|Year : 2021 | Volume
| Issue : 2 | Page : 151-153
Libman-Sacks endocarditis masquerading as suspected rat-bite fever
Rohit Rai, Rahul Singla, Narendra Omprakash Bansal
Department of Cardiology, Grant Medical College and Sir JJ Group of Hospitals, Mumbai, Maharashtra, India
|Date of Submission||02-Jan-2021|
|Date of Decision||04-Jan-2021|
|Date of Acceptance||07-Mar-2021|
|Date of Web Publication||25-Aug-2021|
Dr. Rohit Rai
Department of Cardiology, Grant Medical College and Sir JJ Group of Hospitals, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
Libman-Sacks endocarditis is usually associated with systemic lupus erythematosus and malignancies. It usually presents between ages 40 and 70 years. Our case was a seven year old child who was initially suspected and treated on lines of rat bite fever endocarditis based on history of rat bite but on further investigations patient was diagnosed with libman sacks endocarditis. High index of suspicion should be there for Libman-Sacks endocarditis for early diagnosis and treatment in cases with positive family history.
Keywords: Libman-Sacks endocarditis, rat-bite fever, systemic lupus erythematosus
|How to cite this article:|
Rai R, Singla R, Bansal NO. Libman-Sacks endocarditis masquerading as suspected rat-bite fever. Heart India 2021;9:151-3
| Introduction|| |
Libman-Sacks endocarditis is a type of nonbacterial thrombotic endocarditis. It is also known as verrucous or marantic endocarditis and is associated with systemic lupus erythematosus (SLE), malignancy, or antiphospholipid antibody syndrome. It commonly affects the mitral or the aortic valve and can complicate as embolic cerebrovascular infarct, peripheral artery embolism, or superimposed infective endocarditis. This term was coined in 1924 by Emanuel Libman and Benjamin Sacks. There is a significant correlation between duration and activity of SLE and Libman-Sacks endocarditis hence early diagnosis and treatment are needed to prevent complications.
It is most commonly associated with malignancies followed by SLE. It causes endothelial injury followed by deposition of platelet thrombi and inflammatory molecules leading to the formation of vegetations, especially on the undersurface of the valves.
Streptobacillus moniliformis is nonmotile, fastidious, pleomorphic, noncapsulated, and Gram-negative bacilli found in the oropharynx and nasopharynx of rodents. It is one of the two organisms causing rat-bite fever which manifests as fever, myalgia, rash, and arthralgia, the other being Spirillum minus.
Our case is unique as the patient was young and being treated for rat-bite fever endocarditis on basis of history of rat bite but on further investigations diagnosed with Libman-Sacks endocarditis. No such case has been reported.
| Case Report|| |
A 7-year-old child born to nonconsanguineous marriage at term by normal delivery. The patient cried at birth and achieved all developmental milestones on time. Patient was apparently asymptomatic 4 days back when he developed fever with respiratory distress. On examination, patient had heart rate of 140/min and blood pressure of 98/60 mmHg which was <50th centile for age. Patient had mild hepatomegaly and severe pallor. He had petechiae on palms and soles with hemorrhages under the nails [Figure 1]. On chest X-ray, there was cardiomegaly and electrocardiogram showed tachycardia. On two-dimensional echo, patient had a fixed mass of size 10 mm × 8 mm on undersurface of the anterior mitral leaflet and a mobile mass of 13 mm × 4 mm attached to it and a mobile mass of 4 mm × 4 mm on the tricuspid valve [Figure 2]. Patient had hemoglobin of 6.8 mg/dl and platelet count of 47,000 with white blood cell count of 16,000. Patient had positive immunoglobulin G (IgG) for dengue but negative IgM. Patient's father had given history of rat bite [Figure 3]. Patient was initially thought to have rat-bite fever endocarditis and culture for Streptobacillus moniliformis was sent which came negative. Patient was on anticoagulation and penicillin was started. Ultrasound of the abdomen and thorax showed mild ascites with congestive hepatomegaly. He had negative COVID 19 report and peripheral smear for malarial parasite was also negative. His dsDNA was positive and his mother was a known case of SLE. Patient was intubated on day 6 of admission and expired on day 10.
| Discussion|| |
The prevalence of vegetations in Libman-Sacks endocarditis is 1 in 10 patients. It most commonly affects people between the age of 40–80 years. Our patient was a 7 years old child. It is associated with leaflet thickening and on histopathology vegetations appear in clusters and have verrucous or granular appearance. Mitral valve is more commonly affected than the aortic valve. In our case also mobile vegetations were attached to the mitral valve.
Patients are usually asymptomatic but clinical manifestations are usually due to embolism. Peripheral embolism can present as cold limbs, mesenteric ischemia can present as severe abdominal pain and cerebrovascular embolism can present as stroke or transient ischemic attack. Our case also had petechiae on palms and soles with hemorrhages under the nails. Patients with SLE present as malar rash, pleuritis, pericarditis, or nephrotic syndrome. Patients with antiphospholipid antibody syndrome present with recurrent miscarriages or venous or arterial thrombosis. They can present with cerebrovascular disease manifesting as major neuropsychiatric syndromes of stroke, transient ischemic attack, confusional state or seizures, acute or cumulative neurocognitive dysfunction, and focal lesions in the brain on MRI.,,,, Our case had no seizures and had no cognitive dysfunction.
Blood cultures, complete blood count, and complete metabolic panel should be done. Lupus anticoagulant and antiphospholipid antibody workup should be done. In our case, blood cultures came negative, but anti-dsDNA was strongly positive.
Anticoagulation should be done to prevent embolization. Valvular surgery should be planned if significant valvular dysfunction is present. Immunosuppressive medications may be used to treat the underlying condition, corticosteroids may not be beneficial and may even cause valve damage. Our case was being anticoagulated and surgery was planned but patient did not survive.
Only 22 cases of Streptobacillus moniliformis bacteremia have been published so far and fewer with embolic manifestations out of which ten have expired. Current recommendation is intravenous penicillin G 1.2 million units per day. Endocarditis due to Streptobacillus moniliformis is a rare complication and early diagnosis and prompt treatment with penicillin are important. Our patient was also started on penicillin after taking history of rat bite but the cultures came negative.
One case of dengue hemorrhagic fever has been shown to be associated with Methicillin-resistant Staphylococcus aureus superinfection associated infective endocarditis. In our case, the dengue IgG antibody was positive and blood cultures were negative.
Our case was initially treated as rat-bite fever endocarditis but culture report came negative. On workup for SLE, we diagnosed him as a case of Libman-Sacks endocarditis.
| Conclusion|| |
Early diagnosis and treatment are key to the management of Libman-Sacks endocarditis. High index of suspicion should be there in those with positive family history.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.
Narendra O Bansal supervised the patient care and edited the manuscript. Rohit Rai performed the echocardiography, was involved in patient care and wrote the case report. Rahul Singla was involved in the patient care and edited the manuscript.
Informed consent was obtained from the patient. Patient anonymity has been maintained.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]