Systemic Lupus Erythematosus and Thymoma – A Double-Edged Sword

Abstract

The coexistence of systemic lupus erythematosus (SLE) and thymoma is rare. We describe 2 female patients with this combination. A 48-year-old woman presented with dyspnea due to a left pleural effusion. Her past history revealed over the previous 3 years the development of anemia, thrombocytopenia, alopecia, pericardial effusion and proteinuria. Four months prior to this hospitalization, the patient was first admitted due to purpura. At that time, laboratory tests revealed an elevated sedimentation rate, elevated titers of ANA and anti-DNA. Chest X-ray demonstrated a widened mediastinum, and upon operation an encapsulated thymoma was excised. Four months following the thymectomy, the patient is unresponsive despite high dose steroid therapy. Another patient, a 30-year-old woman, presented with SLE (cutaneous, arthritis, anemia, positive ANA and high titers of anti-DNA) and thymoma simultaneously. Six years after thymectomy the patient is in SLE remission. Thymectomy in mice prone to autoimmunity (NZB/W mice) has been shown to accelerate the autoimmune manifestations. Conversely, the opposite effect is seen in MRL/1pr mice. The immunological effect of adult thymectomy on the course of human SLE remains to be established, on a larger series of patients. It seems that the heterogenicity of human patients is exemplified by the contrasting effects of thymectomy for thymoma in SLE patients.

Thymoma and autoimmunity

“…16 The close interaction between these two diseases is emphasized by the observed remission of steroid therapy-resistant SLE disease following thymomectomy. 17 Notably, several manifestations of SLE have been associated with such comorbidity. In a recent meta-analysis of the link between the two diseases, the authors concluded that the possibility of thymoma should be considered and suspected among patients with late-age-onset SLE.…”

The coexistence of autoimmune rheumatic diseases and thymomas

“…4 In recent years, there have been a few case reports regarding the coexistence of ARDs and thymomas, such as SLE, RA, DM, SSc, and pSS. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Several reports documented the appearance of ARD after a thymectomy for a thymoma. Other reports described the appearance of a thymoma years after a patient was diagnosed with an ARD, as in our 2 cases.…”

Is There a Bridge Between Systemic Lupus Erythematosus and Thymomas?

“…[9] Hence, we began to doubt the accepted assumptions regarding the connection between immunoregulations and thymomas and SLE. [3][4][5][6][7] Additionally, we reviewed 24 cases in the literature published in English along with our own case, all of which had relatively detailed information about the patients' clinical features (Table 1), [4,5,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] and discovered that most of the patients underwent surgery as the primary treatment for the thymoma, with some combining the surgery with radiotherapy and/or chemotherapy. The outcome of SLE varied after the treatment with remission in five of the 24 patients, exacerbation in seven others, no reported changes in five more, and SLE occurrence in the other six.…”