Refractory chylous pleural effusion with systemic lupus erythematosus : Surgical treatment when steroid/immunosuppressant resistant.

We analyzed four cases of systemic lupus erythematosus (SLE) with chylous pleural effusion treated in our hospital from June 2013 to May 2017. The three females and one male ranged in age from 22 to 33 years. The length of disease course was 20 to 120 months. Treatment with glucocorticoids and immunosuppressants (cyclophosphamide or cyclosporine A) did not reduce chylous pleural effusion. Lymphatic obstruction was found in all patients after direct lymphangiography and the pleural effusion was significantly reduced after surgery. A literature search identified six previously reported patients with SLE and chylous pleural effusion. Five were sensitive to classic immunosuppression treatment, probably due to early intervention. However, as shown here, the thoracic duct can be involved in SLE, leading to an intractable chylous pleural effusion with no response to medical treatment. Surgery would be a better choice when lymphatic mechanical obstruction is found.