Appearance of systemic lupus erythematosus after thymectomy: four case reports and review of the literature

Abstract

The appearance of systemic lupus erythematosus (SLE) after thymectomy (or thymomectomy) is presented in four patients together with a comparative review of additional reports found in a Medline search for the years 1966-94 in the English and French literature. Fourteen women and two men of average age of 39 years (range 11-66 years) at presentation, developed SLE after thymectomy (11 patients) or thymomectomy (five patients). Half developed SLE within 3 years after surgery (range 3 months to 18 years). The most common SLE manifestation was polyarthritis occurring in 15 of 16 patients either at presentation or during the first year. Other frequent manifestations included skin rashes, fever, cytopenias and pleuritis. Two rare manifestations of SLE, optic neuritis and transverse myelitis, were reported in two patients. Thymic hormone activity was measured in one patient and was undetectable compared with normal controls. HLA studies in eight patients showed the combination of A1, B8 in four. In conclusion, the appearance of SLE after thymectomy or thymomectomy appears to be more than a coincidence. It may provide insights into the pathogenesis of SLE.

Thymoma and autoimmunity

“…19 Thus, it seems that although the exact mechanisms underlying both pathologies have yet to be elucidated, the association between thymoma and SLE is not incidental. 20,21 Acquired pure red cell aplasia may present as an acute self-limited disease, secondary to viral infection or drug-induced impairments, or as a chronic autoimmune form that may be associated with thymoma. 22 In the latter, damage to erythroid progenitors or precursor cells appears to be T cell-mediated, 23 and it is estimated that 5% of thymomas coexist with pure red cell aplasia.…”

Isolated Adrenocorticotropin Deficiency Associated with Painless Thyroiditis: A Case Report and Review of the Literature

“…Thymectomy is one of the treatment options for myasthenia gravis. Systemic autoimmune disorders including systemic lupus erythematosus, Hashimoto's thyroiditis, pure red cell aplasia, and idiopathic portal hypertension may occur many years after thymectomy in patients with myasthenia gravis [38,39]. Although the role of thymectomy in the onset of autoimmune disorders in patients with myasthenia gravis remains unclear, this sequence of events may be induced by altered regulatory T cell homeostasis [40].…”

A pilot study of the use of 2‐[18F]‐fluoro‐2‐deoxy‐D‐glucose–positron emission tomography to assess the distribution of activated lymphocytes in patients with systemic lupus erythematosus

“…Data on the effect of thymectomy on the course of human lupus are inconsistent. Surgery is performed mostly in cases of thymoma, and the published experience is limited to case reports and case series from which no final conclusions can yet be drawn (16,17). Some authors argue that thymoma is more common in patients with lupus (18), others that there is no such association (19).…”