Pulmonary tuberous sclerosis responds to progesterone treatment

Murray, Dominic; O’Sullivan, David

doi:10.1007/bf02949299

Cited by 5 publications

(6 citation statements)

References 8 publications

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“…The presence of chylomicrons was only specified in 3 cases [46,83,101]. The PF culture was negative in the 16 cases where this was reported [10,21,30,33,37,39,45,46,49,62,65,74,80,81,83,93], as was cytology for malignancy in the 25 cases where this parameter was described [11,12,16,21,22,27,30,35,37,39,40,45,46,48,50,58,60,62,71,72,74,81,87,93,99]. Pleural biopsy results were provided for 7 patients: in 3 cases the results were considered normal [21,40,66]; 1 showed a discrete mesothelial reaction [13]; in another, the histopathological examination of the pleura showed aggregates of nodular smooth muscle cells [22], and in 2 cases the postmortem study showed dilated lymph nodes in 1 case [16] and firm, white pleural thickening containing cystic cavities in the other [18].…”

Section: Resultsmentioning

confidence: 99%

“…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%

“…The largest rate of favorable results was obtained with sirolimus (23/23 patients, 100%) [79,86,89,90,91,94,95] and pleurectomy/decortication (9/11 cases, 81.8%) [10,13,24,45,57,62,63]. Medroxyprogesterone was used in 23 patients, either alone (6 cases [35,40,45,49,63,74]) or in combination with other treatments [29,33,39,41,42,46,48,57,61,63,66,79] (thoracentesis, chest tube pleurodesis, pleurectomy, and oophorectomy) with favorable results [33,40,42,45,48,49,57,63,66,74] in 13 cases (56.5%). Lung transplantation (unilateral or bilateral) was performed in 7 patients with poor response in all cases [44,52,79,94,101], although in 1 case the disease took 5 years to reappear [79].…”

Section: Resultsmentioning

confidence: 99%

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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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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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

et al. 2016

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“…In most reported cases, chylothorax relapsed and required repeated thoracocentesis, chemical pleurodesis or surgical pleurectomy, and a spontaneous improvement of the chylous effusion with time is unlikely. However, the results of hormonal manipulations in chylothorax associated with pulmonary TS [3][4][5] are not well-documented. In one patient with pulmonary LAM associated with TS and chylothorax, LUNA et al [4] reported that tamoxifen therapy was effective in stopping the progressive course and in controlling the chylothorax but their patient had a tetracycline-pleurodesis prior to the hormonal treatment [4].…”

Section: Discussionmentioning

confidence: 99%

“…In one patient with pulmonary LAM associated with TS and chylothorax, LUNA et al [4] reported that tamoxifen therapy was effective in stopping the progressive course and in controlling the chylothorax but their patient had a tetracycline-pleurodesis prior to the hormonal treatment [4]. MURRAY and O'SULLIVAN [5] reported the case of a woman with chylothorax associated with TS that gradually resolved with medroxyprogesterone over a period of 6 months.…”

Section: Discussionmentioning

confidence: 99%

Progesterone treatment in chylothorax associated with pulmonary tuberous sclerosis

Jounieaux¹,

Druelle²,

Mayeux³

et al. 1996

Eur Respir J

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P Pr ro og ge es st te er ro on ne e t tr re ea at tm me en nt t i in n c ch hy yl lo ot th ho or ra ax x a as ss so oc ci ia at te ed d w wi it th h p pu ul lm mo on na ar ry y t tu ub be er ro ou us s s sc cl le er ro os si is s Medroxyprogesterone acetate administration led to a complete disappearance of the chylothorax in 8 months. The patient was kept on therapy, and no recurrence of pleural effusion has been observed up to the present (22 months follow-up). However, a progressive deterioration in pulmonary function was observed, with a significant decrease in pulmonary transfer factor and increase in airway obstruction.In conclusion, this report demonstrates an objective benefit of progesterone therapy on chylous effusion associated with pulmonary tuberous sclerosis.

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Lymphangioleiomyomatosis: a review of the literature

Hancock¹,

Osborne²

2002

Respiratory Medicine

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Lymphangioleiomyomatosis (LAM), characterized by alveolar smooth muscle proliferation and cystic destruction of lung parenchyma, can occur as a rare sporadic disease or as a complication of tuberous sclerosis (TSC). It is a cystic lung disease, usually generalized and progressive, may be extremely difficult to treat and has been considered to have a poor prognosis. It has almost exclusively been reported to present in women of childbearing age, most commonly with dyspnoea and pneumothorax. We reviewed the English literature from 1939 to 1997 for cases of LAM both with and without TSC, in order to document the prevalence, clinical features, investigations, treatment and outcome within and between these two groups. No study has yet determined the prevalence of LAM symptomatically within the general population, but it probably affects 1-3% of the TSC population. Patients with TSC often present with an insidious onset of dyspnoea whilst non-TSC patients present more commonly with acute breathlessness secondary to pneumothorax. Patients with TSC are also less likely to suffer from chylothorax. The age of onset of symptoms and of diagnosis are similar. LAM is rare in children and even less common in males in both groups. The natural course of LAM remains unclear and effect of treatment variable. Although symptomatic LAM is uncommon it causes a significant amount of morbidity and mortality both in the TSC and general population, but asymptomatic LAM is not uncommon in TSC. Further research is required to determine the natural history of this condition and to evaluate current treatment regimes.

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Pulmonary tuberous sclerosis responds to progesterone treatment

Cited by 5 publications

References 8 publications

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

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

Progesterone treatment in chylothorax associated with pulmonary tuberous sclerosis

Lymphangioleiomyomatosis: a review of the literature

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