data derived from patients with lupus or mixed connective tissue disease [52, 53], it has been suggested that patients with pSS-associated PAH and NYHA class I and II should be initially considered for treatment with immunosuppressants alone, such as glucocorticoids with or without cyclophosphamide or azathioprine for a period of 4–6 months [6]. If response is documented on hemodynamic evaluation, a maintenance regimen consisting of azathioprine or mycophenolate mofetil should then be continued [6]. Patients not responding to a trial of immunosuppressants should be switched to standard PAH therapy. For patients in NYHA class III or IV, a regimen consisting of both immunosuppressant and standard PAH medications is recommended for a period of 4–6 months. Maintenance immunosuppressant therapy is then continued when on hemodynamic evaluation response is documented; if there is no response, immunosuppression is replaced with combined standard PAH therapy (Fig. 13.1).

In the patient with symptoms of cardiac autonomic neuropathy, exclusion of potentially reversible causes of orthostatic hypotension is the first and most important management step. Non-pharmacological therapy such as use of elastic stockings or elevation of the head of the bed, avoiding hypotensive drugs (e.g., diuretics, vasodilators) and a high-salt high-fluid diet may be useful. A synthetic mineralocorticoid, 9-a-fluorohydrocortisone, should be considered in patients not responding to nonpharmacological treatment. A sympathomimetic agent, such as midodrine, can be added to fludrocortisone acetate if the patient remains symptomatic. The role of immunosuppression in treating pure autonomic neuropathy is entirely unknown [37].

13.11Conclusion

Symptomatic cardiovascular involvement occurs rarely in primary Sjögren’s syndrome. Cardiovascular complications that may occasionally require clinical attention include myocarditis, pulmonary arterial hypertension, pericarditis, and autonomic cardiovascular dysfunction manifested primarily with orthostatic hypotension and/or syncope. Equally rare are other abnormalities, which are mostly asymptomatic and include left ventricular diastolic dysfunction and mild valvular abnormalities. Management of cardiovascular complications in these patients is based on clinical knowledge acquired from treating similar complications in other autoimmune diseases.

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