Clinical presentation, prognosis, and treatment strategies for thymoma-associated multiorgan autoimmunity: A pooled analysis.

BACKGROUND

Thymoma-associated multiorgan autoimmunity (TAMA) is a rare autoimmune disease related to thymoma, defined as a disorder of the skin, liver, and gastrointestinal tract similar to graft-versus-host disease.

METHODS

We performed a systematic review and a pooled analysis of confirmed TAMA cases published in the literature. Additionally, a case of TAMA diagnosed and treated at our institution was also described and included.

RESULTS

We identified 44 eligible publications providing data on 50 patients. At presentation, 82 % of patients had cutaneous lesions, 46 % gastrointestinal symptoms, 26 % hepatic manifestations, and 2 % had other less common manifestations. The main strategy for TAMA was to control neoplastic disease, however, only 20 % of patients underwent surgery and 10 % received chemotherapy. Immunosuppressive therapy was the complementary approach, with 78 % of patients receiving corticosteroids and 54 % receiving other immunosuppressive drugs. In the pooled analysis, the median overall survival from diagnosis of TAMA was 7 months (95 % CI: 0.03-14.0 months). An explorative analysis of potential prognostic factors showed that older age had a protective effect (HR 0.37; 95 % CI, 0.14-0.95; p = 0.04) and active treatment of thymoma may prolong survival of patients with TAMA (HR 0.30, 95 % CI: 0.08-1.00, p = 0.05).

CONCLUSIONS

This pooled analysis shows that TAMA's onset is associated with a poor prognosis in patients with thymoma and needs to be recognized early to provide effective treatments. Combining antineoplastic and immunosuppressive therapy is the most effective way to manage TAMA symptoms and improve patient outcomes.