AB045. Are complement inhibitors safe for thymoma-associated myasthenia gravis patients?—a literary review and case report
Case Report

AB045. Are complement inhibitors safe for thymoma-associated myasthenia gravis patients?—a literary review and case report

Angelo Luciano1, Martina Di Meo2, Erica Pietroluongo2,3, Carmine Caso2, Lucia Salatiello2, Margaret Ottaviano4, Roberto Bianco2, Alberto Servetto2, Giovannella Palmieri2

1Medical Oncology Unit, San Giovanni di Dio e Ruggi D’Aragona, Salerno, Italy; 2Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy; 3Department of Medicine, Section of Hematology and Oncology, Thoracic Oncology Program, The University of Chicago Medicine, Chicago, IL, USA; 4Department of Melanoma, Cancer Immunotherapy and Development Therapeutics, Istituto Nazionale Tumori IRCCS (Istituto di Ricovero e Cura a Carattere Scientifico) Fondazione G. Pascale, Naples, Italy

Correspondence to: Angelo Luciano, MD. Medical Oncology Unit, San Giovanni di Dio e Ruggi D’Aragona, Via San Leonardo, 84131, Salerno, Italy. Email: angelo.luciano1990@gmail.com.

Background: Thymoma-associated myasthenia gravis (TAMG) occurs in about 30% of patients affected by thymic epithelial tumors (TETs) and leads to a suboptimal response to treatment. Myasthenia gravis (MG) is a chronic autoimmune disease caused by acetylcholine receptor (AChR) generally and other autoantibodies reacting against the neuromuscular junction. The complement system plays an important role in the pathogenesis of MG. AChR antibodies [immunoglobulin G (IgG) oligomers] in MG can activate the complement cascade binding C1q; consequently, complement inhibitors, such as eculizumab, are now available for treatment in MG refractory to standard treatment. Before starting treatment with complement inhibitors, it is mandatory to be vaccinated against Neisseria meningitidis, Streptococcus pneumoniae and Haemophilus influenzae type B. Limited data are available regarding TAMG: the most significant study is a real-world cohort presented by Lei Jin, and sporadic case reports. In the phase III, randomized, double-blind, placebo-controlled REGAIN study patients with a history of thymoma or thymic neoplasm were excluded. Conversely, Lei Jin’s study involving eculizumab included 42 seropositive AChR antibodies patients with refractory MG, but only 22 patients were included. Two patients reported coronavirus disease 19 (COVID-19) and herpes labialis infection, while four patients died from respiratory or circulatory failure linked to thymoma metastasis.

Case Description: We reported our experience with a young patient who underwent thymectomy diagnosed with stage IV thymoma type B2, with lung and pleural metastasis (pT3 pNx M1a) and a higher frequency of myasthenic crisis. Cisplatin, adriamycin and cyclophosphamide (PAC schedule) were administered for six cycles. Good’s syndrome (GS) was diagnosed based on low levels of B lymphocytes and Immunoglobulins. MG was treated with corticosteroids, pyridostigmine, immunoglobulins and plasmapheresis. The patient was included in an experimental protocol involving complement inhibitors for the treatment of refractory MG. After two months from the initiation of treatment, the patient experienced severe lung infections with exacerbation of myasthenic symptoms and subsequently died.

Conclusions: Further studies are needed in patients with myasthenia and TETs with associated immunopathies, particularly GS. Antibacterial prophylaxis, vaccinations, monitoring of C-reactive protein, white blood cell count, immunoglobulins and the diagnosis of refractory MG are mandatory prior to starting treatment with complement inhibitors.

Keywords: Case report; thymomas; myasthenia gravis (MG); Good’s syndrome (GS); complement inhibitors

Acknowledgments

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Footnote

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://med.amegroups.com/article/view/10.21037/med-25-ab045/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

doi: 10.21037/med-25-ab045
Cite this abstract as: Luciano A, Di Meo M, Pietroluongo E, Caso C, Salatiello L, Ottaviano M, Bianco R, Servetto A, Palmieri G. AB045. Are complement inhibitors safe for thymoma-associated myasthenia gravis patients?—a literary review and case report. Mediastinum 2025;9:AB045.

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