Asian Cardiovasc Thorac Ann 2006;14:e115-e117
© 2006 Asia Publishing EXchange Ltd
Infective Endocarditis Associated with Sjögren’s Syndrome
Koichi Sughimoto, MD,
Kiyoharu Nakano, MD,
Akihiko Gomi, MD,
Hayao Nakatani, MD,
Yoshitsugu Nakamura, MD,
Atsuhiko Sato, MD
Department of Cardiovascular Surgery, Kanto Medical Center NTT EC, Tokyo, Japan
For reprint information contact: Koichi Sughimoto, MD Tel: 81 33 448 6111 Fax: 81 33 448 6566 Email: cedarh7dion{at}yahoo.co.jp, Kanto Medical Center NTT EC, 141-8625, 5-9-22 Higashi-Gotanda, Shinagawa-ward, Tokyo, Japan.
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ABSTRACT
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Sjögren’s syndrome is one of the major autoimmune diseases, however infective endocarditis associated with Sjögren’s syndrome has not previously been reported. A patient with Sjögren’s syndrome associated with aortic valve regurgitation due to infective endocarditis, underwent successful aortic valve replacement. Patients with Sjögren’s syndrome tend to be at a higher risk of intraoral infections due to diminished secretion of saliva. Particular care must be taken of Sjögren’s syndrome patients as they can also be at an increased risk of infective endocarditis.
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CASE REPORT
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A 42-year-old male was admitted to our hospital presenting with dyspnea, orthopnea, and severe cough. Three months before admission, he had undergone an odontectomy because of severe tooth decay. In a previous hospital, the patient was diagnosed with Sjögren’s syndrome by positive Schirmer test, residue of iodine contrast medium in the salivary gland, and positive anti-SS-A antibody. On physical examination, a diastolic murmur (Levine III of VI) was heard at the second right sternal border. Dry, belt-shaped skin pigmentation was observed on the lower abdomen. There was non-pitting edema in the lower extremities. A Roth spot was observed on the eyegrounds. An electrocardiogram showed a heart rate of 95 beats·min–1, normal sinus rhythm and normal axis deviation. During the night, a II degree atrio-ventricular block was transiently evident. Echocardiography revealed severe aortic regurgitation, a dilated left ventricle and a 6 mm abscess with high-echo density on the right coronary cusp. These findings were consistent with infective endocarditis (IE).
Data on admission revealed a C-reactive protein (CRP) level of 4.7 mg·dL–1, normal white blood cell count of 5,300 mU·L–1, severe anemia with a low hemoglobin level of 6.5 g·dL–1, hematocrit of 19.9 %, and slightly elevated lactate dehydrogenase of 301 IU·L–1. Low haptoglobin and elevated reticulocyte led us to suspect hemolytic anemia, however a direct Coombs’ test proved negative. Immunological investigation revealed elevated anti-nuclear antibody, anti-deoxyribonucleic acid antibody, and rheumatoid factor. No antibiotics had been previously administered to the patient until benzylpenicillin potassium (PCG) was administered after taking several blood cultures. Only 1 of 6 consecutive blood cultures was positive, and revealed that the causative bacillus was Staphylococcus saccharolyticus, one of the coagulase negative staphylococci sensitive to PCG. To control congestive heart failure, diuretics and catecholamine agents were administered, and red cells were transfused to correct the anemia.
On the 14th day after admission, cough and orthopnea were aggravated and became medically uncontrollable. A chest X-Ray showed deteriorating pulmonary congestion. A Swan-Ganz catheter was inserted into the cervical vein, showing elevated pulmonary arterial pressure (50–60/13–22 mm Hg). As a result, an urgent operation was required.
Under usual anesthesia, the pericardium was opened, and cardiopulmonary bypass was established. The blood was cooled to achieve cardiac arrest, the ascending aorta was incised, and cardioplegia was administered into the coronary ostium. The aortic valve was carefully excised (Figure 1
). The aortic valve was a tricuspid valve, and no chronic inflammatory lesion was found. Vegetation was observed between the right coronary cusp and the non-coronary cusp, and the non-coronary cusp was torn. A drop lesion was observed on the anterior leaflet of the mitral valve. The vegetation was carefully excised. Aortic valve replacement was performed, with an ATS #21 mm aortic valve (ATS Medical Inc. Minneapolis, USA), onto the para-annular position so as not to leave pledgets in the left ventricular side. Weaning from cardiopulmonary bypass was uneventful. The patient was extubated on the second day in the intensive care unit.
