FRI0593 Association of thymoma with autoimmune diseases in a series of 83 cases

Abstract

BackgroundThymoma is the most common neoplasm originated from the thymus gland and accounting for 50% of anterior mediastinal tumors. Within its clinical manifestations are included the loss of self-tolerance and the development of autoimmunity.ObjectivesTo study the frequency of autoimmune diseases (AD) in patients with thymoma and to describe their clinical characteristics and outcome.MethodsWe performed a retrospective observational study of a cohort of patients diagnosed with thymoma and followed-up in our center between January 1985 and September 2016. The variables evaluated included demographics, thymoma characteristics, clinical and analytical manifestations of autoimmunity, treatment and outcome.ResultsA total of 83 patients were included, 56.6% of them women, with a mean age at diagnosis of the thymoma of 58.4±15.8 years (range: 16–94), 31.3% of which corresponded to type I The classification of Masaoka, 39.1% to II, 17.2% to III and 10.9% to IV. There were one or more AD associated in 41 cases (49.4%). The most frequent diagnoses were myasthenia gravis (19), systemic lupus erythematosus (SLE) (4), subacute cutaneous lupus erythematosus (1), Sjögren's syndrome (1), rheumatoid arthritis (1), spondyloarthritis (1), sarcoidosis (1), hemolytic anemia (2), pernicious anemia (1), aplastic anemia (1), cutaneous limited systemic sclerosis (1), urticaria-vasculitis, erythroblastopenia (1), recurrent pericarditis (1), thyroid disease (2) and lichen planus (1). The diagnosis of AD preceded to thymoma in 38.2% of cases and was later in the remaining cases. In 4 cases there was also a concomitant primary immunodeficiency (variable common immunodeficiency 3, CD4 immunodeficiency 1). The most frequently identified autoantibodies were anti-acetylcholine receptor (14/41, 34.1%), anti-striated muscle (3/41, 7.3%), ANA (11/41, 26.8%), (3/41, 7.3%), rheumatoid factor (3/41, 7.3%), anti-thyroid (3/41, 7.3%), antiphospholipids (2/41, 9%) and anticentromere (1/41, 2.4%). In the comparison of patients with and without associated AD, no significant differences were found regarding age, sex or Masaoka classification. There were 6 deaths, 4 in group with associated AD and 2 in the group without AD, but without significant difference (p=0.3797).ConclusionsIn the analyzed population of patients with thymoma of our center, almost half of them developed AD, which in a major group preceded the diagnosis of neoplasia. The spectrum of autoimmunity associated with thymoma was quite broad, including organ-specific AD such as myasthenia gravis (which is most frequently described in the literature) and autoimmune cytopenias, but also to systemic AD, the most common being SLE. The autoimmunity study should be included in the assessment of the patient with thymoma as it could contribute to the early diagnosis of associated AD.Disclosure of InterestNone declared

Paraneoplastic glomerulopathy

“…Older reports, based mainly on autopsy series, suggest that up to 2% of patients with renal cell carcinoma have evidence of AA amyloidosis. 37,38 While admixture of amyloid with tumor lends credence to this association in some cases, 32 the absence of AA amyloidosis in 356 tumor nephrectomy specimens from contemporary series suggest that this association is much rarer in the modern era, perhaps in part due to earlier discovery and improved treatment. 39,40 Similarly, while amyloidosis was for many years considered to be the second most common renal lesion reported in patients with Hodgkin’s lymphoma (after MCD), it was identified in only 0.1% (n = 2) of patients cases in two largest series with more than 1700 patients with Hodgkin’s lymphoma.…”