Published online May 26, 2020. doi: 10.12998/wjcc.v8.i10.2056
Peer-review started: January 31, 2020
First decision: March 18, 2020
Revised: March 21, 2020
Accepted: April 18, 2020
Article in press: April 18, 2020
Published online: May 26, 2020
Processing time: 116 Days and 5.2 Hours
Fulminant lupus myocarditis is a rare but fatal manifestation of systemic lupus erythematosus. Aggressive immunosuppressive treatments are important in its successful management. However, they can significantly damage the immunity and are associated with a considerable risk of infection development and spread. We present a rare and complicated case of a 20-year-old female diagnosed with fulminant lupus myocarditis accompanied by pneumonia. The patient was successfully treated with plasma exchange (PE) for fulminant lupus myocarditis.
A 20-year-old Chinese woman presented to the Hematology Department complaining of fatigue and knee pain. Blood test showed anemia and thrombocytopenia. On the second day of hospitalization, she was transferred to the ICU due to dyspnea and hypotension. Autoimmune profiles showed hypocomplementemia and positive antinuclear antibodies. Computer tomography showed an enlarged heart and pneumonia. Ultrasound revealed an enlarged heart with a low left ventricular ejection fraction. Fulminant lupus myocarditis with cardiogenic shock was initially considered. Due to the accompanying pneumonia, aggressive immunosuppression was contraindicated. Her cardiac function remained critical after the initial therapy of intravenous immunoglobulin and corticosteroids at a conventional dose, but she responded well to later PE therapy plus corticosteroids administration. The patient fully recovered with normal cardiac function.
This case indicates that PE is a valuable treatment choice without adverse effects of immunosuppression in patients with fulminant lupus myocarditis and coexisting infection.
Core tip: Fulminant lupus myocarditis with cardiogenic shock is rare but life-threatening. Although aggressive immunosuppressive treatment plays an important role in its successful management, it may lead to a considerable risk of infection development and spread. Plasma exchange (PE) can quickly remove antibodies and antigen-antibody complexes from lupus patients without adverse effects of immunosuppression and infection spread. Here, we present a rare and complicated case of a female patient successfully treated with PE for fulminant lupus myocarditis accompanied by pneumonia. This case indicates that PE is a valuable treatment choice without immunosuppression, especially for severe lupus myocarditis patients complicated by infection.