Pulmonary arterial involvement leading to alveolar hemorrhage in lymphangioleiomyomatosis

Baldi, Bruno Guedes; Pimenta, Suzana Pinheiro; Kawassaki, Alexandre de Melo; Bernardi, Fabı́ola Del Carlo; Dolhnikoff, Marisa; Carvalho, Carlos Roberto Ribeiro

doi:10.1590/s1807-59322011000700031

Cited by 10 publications

(6 citation statements)

References 4 publications

(12 reference statements)

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“…Previous reports of LAM cells involving pulmonary vessels and vascular remodeling could suggest a direct pulmonary vascular involvement as the cause of PH in LAM [ 11 , 12 ]. However, these previous findings were observed mostly in patients with severe parenchymal lung disease, and our study also showed that PH was associated with a degree of parenchymal involvement in LAM, as in other lung diseases, which explains the higher prevalence of use of sirolimus in those with PH.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method

Freitas

Baldi

Jardim

et al. 2017

Orphanet J Rare Dis

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BackgroundLymphangioleiomyomatosis (LAM) is included within group 5 of the current PH classification (unclear multifactorial mechanisms). However, data regarding the occurrence of PH in LAM are scarce. The aims of the study were to describe the prevalence and characteristics of PH in a large cohort of LAM patients with different levels of severity, and to evaluate the role of echocardiography and carbon monoxide diffusion capacity (DLCO) as screening methods for PH in LAM.MethodsOne hundred five LAM patients underwent transthoracic echocardiography, pulmonary function tests (PFTs) and 6-min walk test (6MWT). Patients with a suspicion of PH on echocardiography, defined by the presence of estimated systolic pulmonary artery pressure (PAP) over 35 mmHg or PFT showing DLco below 40% of the predicted value, underwent right heart catheterisation to confirm the diagnosis of PH.ResultsEight patients (7.6%) had PH confirmed on right heart catheterisation, six patients (5.7%) had a pre-capillary pattern and two patients (1.9%) had a post-capillary profile. Only one patient (1%) had mean PAP over 35 mmHg. Patients with PH had lower FEV1 and DLCO in PFTs and greater oxygen desaturation and dyspnea intensity during 6MWT compared with those without PH. In 63% of the patients with confirmed PH, the right heart catheterisation was performed based only on DLCO result.ConclusionsThe prevalence of PH is low in LAM patients. Pulmonary hypertension in LAM is typically mild and significantly associated with pulmonary parenchymal involvement. Carbon monoxide diffusion capacity significantly improved the identification of PH in LAM patients.

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Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method

Freitas

Baldi

Jardim

et al. 2017

Orphanet J Rare Dis

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“…It has been previously reported that ground-glass attenuation is often demonstrated on HRCT images of LAM patients and is considered to represent intra-alveolar hemorrhage or lymphatic congestion 7 , 12 – 15 . It would be easier to consider these radiologic findings as intra-alveolar hemorrhage or lymphatic congestion when found after the diagnosis of LAM had been established (Fig.…”

Section: Discussionmentioning

confidence: 99%

Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis

Sekimoto

Suzuki

Okura

et al. 2021

Sci Rep

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Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances present challenges to establishing the proper LAM diagnosis. The objective of this study is to accrue uncommon chest HRCT appearances, determine frequencies in both tuberous sclerosis complex (TSC)-associated LAM (TSC-LAM) and sporadic LAM (S-LAM) patients. 311 females referred to our hospital, including 272 S-LAM patients (mean age 39.2 years) and 39 TSC-LAM patients (mean age 38.3 years), were retrospectively evaluated. We found 2 types of radiologic findings likely to make HRCT cyst appearance atypical: characteristics of the cyst itself and uncommon findings in addition to cysts. We found that approximately 80% of LAM patients, whether TSC-associated or sporadic, showed typical HRCT appearance with mild to severe cystic destruction. The remaining 20% displayed unusual profiles in cyst appearance as well as additional findings aside from cyst: the former includes large cyst, thickened walls, and irregularly shaped whereas the latter includes ground glass attenuation and diffuse noncalcified nodules. It is important to be aware of various radiologic findings that make HRCT cystic appearance atypical of LAM.

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“…AMLs are twice as common in females, and can occur sporadically or in association with other disorders, such the autosomal dominant condition Tuberous Sclerosis Complex (TSC) and sporadic lymphangioleiomyomatosis (LAM). In particular, LAM is a progressive disease of the lung histologically characterized by a diffuse proliferation of atypical smooth muscle cells (LAM cells) in the alveoli and cystic degeneration of the normal lung parenchyma [ 11 ].…”

Section: Introductionmentioning

confidence: 99%

Human renal angiomyolipoma cells of male and female origin can migrate and are influenced by microenvironmental factors

et al. 2018

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BackgroundImproving the knowledge of angiomyolipoma physiopathology might help in refining its pharmacological treatment. We investigated if angiomyolipoma cells have migratory properties, how their growth and motility can be influenced by the hormonal milieu, and if this can be related to a specific gender.MethodsPrimary cells were isolated from angiomyolipomas surgically resected for therapeutical reasons in a female and in a male patient. The genetic control demonstrated no TSC2 deletion. Bi- (wound healing) and three-dimensional (transwell assay) migration were analyzed in vitro in basal conditions and under the influence of 17- β-estradiol and SDF-1α.ResultsTreatment up to 72 hours with 17-β-estradiol (0.1–100 nM), tamoxifen (0.2–20 μM) or with both, does not modify angiomyolipoma cells proliferation. On the other hand, SDF-1α and 17-β-estradiol treatment induce a significant motility increase (both bi- and three-dimensional) which becomes evident already after 2 hours of incubation. Angiomyolipoma cells express mRNA coding for SDF-1α and 17-β-estradiol receptors and secrete both the metalloproteases principally involved in malignant phenotype acquisition, i.e. MMP-2 and MMP-9.ConclusionAngiomyolipoma cells behave similarly, despite their different source. Primary angiomyolipoma cells migrate in response to hormonal milieu and soluble factors, and produce active metalloproteases, both aspects being consistent with the theory claiming they can migrate to the lungs (and/or other organs) and colonizing them. No main feature, among the aspects we analyzed, seems to be referable to the gender of origin.

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Pulmonary arterial involvement leading to alveolar hemorrhage in lymphangioleiomyomatosis

Cited by 10 publications

References 4 publications

Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method

Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method

Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis

Human renal angiomyolipoma cells of male and female origin can migrate and are influenced by microenvironmental factors

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