Pathological findings revealed complete destruction of the layers of the aortic valve and dead bacillus bodies with elastic fiber stain. With hematoxylin and eosin stain, inflammatory cells; mainly neutrophils, invading into the layers were observed, while few lymphocytes were evident (Figure 2). Whether the occurrence of chronic inflammation of the aortic valve was associated with Sjögren’s syndrome was unknown from the pathology.
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Figure 2. HE stain of the excised aortic valve; neutrophil invasion was found, however evidence of lymphocytes was not seen.
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The antibiotics were continued for 21 days after the operation. The patient was discharged on the 28th postoperative day when the CRP became negative. The patient is currently symptom-free and undergoing regular follow-up.
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DISCUSSION
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Preoperatively, the patient presented with severe anemia. In 70% to 90% of all cases of IE, anemia is present and deteriorates with the duration of the illness. Rheumatoid factor is also present in 50% of patients with IE.1 Low haptoglobin and elevated reticulocyte led us to suspect hemolytic anemia, but the direct antiglobulin test (DAT) was negative. In this case, presumably, the anemia may not have been associated with autoimmune hemolytic anemia (AIHA) but simply with IE, although AIHA with negative DAT rarely exists.2,3
Infective endocarditis is occasionally associated with immunodeficiency; in patients chronically medicated with steroids, or those who have diabetes mellitus or cancer.4 The patient’s aortic valve was tricuspid, and no congenital morphological malformation was found. This is a case in which bacterial endocarditis occurred in a native valve, the incidence of which is estimated at 12.8%.5 Pathological research revealed that inflammatory cells (mainly neutrophils) were invading the layers while few lymphocytes were found. Whether the occurrence of chronic inflammation of the aortic valve was associated with Sjögren’s syndrome was unknown from the pathology.
Sjögren’s syndrome is one of the major autoimmune diseases and manifests as diminished lacrimal and salivary gland function, xerostomia, keratoconjunctivitis sicca, and parotid gland enlargement.6 In Sjögren’s syndrome, the main cause for the sicca syndrome is presence of antibody to the gland antigen. B-cell activation is a consistent finding as B and T cells invade and destroy target organs. Reduction of salivary volume and the subsequent loss of the antibacterial properties of saliva accelerate bacillus infection, tooth decay, and periodontal disease.6 The patient underwent odontectomy three months before admission to the hospital. This could have been the main causative etiology of IE in this patient.
The risks of IE are still poorly defined.7 To our knowledge, the association between IE and Sjögren’s syndrome has not previously been reported, but Sjögren’s syndrome is considered one of the high risk factors for IE. Patients with Sjögren’s syndrome need to be cautious when being treated for tooth caries. Prior to odontectomy, appropriate antibiotic prophylaxis according to the guidelines should be instigated, and careful follow-up is necessary.8
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REFERENCES
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- Donald Kaye, Sherwood L. Gorbach, John G. Bartlett, Neil R. Blacklow. Infective endocarditis, In: Infectious disease third edition, Lippincott Williams & Wilkins 2004: 569–81.
- Anne T. Neff. Autoimmune Hemolytic Anemias In: 11th edition Wintrobe’s clinical hematology, 2003:1157–82.
- Onoe M, Magara T, Yamamoto Y. Cardiac operation for a patient with autoimmune hemolytic anemia with warm-reactive antibodies. Ann Thorac Surg 2001;71:351–2.[Abstract/Free Full Text]
- Netzer RO, Altwegg SC, Zollinger E, Tauber M, Carrel T, Seiler C. Infective endocarditis: determinants of long term outcome. Heart 2002;88:61–6.[Abstract/Free Full Text]
- John W. Kirklin, Barratt-Boyes. Infective endocarditis. In: Cardiac Surgery third edition, Chirchill Livingstone Inc. 2003;689–711.
- Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 2004;164:1275–84.[Abstract/Free Full Text]
- Strom BL, Abrutyn E, Berlin JA, Kinman JL, Feldman RS, Stolley PD, et al. Risk factors for infective endocarditis: oral hygiene and nondental exposures. Circulation 2000;102:2842–8.
- Dajani AS, Taubert KA, Wilson W, Bolger AF, Bayer A, Ferrieri P, et al. Prevention of bacterial endocarditis. Recommendations by the American Heart Association. JAMA 1997;277:1794–801.[Abstract/Free Full Text]
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