Efficacy and safety of transbronchial lung biopsy for the diagnosis of lymphangioleiomyomatosis: A report of 24 consecutive patients

Koba, Taro; Arai, Toru; Kitaichi, Masanori; Kasai, Takahiko; Hirose, Masaki; Tachibana, Kazunobu; Sugimoto, Chikatoshi; Akira, Masanori; Hayashi, Seiji; Inoue, Yoshikazu

doi:10.1111/resp.13190

Cited by 19 publications

(18 citation statements)

References 30 publications

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“…Previously, a surgical lung biopsy (SLB) was considered the gold standard but more recently, emphasis has been placed on obtaining a diagnosis through less invasive means. Transbronchial lung biopsy has an estimated yield >50% for the diagnosis of LAM and markers of LAM severity such as abnormal D LCO are associated with an increased diagnostic yield [43]. As yet, the true rate of complications is unknown as there have been no prospective studies.…”

Section: Lung Biopsymentioning

confidence: 99%

Lymphangioleiomyomatosis: a clinical review

O'Mahony

Lynn

Murphy

et al. 2020

Breathe

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Lymphangioleiomyomatosis (LAM) is a diffuse cystic lung disease. There are two main types of LAM: sporadic, and LAM associated with the tuberous sclerosis complex (TSC), which is caused by mutations in the TSC1 and TSC2 genes. LAM is characterised by cystic lung disease resulting in progressive dyspnoea, renal angiomyolipomas and lymphatic complications. Pneumothorax occurs frequently (70%) and definitive management with pleurodesis is recommended as the risk of recurrence is high. Characteristic thin-walled cysts are seen on computed tomography and the presence of elevated serum levels of a vascular endothelial growth factor-D has good diagnostic specificity. Currently, no single clinical or serological factor has been shown to predict prognosis. However, over the past decade, significant advances in our understanding of the pathophysiology of LAM has led to improved recognition of this rare disease and identification of treatment options. Mechanistic target of rapamycin inhibitors slow the rate of lung function decline and can resolve chylous effusion and regress angiomyolipomas. Life expectancy in patients with LAM is favourable, with a mean transplant-free survival >20 years from the time of diagnosis. Continued advances in understanding the molecular basis of LAM will lead to improved therapeutic targets and the development of more robust prognostic indicators.Educational aimsTo illustrate the clinical features, common presentations and radiological features of LAMTo outline the diagnostic approach to LAM, including the role of VEGF-DTo review the current prognostic indicators in LAM, and outline the impact of lung function, hormonal status, VEGF-D and clinical presentation on outcomeTo inform clinicians on the management options for LAM both pharmacological and nonpharmacological

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Section: Lung Biopsymentioning

confidence: 99%

Lymphangioleiomyomatosis: a clinical review

O'Mahony

Lynn

Murphy

et al. 2020

Breathe

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“…Because LAM is exceedingly rare in males, the final diagnosis should be made in each case on the basis of a typical result of a lung biopsy [7]. A transbronchial biopsy has been shown to be a relatively safe method in the group of LAM patients [55].…”

Section: Diagnosismentioning

confidence: 99%

Rozpoznanie i leczenie limfangioleiomiomatozy (LAM) z grupy PEComa

et al. 2021

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Lymphangioleiomyomatosis (LAM) is a rare, proliferative lung disease, leading to progressive damage of their structure and is a member of the PEComa neoplasm family (perivascular epithelioid cell tumors). In the patients, solid-cystic masses described as lymphangioleiomyoma or extrapulmonary LAM (E-LAM) can occur. E-LAM foci have been described in the mediastinum, supraclavicular lymph nodes, the liver, walls of the small and large intestine, the pancreas, mesentery. E-LAM masses can attain very large sizes -tumors 15-22 cm long have been described. On the basis of positive results of clinical trials sirolimus, a drug from the group of mTOR kinase inhibitors, was registered by the Food and Drug Administration (FDA) in May 2015 as the first and currently only drug for systemic LAM therapy. Sirolimus use is recommended in patients with LAM, accompanied by rapidly progressing deterioration of respiratory system function or FEV 1 ≤ 70% predicted value and in patients with pleural lymph exudate before applying invasive methods of treatment.

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“…It is worth highlighting that, in certain situations, especially patients with mild disease who would not warrant initiation of pharmacotherapy, a probable diagnosis of LAM with serial monitoring may be a reasonable strategy. In cases where a definite diagnosis is needed, transbronchial lung biopsy has a yield of Ͼ 60%, appears to be safe based on small series, [47][48][49] and should be performed prior to pursuing surgical lung biopsy. 50 Critical review of the pathology by an expert pathologist is essential to avoid false negatives on biopsy.…”

Section: Diagnosismentioning

confidence: 99%

Diffuse Cystic Lung Diseases

et al. 2019

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Diffuse cystic lung diseases (DCLDs) are a group of diverse pulmonary disorders with varying pathophysiology that are characterized by the presence of thin-walled, air-filled spaces within lung parenchyma. High-resolution computed tomography plays a crucial role in the evaluation of DCLDs, and cyst characteristics such as morphology, distribution, and the presence of other associated radiologic findings can help distinguish between different DCLDs. DCLDs can be classified according to their underlying pathophysiology as neoplastic, genetic, lymphoproliferative, infectious, associated with other forms of interstitial lung disease, or related to smoking. In this review we will provide a clinical overview on the most common DCLDs that are encountered in clinical practice: lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, Birt-Hogg-Dubé syndrome, and lymphoid interstitial pneumonia/follicular bronchiolitis, with a focus on practical aspects that can help clinicians in the optimal diagnosis and management of patients with DCLDs.

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Efficacy and safety of transbronchial lung biopsy for the diagnosis of lymphangioleiomyomatosis: A report of 24 consecutive patients

Abstract: TBLB is a safe and effective method for the pathological diagnosis of LAM.

Cited by 19 publications

References 30 publications

Lymphangioleiomyomatosis: a clinical review

Lymphangioleiomyomatosis: a clinical review

Rozpoznanie i leczenie limfangioleiomiomatozy (LAM) z grupy PEComa

Diffuse Cystic Lung Diseases

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