How to manage heart failure in patients with Sjögren’s syndrome?

From the Guidelines

Managing heart failure in patients with Sjögren's syndrome requires a comprehensive approach that includes enrolling patients in a multidisciplinary heart failure management program, initiating treatment with an ACE inhibitor, an MRA, an SGLT2 inhibitor, and a beta-blocker, as recommended by the 2024 ESC guidelines for patients with chronic coronary syndromes and heart failure 1.

Key Considerations

• The treatment should focus on reducing the risk of heart failure hospitalization and improving survival, with an SGLT2 inhibitor recommended for patients with heart failure with mildly reduced ejection fraction or heart failure with preserved ejection fraction 1.
• An ARB is recommended in symptomatic patients with heart failure and reduced ejection fraction who are unable to tolerate an ACE inhibitor or ARNI 1.
• Sacubitril/valsartan is recommended as a replacement for an ACE inhibitor or ARB in patients with heart failure and reduced ejection fraction to reduce the risk of heart failure hospitalization and cardiovascular death 1.
• Diuretics are recommended to alleviate symptoms, improve exercise capacity, and reduce heart failure hospitalizations in patients with signs and/or symptoms of congestion 1.

Additional Recommendations

• For patients with Sjögren's syndrome, immunomodulatory therapy may be necessary to manage the underlying autoimmune condition, which can contribute to cardiac dysfunction.
• Regular monitoring of cardiac function with echocardiography and vigilance for electrolyte abnormalities due to sicca symptoms and diuretic use are essential.
• The 2023 ACC expert consensus decision pathway on management of heart failure with preserved ejection fraction also emphasizes the importance of risk stratification, management of comorbidities, and symptom management with loop diuretic agents, SGLT2 inhibitors, and mineralocorticoid antagonists 1.

Treatment Approach

• Initiate standard heart failure treatment with ACE inhibitors, beta-blockers, and diuretics to manage fluid overload.
• Consider adding an SGLT2 inhibitor for patients with reduced ejection fraction.
• Treat the underlying Sjögren's syndrome with immunomodulatory therapy, which may include hydroxychloroquine or methotrexate.
• Use corticosteroids in cases where Sjögren's directly affects the heart through myocarditis or pericarditis, with gradual tapering.

From the Research

Management of Heart Failure in Patients with Sjögren's Syndrome

• The management of heart failure in patients with Sjögren's syndrome requires a multidisciplinary approach, involving cardiologists and rheumatologists 2.
• Patients with Sjögren's syndrome are at increased risk of cardiovascular morbidity, including heart failure, and should be screened for cardiovascular comorbidities and considered for preventive interventions 2.
• The mainstay therapies for heart failure in patients with reduced systolic function are angiotensin-converting enzyme (ACE) inhibitors and beta-blockers, with diuretics to control fluid balance 3.
• In patients who cannot tolerate ACE inhibitors, valsartan can be substituted, but should not be added to patients already taking an ACE inhibitor and a beta-blocker 3.
• Spironolactone is recommended in patients with New York Heart Association (NYHA) class III to IV symptoms despite maximal therapies with ACE inhibitors, beta-blockers, diuretics, and digoxin 3.
• Low-dose digoxin can be added to improve symptoms and possibly mortality, while the combination of hydralazine and isosorbide dinitrate may be useful in patients who cannot tolerate ACE inhibitors or valsartan due to hypotension or renal dysfunction 3.

Cardiac Involvement in Primary Sjögren's Syndrome

• Cardiac involvement is a common feature of primary Sjögren's syndrome, with increased morbidity and mortality due to cardiovascular disease 4.
• The risk of cardiovascular disease in patients with primary Sjögren's syndrome is associated with various risk factors, including age, gender, hypertension, diabetes mellitus, dyslipidemia, and disease duration 4.
• Early recognition and effective treatment of cardiovascular disease may play a crucial role in improving adverse cardiovascular prognosis in patients with primary Sjögren's syndrome 4.
• Echocardiographic studies may reveal valvular regurgitation, pericardial effusion, pulmonary hypertension, and increased left ventricular mass index in patients with primary Sjögren's syndrome, even in the absence of clinically apparent heart disease 5.

Treatment of Heart Failure in Patients with Sjögren's Syndrome

• Corticosteroids may be effective in treating autoimmune myocarditis and congestive heart failure in patients with primary Sjögren's syndrome 6.
• The treatment of heart failure in patients with Sjögren's syndrome should be individualized, taking into account the underlying cardiac involvement and the presence of other systemic manifestations of the disease 2, 4.
• A multidisciplinary approach, involving cardiologists, rheumatologists, and other specialists, is essential for the optimal management of heart failure in patients with Sjögren's syndrome 2, 4.