Pulmonary hypertension caused by fibrosing mediastinitis: A case report and literature review

Rationale: Fibrosing mediastinitis (FM) is a rare benign disorder characterized by excessive fibrotic proliferation within the mediastinum. Due to its insidious onset and nonspecific clinical presentation, FM is often misdiagnosed or diagnosed at a late stage, leading to significant morbidity. Case reports detailing its long-term course and response to interventional management remain limited. This case is reported to highlight the diagnostic challenges in FM presenting with prolonged hemoptysis and to demonstrate the therapeutic potential of pulmonary vascular intervention in alleviating vascular compression and improving hemodynamics. Patient concerns: A 56-year-old male patient presented with intermittent hemoptysis lasting over 5 years, which had worsened over the past week. Diagnoses: Imaging studies, including contrast-enhanced chest computed tomography and pulmonary artery CTA, revealed calcified mediastinal lymph nodes in both hilar regions, right middle lobe atelectasis, and pulmonary artery branch stenosis with secondary pulmonary hypertension. A final diagnosis of fibrosing mediastinitis complicated by pulmonary hypertension and lobar collapse was established. Interventions: Five years earlier, the patient underwent bronchoscopy and received cryotherapy and dilation for right middle lobe atelectasis, with no significant improvement observed. During the current admission, right heart catheterization and pulmonary angiography revealed multiple sites of extrinsic pulmonary artery stenosis, for which stents were implanted at the narrowed segments. Outcomes: Following the procedure, pulmonary artery pressure decreased significantly, clinical symptoms improved markedly, and no further episodes of hemoptysis were reported during follow-up. Lessons: This case underscores the importance of considering FM in the differential diagnosis of chronic unexplained hemoptysis, particularly when accompanied by mediastinal calcification, lobar collapse, and pulmonary hypertension. Early use of pulmonary artery CTA can aid in timely diagnosis, and endovascular stent placement represents a effective minimally invasive treatment option for selected cases of FM-induced pulmonary artery stenosis.