Disease severity and outcome in thymoma myasthenia gravis: a long-term observation study.
Thymomas occurring in myasthenia gravis (MG) are usually of the cortical subtype and are usually treated by thymectomy. However, the factors that influence MG outcome in thymoma MG patients are not known. In a long-term study, MG severity and treatment was observed in 24 thymoma and 24 non-thymoma MG patients for up to 30 years, and the occurrence of muscle autoantibodies was assayed. The rate of complete stable remission was low and did not differ between the two groups. There was no significant difference in MG severity between thymoma and non-thymoma MG patients at any time during the study. Titin and ryanodine receptor (RyR) antibody occurrence was significantly higher in thymoma MG patients. Four thymoma (all titin and RyR antibody positive) and two non-thymoma (both titin and one RyR antibody positive) MG patients died from MG-related respiratory insufficiency. Seventy percent of thymoma and 75% of non-thymoma MG patients were treated with immunosuppressive drugs. The number of patients who received plasmapheresis did not differ in those who were treated because of acute MG deterioration, irrespective of planned surgery.This study shows equal MG severity and outcome in thymoma and non-thymoma MG, but the presence of RyR antibodies in thymoma MG and titin/RyR in non-thymoma MG indicates a less favorable prognosis.
Romi
F
F
Department of Neurology, Haukeland University Hospital, Bergen, Norway. fredrik.romi@haukeland.no
Gilhus
N E
NE
Varhaug
J E
JE
Myking
A
A
Aarli
J A
JA
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D017668:Age of Onset; D001323:Autoantibodies / Q000276:immunology; D001379:Azathioprine / Q000627:therapeutic use; D003131:Combined Modality Therapy; D064211:Connectin; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009124:Muscle Proteins / Q000276:immunology; D009157:Myasthenia Gravis / Q000150:complications* / Q000401:mortality / Q000601:surgery; D011494:Protein Kinases / Q000276:immunology; D011950:Receptors, Cholinergic / Q000276:immunology; D019837:Ryanodine Receptor Calcium Release Channel / Q000276:immunology; D013934:Thymectomy; D013945:Thymoma / Q000209:etiology* / Q000401:mortality / Q000601:surgery; D013950:Thymus Gland / Q000473:pathology; D013953:Thymus Neoplasms / Q000209:etiology* / Q000401:mortality / Q000601:surgery; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D064211:Connectin; D007166:Immunosuppressive Agents; D009124:Muscle Proteins; D011950:Receptors, Cholinergic; D019837:Ryanodine Receptor Calcium Release Channel; C577285:TTN protein, human; D011494:Protein Kinases; D001379:Azathioprine