Healing of non – infective endocarditis under immunosuppression

INTRODUCTION

Antiphospholipid syndrome (APS) is an immune coagulopathy. As a rare occurrence, non – infectious valvular heart disease can be diagnosed as Libman - Sacks endocarditis (nonbacterial thrombotic endocarditis; NBTE) in the presence of antiphospholipid antibodies (aPL) and must not be overlooked.

CASE PRESENTATION

A 27 – year ­– old female presented with night sweats, weight loss, reduction in performance and dizziness. Her past medical history showed an APS, acquired 5 years earlier in connection with a miscarriage within the 29^th week. Other findings such as recurring thrombosis / embolisms or signs of connective tissue disease were not reported. She gave birth to two healthy children. One before and one after the diagnosis of APS. Prior to her presentation, the patient took no permanent medication. Her vital signs were stable, and the physical and neurological examination remained non – remarkable. A TTE and then a TEE were performed showing typical changes in accordance with a non – infectious endocarditis (Libman – Sacks), with a thrombotic deposit measuring 16 x 9 mm on the mitral valve on the PML (P2 and P3) as well as on the tip of the AML (A1) with increased LV inflow and involvement of the posteromedial papillary muscle (Figure 1). Only a trace of mitral regurgitation was present without irregular left / right ventricular function, segmental wall – motion abnormalities or other valve malfunctions. Corresponding to that, a normal BNP could be measured (21pg/ml). A cardiac MRI ruled out: late enhancement and myocardial edema as well as thrombus formation in the left / right ventricle and atrial appendage. Important laboratory findings included negative ANAs and normal anti – dsDNA, anti – SSA / anti – SSB antibodies, p – ANCA / c – ANCA and anti – cardiolipin antibodies (IgG, IgM). CRP and blood count were normal. Multiple blood cultures remained sterile. The patient was put on a vitamin K antagonist (target INR 2 – 3) and a combined immunomodulation with hydroxychloroquine (200mg / 400mg on alternating days) and prednisolone (initial dose of 15mg once daily with weekly reduction by 2.5mg until 5mg once daily for a month and final reduction to 2.5mg once daily followed by termination after four months). She was discharged with an INR of 2.9. The symptoms of the patient resolved completely. The patient was followed up regularly. Within 3 months after starting the treatment, the patients echo showed a normal mitral valve function (only trace of regurgitation) with only a slight regional thickening of the PML measuring 3 x 3mm without additional malfunctions / abnormalities of the other valves (Figure 2). Over 7 years, she remained clinically stable and asymptomatic on hydroxychloroquine (200mg once daily) and a vitamin K antagonist (target INR 2 – 3). She became pregnant again with her third child. Her medication was continued until the 5^th week of the pregnancy (gestational age) and then interrupted. Anticoagulation remained but was changed to 20.000 IE fragmin s.c. daily. Her post – pregnancy medication was prescript in the same form and dosage after giving birth as it was before the pregnancy. 

CONCLUSION

In the case of our patient with APS and NBTE, our treatment concept of anticoagulation and immunosuppression seems to be a promising option to guarantee effective and safe management of the underlying disease and its initial symptoms. To this day, this therapy remains successful over 7 years of follow – up.