Vincenzo Carlomagno
ANTI-SRP IMMUNE-MEDIATED NECROTIZING MYOPATHY IN A PATIENT WITH ANTI-ACHR MYASTHENIA GRAVIS AND THYMOMA.
Autori
- VINCENZO CARLOMAGNO (FONDAZIONE POLICLINICO GEMELLI, UNIVERSITA’ CATTOLICA DEL SACRO CUORE ROMA – NEUROLOGIA)
- ALESSANDRA CICIA (FONDAZIONE POLICLINICO GEMELLI, UNIVERSITA’ CATTOLICA DEL SACRO CUORE ROMA – NEUROLOGIA)
- ELEONORA TORCHIA (FONDAZIONE POLICLINICO GEMELLI, UNIVERSITA’ CATTOLICA DEL SACRO CUORE ROMA – NEUROLOGIA)
- SARA BORTOLANI (FONDAZIONE POLICLINICO GEMELLI, UNIVERSITA’ CATTOLICA DEL SACRO CUORE ROMA – NEUROLOGIA)
- RAFFAELE IORIO (FONDAZIONE POLICLINICO GEMELLI, UNIVERSITA’ CATTOLICA DEL SACRO CUORE ROMA – NEUROLOGIA)
- ENZO RICCI (FONDAZIONE POLICLINICO GEMELLI, UNIVERSITA’ CATTOLICA DEL SACRO CUORE ROMA – NEUROLOGIA)
- MASSIMILIANO MIRABELLA (FONDAZIONE POLICLINICO GEMELLI, UNIVERSITA’ CATTOLICA DEL SACRO CUORE ROMA – NEUROLOGIA)
- MATTEO LUCCHINI (FONDAZIONE POLICLINICO GEMELLI, UNIVERSITA’ CATTOLICA DEL SACRO CUORE ROMA – NEUROLOGIA)
Presentatore
VINCENZO CARLOMAGNO
Modalità
Poster Session
Abstract
“Myasthenia gravis is a rare disease mainly associated with anti-AChR antibodies. About 10% of anti-AChR+ myasthenic patients present with thymoma. Rarely, immune-mediated myositis may occur in these patients. A 43-year-old female was diagnosed in 2017 with anti-AChR+ myasthenia gravis associated with thymoma, treated with thymectomy, achieving pharmacological remission with azathioprine 75 mg daily. A CT chest scan performed in June 2022 showed a small nodule suspected of thymoma recurrence. In August 2022, she presented with acute onset myalgia and symmetrical proximal limb weakness with elevated creatine kinase (CK). Needle EMG showed mild signs of myopathic changes in proximal limb muscles. MRI demonstrated bilateral proximal and distal myositis involvement of all four limbs without signs of fat replacement. Muscle biopsy revealed a necrotizing myopathy pattern, and anti-SRP antibodies were detected in the serum. The patient was treated with intravenous corticosteroids, with clinical benefit, and was discharged on prednisone 50 mg daily. Two months later, following an acute upper airway infection, she developed respiratory failure due to myasthenic exacerbation and was admitted to intensive care, requiring oro-tracheal intubation. She underwent 4 cycles of plasmapheresis with resolution of respiratory distress. After discharge, prednisone was gradually tapered. The patient is currently in clinical remission, maintained on azathioprine 75 mg daily and prednisone 20 mg every other day. In myasthenic patients, the presence of new-onset non-fluctuating muscle weakness associated with CK elevation, albeit rare, should suggest a concomitant myositic process. Moreover, thymic recurrence could possibly play a pathogenic role.”