# Ultrasound-guided transbronchial biopsy in the diagnosis of fibrosing mediastinitis-associated pulmonary hypertension

**Authors:** Yu Zhang, Han-Xiang Song, Yong-Jia Qi, Nan-Nan Sun, Zan-Sheng Huang, Wan-Lei Fu, Jing Zhang, Felix J. F. Herth, Ye Fan

PMC · DOI: 10.1186/s13023-025-03695-3 · Orphanet Journal of Rare Diseases · 2025-04-15

## TL;DR

This study shows that a new biopsy technique can safely and effectively diagnose a rare lung condition linked to high blood pressure in the lungs.

## Contribution

Demonstrates the safety and diagnostic superiority of cryobiopsy over standard methods for fibrosing mediastinitis-associated pulmonary hypertension.

## Key findings

- Cryobiopsy achieved 100% diagnostic yield for FM-PH compared to 40% for needle aspiration.
- Immunohistochemistry revealed mixed inflammatory cells in FM-PH lesions.
- No significant complications were observed with cryobiopsy.

## Abstract

Fibrosing mediastinitis is a rare benign disease frequently complicated by pulmonary hypertension. A definitive diagnosis for fibrosing mediastinitis-associated pulmonary hypertension (FM-PH) and its etiologies necessitates mediastinal biopsy and subsequent pathological assessment. Endobronchial ultrasound (EBUS)-guided transbronchial mediastinal cryobiopsy is a recently developed technique that provides diagnostic advantages over standard needle biopsy, particularly in benign mediastinal disorders. Nevertheless, their safety and efficacy in diagnosing FM-PH remain elusive.

We retrospectively studied patients with mediastinal lesion and pulmonary vascular compression who underwent both transbronchial needle aspiration and mediastinal cryobiopsy with EBUS guidance. Diagnostic yields of FM-PH and its etiologies, along with procedure-related adverse events, were analyzed. Immunohistochemical study was conducted to identify immunological properties of FM-PH.

Of the 529 patients with mediastinal lesions, 80 exhibited pulmonary vessel compression, including 10 who were ultimately diagnosed with FM-PH following mediastinal biopsy and right heart catheterization. Cryobiopsy showed a higher diagnostic yield for FM-PH compared to needle aspiration (100% versus 40%, p = 0.011). Disease etiologies included pneumoconiosis in 5 cases, tuberculosis in 3, and idiopathic FM-PH in the remaining 2. Cryobiopsy appeared to be superior to needle biopsy for etiological diagnosis, although this difference was not statistically significant (80% versus 60%, p = 0.628). Immunohistochemical analyses of cryosamples revealed mixed inflammatory infiltrates of B and T lymphocytes, as well as macrophages, surrounding or within FM-PH lesions. There was no significant bleeding or other complications.

Transbronchial mediastinal cryobiopsy might be a safe and effective diagnostic tool for FM-PH, offering valuable information for personalized treatment.

The online version contains supplementary material available at 10.1186/s13023-025-03695-3.

## Linked entities

- **Diseases:** fibrosing mediastinitis (MONDO:0018978), pulmonary hypertension (MONDO:0005149), pneumoconiosis (MONDO:0015926), tuberculosis (MONDO:0018076)

## Full-text entities

- **Diseases:** pneumoconiosis (MESH:D011009), bleeding (MESH:D006470), tuberculosis (MESH:D014376), FM-PH (MESH:C536136), pulmonary hypertension (MESH:D006976), pulmonary vascular compression (MESH:D001261), mediastinal disorders (MESH:D008480), inflammatory (MESH:D007249), mediastinal lesion (MESH:D008477)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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Source: https://tomesphere.com/paper/PMC12001500