Fibrosing Mediastinitis With Progressive Symptoms or Mediastinal Compression After First-Line Medical Therapy Did Not Achieve Goals

Clinical scenario

This protocol addresses patients with active symptoms of fibrosing mediastinitis, or significant progressive compression of vital mediastinal structures. In this population, management is typically divided between medical and interventional approaches.

When first-line therapy did not reach its targets

Initial medical therapy — which may include corticosteroids as the primary option, or alternatives such as corticosteroids combined with mycophenolate, high-dose corticosteroids combined with methotrexate, tamoxifen, or antifungal therapy with itraconazole — did not achieve the expected goals of symptom improvement and reduction in fibrosis findings on chest CT. This protocol defines the next step after that failure.

Next-line approach (partial overview)

In refractory fibrosing mediastinitis, a targeted agent directed at a specific cellular mediator of the fibrotic process represents the therapy of choice. The complete protocol — including selection criteria, regimen details, and monitoring guidance — is available below.

Treatment goals

Reduction in symptom burden, reduction in PET avidity, reduction in lesion volume, and no evidence of disease progression.

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References

DOI: 10.1007/s13665-025-00382-3

In patients with symptoms or significant progressive compression of vital structures, therapies are typically divided into medical versus interventional.
Finally, in refractory disease, rituximab is the typical therapy of choice given the proposed mechanism of fibrosis involving accumulation of CD20 positive B cells triggering the fibrotic cascade.
In a case series published in 2014 by Westerly et al. [25], they examined the therapeutic response to the usage of rituximab with differing levels of mediastinal involvement with reduction in symptom burden and PET avidity noted in all three cases.
None of the 22 patients showed evidence of disease progression after receiving rituximab.
Almost half of the patients had reduction in lesion volume and 73% experienced improvement in symptoms.

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