Cardiogenic shock in autoimmune rheumatologic diseases: an insight on etiologies, management, and treatment outcomes

Autoimmune rheumatological disorders are known to have an increased risk for cardiovascular diseases including coronary artery disease (CAD), myocarditis, pericarditis, valvulopathy, and in consequence cardiogenic shock. Data on cardiogenic shock in rheumatological diseases are scarce; however, several reports have highlighted this specific entity. We sought to review the available literature and highlight major outcomes and the management approaches in each disease. Systematic literature search, including PubMed, Ovid/Medline, Cochrane Library, and Web of Science, was conducted between January 2000 and December 2009. We reviewed all cases reporting cardiogenic shock with rheumatologic conditions, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), Takayasu's arteritis (TA), granulomatosis with polyangiitis (GPA), giant cell arteritis (GCA), and antiphospholipid syndrome (APS). We selected 45 papers reporting a total of 48 cases. Mean age was 39 ± 7.3 years and 68.8% were females. Most common rheumatologic conditions associated with cardiogenic shock were SLE (31%), GPA (23%), TA (14.6%), APA (10.4%), and RA (8.3%). Cardiogenic shock was found to be caused by eosinophilic myocarditis in 58% of cases, CAD in 19% of cases, and valvulopathy in 6% of cases. Most patient required high-dose steroids and second immunosuppressant therapy. Mechanical circulatory supported was required in 23 cases, IABP in 16 cases, and ECMO in 12 cases. Complete recovery occurred in 37 patients while 9 patients died and 2 required heart transplant. Responsible for two-thirds of cases, eosinophilic myocarditis should be suspected in young cardiogenic shock patients with underlying rheumatologic conditions. Lupus and GPA are the two most common conditions.