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Thymoma-Associated Systemic Lupus Erythematosus and Myasthenia Gravis
Keysha González-Ramos1, Ileana Rivera-Burgos2, Carlos Cedeño3
1Division of Rheumatology, Department of Medicine, University of Puerto Rico School of Medicine, Medical Sciences Campus, San Juan, Puerto Rico, USA.
Abstract:
Thymomas, rare tumors originating from thymic epithelial cells, can disrupt immune tolerance and contribute to the development of autoimmune diseases. While thymomas are commonly associated with myasthenia gravis (MG), their link to autoimmune rheumatic diseases such as systemic lupus erythematosus (SLE) is less frequently observed. We present a case of a 32-year-old woman with SLE who was admitted with headache, blurred vision in her right eye with ptosis, generalized weakness, and dysphagia. She had been experiencing dysphagia and weakness for the past year, with symptoms intensifying in the three weeks leading up to admission. Her SLE had been diagnosed a year earlier, presenting with polyarthritis, positive antinuclear and anti-dsDNA antibodies, and low levels of C3 and C4. Inpatient laboratories revealed positive anti-acetylcholine receptor antibodies. A chest computed tomography (CT) scan revealed an anterior mediastinal mass invading the left pleural space as well as the left brachial and cephalic veins. A CT-guided biopsy of the mass confirmed a thymoma consistent with World Health Organization type AB. She was diagnosed with MG and thymoma. MG was managed with prednisone, pyridostigmine, and intravenous immunoglobulin. She underwent four cycles of neoadjuvant chemotherapy for her thymoma, with two additional cycles, with a modified treatment regimen, administered to enhance tumor reduction prior to surgery. She underwent thymectomy, pericardiectomy, venorrhaphy, and pneumorrhaphy and was initiated on radiation therapy due to tumor invasion of adjacent structures. At 12-month follow-up, her MG symptoms had completely resolved. As for her SLE, arthralgias persisted in the absence of arthritis, and no additional lupus manifestations developed. This case illustrates the diagnostic challenges posed by overlapping autoimmune disorders and emphasizes the need for careful evaluation to identify underlying malignancies, like thymoma, in patients presenting with atypical or evolving autoimmune symptoms.
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