Michelangelo Maestri Tassoni
EFFECTIVENESS OF THYMECTOMY IN JUVENILE MYASTHENIA GRAVIS: PRELIMINARY RESULTS OF A15-YEAR FOLLOW UP STUDY
Autori
- ESTER LATINI (Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Italy – )
- ALBA CEPELE (Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Italy – )
- MELANIA GUIDA (Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Italy – Neurologia)
- ILARIA CECCARELLI (Minimally Invasive and Robotic Thoracic Surgery-Surgical, Medical, Molecular and Critical Care Pathology Department, University Hospital of Pisa – Chirurgia Toracica)
- CARMELINA CRISTINA ZIRAFA (Minimally Invasive and Robotic Thoracic Surgery-Surgical, Medical, Molecular and Critical Care Pathology Department, University Hospital of Pisa – Chirurgia Toracica)
- FRANCA MELFI (Minimally Invasive and Robotic Thoracic Surgery-Surgical, Medical, Molecular and Critical Care Pathology Department, University Hospital of Pisa – Chirurgia Toracica)
- MICHELANGELO MAESTRI TASSONI (Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Italy – Neurologia)
Presentatore
MICHELANGELO MAESTRI TASSONI (DEPARTMENT OF CLINICAL AND EXPERIMENTAL MEDICINE, NEUROLOGY UNIT, UNIVERSITY OF PISA, ITALY)
Modalità
Oral Communication
Abstract
“Background: Thymectomy has not been trialed in pediatric patientsandits potential use in Juvenile Myasthenia Gravis (JMG) is still controversial. However, data from systematic reviews (Madenci et al., 2017) and small retrospective cohort studies indicate that the procedure is generally safeand effective also in JMG patients.
Aim and Methods: The aim ofthis retrospective cohort study was to investigatesafety andlong-term outcomes of thymectomy in our population of JMG patients. We therefore reviewed clinical, therapeutic and surgical data of all JMG patients that underwent thymectomy at the MG Clinic of Azienda Ospedaliero-Universitaria Pisana from 1999 to 2022.
Results:We identified a total of 33 patients (M:F=7:26 – median age at diagnosis 12.5 ± 3.7years). Most of subjects underwent surgery within two years from diagnosis. The median follow-up time since thymectomy was 78 .4± 51.6 months. According to MGFA-PIS classification at last evaluation, we observed an improvement in clinical status in 29 (87.9%) patients after thymectomy and a complete stable remission was seen in 7 (21.2%) patients. Medium prednisone dose at last follow-up was 3.2 ± 6.3 mg/die with a 85% decrease compared to preoperative dosage (27.8 ± 17.6 mg/die).
Discussion and conclusions: To date this is the largest cohort of JMG thymectomized patients analyzed for clinical outcomes over such a long follow-up period. Thymectomy in our cohort was safe andeffective, as evidenced by improvement of clinical scales and decreased steroid burden compared to pre-operative status, especially after 3 years. Moreover, clinical improvement and remission rate remained stableover time.”