Poly-autoimmunity in patients with myasthenia gravis: A single-center experience

Abstract

We evaluated the co-occurrence of autoimmune diseases (ADs) in a large population of myasthenia gravis (MG) patients from a single center. Our survey included 984 patients, 904 with anti-acetylcholine receptor antibodies and 80 with anti-muscle specific kinase antibodies. The anti-acetylcholine receptor positive population included patients with early-onset (age at onset ≤50 years), late-onset and thymoma-associated disease. Follow-up ranged 2–40 years. Two-hundred and fourteen ADs were diagnosed in 185 patients; 26 of them had two or more ADs in association with MG. Thyroid disorders were the most common and, together with vitiligo and thrombocytopenia, occurred in all disease subsets. Otherwise, there was a broad variability with partial overlap among patient groups. The highest rate of ADs was observed in early-onset patients, while clusters, i.e. 2 or more ADs other than MG in the same individual, were more common among thymoma cases. Thirty-four diseases were diagnosed at the same time, 88 occurred before and 92 after the onset of MG. On multivariate analysis, immunosuppressive treatment was the only independent variable which negatively influenced the risk of developing other ADs in our cohort.     

Myasthenia gravis with thymoma associated with occult thyroid carcinoma

We report herein a case of myasthenia gravis associated with thymoma and occult thyroid carcinoma with metastasis to a cervical lymph node, which was treated in two stages by a total thymectomy and radical dissection of the bilateral neck after a total thyroidectomy. A 48 year old woman was admitted with right blepharoptosis and hypodynamia of the proximal muscles. After various examinations, a diagnosis of myasthenia gravis was made. The association of thymoma was clarified upon CT scanning and a total thymectomy performed. However, after surgery, swelling of the cervical lymph nodes became apparent and a biopsy revealed metastasis of thyroid carcinoma in the lymph nodes. A diagnosis of occult thyroid carcinoma with metastasis to a cervical lymph node was subsequently made and a total thyroidectomy and radical dissection of the bilateral neck performed 37 days after the total thymectomy. The patient's postoperative course was uneventful and for the time being, no recurrence is expected. It is considered better to perform two-stage operations for easier and safer postoperative management when myasthenia gravis associated with occult thyroid carcinoma is treated, although it depends on the development of thyroid carcinoma being occult.     

Fulminant myasthenia gravis and polymyositis after thymectomy for thymoma

Two days after thymectomy for thymoma a patient developed myasthenia gravis and polymyositis; these rapidly progressed to a near total paralysis.     

Alopecia Areata Associated with Myasthenia Gravis and Thymoma: A Case of Alopecia with Marked Improvement Following Thymectomy and High Level Prednisolone Administration

A 57-year-old Japanese woman who suffered from alopecia areata associated with myasthenia gravis (MG) and thymoma responded well to thymectomy and high doses of glucocorticosteroid administration. Several treatments for alopecia areata including administration of systemic prednisolone were attempted, but loss of hair on the scalp progressed. After thymectomy and high level glucocorticosteroid administration for MG, the lesions on the scalp improved within four weeks. Consequently, we suggest that this thymectomy and high level glucocorticosteroid administration assisted in improving the immune dysfunction causing the alopecic lesions in this patient.     

Associated conditions in myasthenia gravis: response to thymectomy

To compare the response of thymectomy in patients with associated conditions (PWAC) and without associated conditions (PWOAC). Comparative, retrospective. 198 patients with the established diagnosis of myasthenia gravis who had a thymectomy between 1987 and 2000, and who were folowed up for at least 3 years. We formed two groups, one with associated conditions and the second without associated conditions. The patients were divided into four groups: (i) patients in remission, (ii) patients with improvement, (iii) patients without changes, and (iv) patients whose condition worsened. Associated conditions (AC) were found in 49 patients (26%). The main associated conditions were hyperthyroidism in 16 patients (33%) hypothyroidism in seven (14%), rheumatoid arthritis in five (10%) and hypothyroidism and Sjogren syndrome in three (6%). Concerning the response of thymectomy, 13 patients WAC showed remission (27%), vs. 54 patients WOAC (39%). Twenty patients WAC showed improvement (41%) vs. 46 WOAC (33%). Thirteen patients WAC had no changes (27%) vs. 37 WOAC (26%). Finally, in three patients WAC their condition worsened (6%) vs. three WOAC (2%). The response to thymectomy was high (69%) in both groups. We did not identify significant differences.     

胸腺瘤切除术后肌无力危象和抢救

1984年10月～1990年6月,手术治疗胸腺瘤30例,均经病理证实。其中5例术后发生重症肌无力,4例突发肌无力危象,经抢救2例成功、2例失败。本文着重就抢救的经验及教训进行讨论和总结。     

Defective suppressor function of spleen cells by neonatal thymectomy and persistence of experimental auto-immune hepatitis in mice

The effects of neonatal thymectomy on a model of auto-immune hepatitis prepared by immunizing A/J mice with syngeneic crude liver proteins were studied. Hepatitis was more severe in neonatally thymectomized mice (Group B) than in non-thymectomized controls (Group A). Changes in the hepatic lobules were reduced in both groups 3 months after the final immunization, but inflammation around the portal areas tended to persist in Group B; only this group showed frequent infiltration of mononuclear cells accompanied by destruction of the limiting plate in the portal area. The serum level of auto-antibody to liver-specific membrane lipoprotein complex (LSP) and delayed-type hypersensitivity (DTH) to LSP were higher in Group B than in Group A. The auto-antibody to LSP was positive in both groups 3 months after the final immunization. Although it was significantly reduced in Group A compared with the level 3 days after the final immunization, it remained high in Group B. Liver damage, production of the auto-antibody to LSP and DTH to LSP were reduced by adoptive transfer of normal murine spleen cells, but the spleen cells of neonatally thymectomized mice showed no such effects. These results suggest that normal spleen cells possess the capability of suppressing auto-immune hepatitis, and the defect of this capability due to T-cell dysfunction by neonatal thymectomy contributes to the promotion and persistence of auto-immune hepatitis.     

Thymoma and agranulocytosis: two case reports and literature review

Thymoma with agranulocytosis is a rare association. We describe two cases of agranulocytosis presenting with sepsis which were both found to have coincident benign spindle cell thymomas. One case, associated with promyelocyte arrest and hypogammaglobulinaemia, was treated successfully with granulocyte colony-stimulating factor (G-CSF). Thymectomy had no effect. The other case, associated with complete myeloid aplasia, proceeded to a fatal outcome after failure of treatment with granulocyte-macrophage colony stimulating factor (GM-CSF), plasmapheresis, thymectomy, intravenous immunoglobulin, cyclophosphamide and methylprednisolone. We also review the literature of thymoma in association with agranulocytosis.     

胸骨横切口胸腺切除术治疗重症肌无力(附19例报告)

目的 探讨胸骨横切口胸腺切除术治疗重症肌无力的方法。方法 采用胸骨横切口（8－10cm)不进入胸腔的方法，进行胸腺瘤或胸腺组织的清除，包括前纵隔脂肪组织，手术共进行19例。结果 无死亡病例，术后症状减轻或消失占84％。结论 胸骨横切口胸腺切除术可减轻患者的创伤及痛苦，有利于术后近期恢复及减少气管切开术后的并发症，实为一种较好的临床治疗方法。     

Structure and function of the thyroid gland after thymectomy

Tomsk Medical Institute. (Presented by Academician of the Russian Academy of Medical Sciences N. V. Vasil'ev.) Translated from Byulleten' Éksperimental'noi Biologii i Meditsiny, Vol. 114, No. 9, pp. 329–332, September, 1992.