“Heart in DRESS”: Cardiac Manifestations, Treatment and Outcome of Patients with Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome: A Systematic Review

Abstract

Cardiac involvement in drug reaction with eosinophilia and systemic symptoms (DS) is rare but associated with high mortality. The aim of this research was to systematically review case reports by PRISMA guidelines in order to synthetize the knowledge of cardiac manifestations of DS. We identified 42 cases from 36 case reports. Women were two times more affected than men. Two-thirds of patients had cardiac manifestation in the initial phase of the disease, while in one-third of cases cardiac manifestations developed later (mean time of 70 ± 63 days). The most common inciting medications were minocycline (19%) and allopurinol (12%). In 17% of patients, the heart was the only internal organ affected, while the majority (83%) had at least one additional organ involved, most commonly the liver and the kidneys. Dyspnea (55%), cardiogenic shock (43%), chest pain (38%), and tachycardia (33%) were the most common cardiac signs and symptoms reported. Patients frequently had an abnormal ECG (71.4%), and a decrease in left ventricular ejection fraction was the most common echocardiographic finding (45%). Endomyocardial biopsy or histological examination at autopsy was performed in 52.4%, with the predominant finding being fulminant eosinophilic myocarditis with acute necrosis in 70% of those biopsied. All patients received immunosuppressive therapy with intravenous steroids, while non-responders were more likely to have received IVIG, cyclosporine, mycophenolate, and other steroid-sparing agents (60%). Gender and degree of left ventricular systolic dysfunction were not associated with outcomes, but short latency between drug exposure and the first DRESS symptom onset (<15 days) and older age (above 65 years) was associated with death. This underscores the potential importance of heightened awareness and early treatment.

Role of Endomyocardial Biopsy in Diagnostics of Myocarditis

“…Cardiac lesions are common (29%), usually presenting in a delayed manner. Allopurinol and minocycline have been frequently reported as causes (12% and 19%, respectively) [ 1 , 17 , 131 ]. Reports of parasitic infections mention malaria, schistosomiasis, filariasis and helminths.…”

Comparison of cyclosporine and systemic corticosteroid for treating drug reaction with eosinophilia and systemic symptoms syndrome: A retrospective 20‐year single‐centre study in South Korea

“…In particular, both cyclosporine and corticosteroid treatment showed rapid resolution of liver injury. Regarding liver involvement‐induced hepatic necrosis being the most common cause of death in DRESS syndrome, 5 it is encouraging that cyclosporine showed therapeutic efficacy for the resolution of liver injury compared to systemic corticosteroid, with similar hospitalized days and treatment period. However, serum creatinine levels and GFR did not change significantly.…”

Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome in a Child with Cystic Fibrosis

“…The liver, the kidney, and the lung injury can also occur [ 3 ]. DRESS may rarely affect the heart but is associated with high mortality [ 4 ]. The degree of symptoms and the extent of organ involvement in patients with the DRESS syndrome can range from mild to severe.…”