control subjects, cases did not differ in the use of the oral contraceptive pill (odds ratio (OR) 0 39, 95% CI 0 09 to 1.68), diagnosis of fibroids (OR [3][4][5][6][7][8][9][10][11][12] The aetiology of pulmonary lymphangioleiomyomatosis is unknown, but sex hormones have been assumed to be important since the disease develops exclusively in women and almost invariably women of reproductive age. '3"6 Treatment has usually invoved antioestrogen measures in the form of oophorectomy347 or treatment with tamoxifen,8-'0 medroxyprogesterone,8 '-and luteinising hormone releasing hormone analogues.…”

Section: Introductionmentioning

confidence: 95%

Relation of pulmonary lymphangio-leiomyomatosis to use of the oral contraceptive pill and fertility in the UK: a national case control study.

Wahedna

Cooper

Williams

et al. 1994

Thorax

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Background -Pulmonary lymphangioleiomyomatosis is a rare progressive disease ofunknown aetiology affecting premenopausal women. Since the oral contraceptive pill has been implicated in its pathogenesis, a case control study was carried out to determine whether women with the disease were more likely to have taken the oral contraceptive pill, and whether the disease was associated with other conditions related to sex hormones including pregnancy, parity, and fibroids. Methods -All chest physicians in the UK were asked for details of all live patients with pulmonary lymphangioleiomyomatosis; the patient's family doctor was then asked for four age and sex matched control subjects from their patient register. Details of lifetime use of the oral contraceptive pill, pregnancy, parity, history of fibroids, and smoking were obtained from cases and controls. Relative odds of exposure to potential risk factors were estimated by conditional logistic regression. Results The aetiology of pulmonary lymphangioleiomyomatosis is unknown, but sex hormones have been assumed to be important since the disease develops exclusively in women and almost invariably women of reproductive age.'3"6 Treatment has usually invoved antioestrogen measures in the form of oophorectomy347 or treatment with tamoxifen,8-'0 medroxyprogesterone,8 '-and luteinising hormone releasing hormone analogues. 14 None of these treatments has been assessed in a controlled trial and their value is uncertain. 5 Reports of the disease occurring in women on the oral contraceptive pill2 16 -18 and of exacerbations of the disease during pregnancy8 have added to the suspicion that sex hormones are involved. We have therefore conducted a case control study to determine whether pulmonary lymphangioleiomyomatosis is associated with the use of the oral contraceptive pill or other conditions associated with sex hormones such as pregnancy, parity, and fibroids. Methods CASES AND CONTROLSChest physicians in the UK on the British Thoracic Society register were sent an explanation of the study and asked for details of all known live patients with pulmonary lymphangioleiomyomatosis. The physicians were asked to forward to such patients a written request asking them to participate in the study, plus a questionnaire and consent form which

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Section: Introductionmentioning

confidence: 95%

Relation of pulmonary lymphangio-leiomyomatosis to use of the oral contraceptive pill and fertility in the UK: a national case control study.

Wahedna

Cooper

Williams

et al. 1994

Thorax

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“…In addition, an immunohistochemical approach cannot differentiate between CPL and DPL, both of which share positive stainings for vimentin, CD31, CD34, factor VIII-related antigen, and D2-40. Histologically, DPL is characterized by an increased number of complex anastomosing lymphatic channels, in which variable dilation or expansion is a secondary phenomenon within the lungs and mediastinum, whereas in CPL the lymphatics are not increased in number and are relatively more regular in size and shape (Bush JK et al, 1969;Brown M et al, 1999;Hirano H et al, 2004;Moerman P et al, 1993). Based on these features, DPL could also be excluded in our case.…”

Section: Discussionmentioning

confidence: 70%

“…Besides PIE, CPL should be distinguished from diffuse pulmonary lymphangiomatosis (DPL) (Hirano H et al, 2004;Bush JK et al, 1969;Brown M et al, 1999), because of their similar clinical manifestations and histological features. In addition, an immunohistochemical approach cannot differentiate between CPL and DPL, both of which share positive stainings for vimentin, CD31, CD34, factor VIII-related antigen, and D2-40.…”

Section: Discussionmentioning

confidence: 99%

An Autopsy Case of Congenital Pulmonary Lymphangiectasis Masquerading as Pulmonary Interstitial Emphysema

Yamada¹,

Sasaguri²

2012

Congenital Anomalies - Case Studies and Mechanisms

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“…Figure 1 presents a flowchart showing the complete breakdown of the steps used in the identification of appropriate studies [9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,…”

Section: Resultsmentioning

confidence: 99%

“…In another case, chylothorax appeared after bilateral lung transplant [76]. PE was not treated in 7 cases [16,20,28,44,57,69], and the choice of treatment was not specified in another 75 patients with PE.…”

Section: Resultsmentioning

confidence: 99%

Characteristics of Patients with Lymphangioleiomyomatosis and Pleural Effusion: A Systematic Review

et al. 2016

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The characteristics of patients with lymphangioleiomyomatosis (LAM) are poorly defined, as they may present with or without pleural effusion (PE). We performed a systematic review across four electronic databases searching for studies reporting clinical findings, PE characteristics, and the most effective treatment of LAM. Case descriptions and retrospective studies were included, unrestricted by year of publication. The review consisted of 94 studies (199 patients) spanning a period of nearly 55 years. The median age was 38 years (range: 1 month to 69 years), and 79.7% were between 21 and 50 years old. All cases had dyspnea, 95% had a cough, and 87.5% had chest pain. PE was exudative chylothorax, usually unilateral (76%) and right-sided, predominantly lymphocytic, and with proportionately higher levels of proteins than lactate dehydrogenase. Sirolimus was effective in all cases, completely in 87%, and partially in 13%, although the number of patients receiving sirolimus was small. The present study confirmed that LAM and PE mainly occur in women of childbearing age (third to fifth decade of life). PE was usually unilateral and presented as a lymphocyte-predominant chylous exudate. The most effective treatment for PE seems to be sirolimus, although studies with larger series are needed to confirm this.

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Diffuse lung disease due to lymphangiomyoma

Cited by 53 publications

References 14 publications

Relation of pulmonary lymphangio-leiomyomatosis to use of the oral contraceptive pill and fertility in the UK: a national case control study.

Relation of pulmonary lymphangio-leiomyomatosis to use of the oral contraceptive pill and fertility in the UK: a national case control study.

An Autopsy Case of Congenital Pulmonary Lymphangiectasis Masquerading as Pulmonary Interstitial Emphysema

Characteristics of Patients with Lymphangioleiomyomatosis and Pleural Effusion: A Systematic Review

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