A case of rheumatic fever complicating carditis detected by a newly-developed systolic murmur in an adult woman

A 62-year-old woman presented to a primary care doctor on January 2006 due to a sore throat and high fever, and had received medication for a common cold. She was referred to our hospital in February 2006 because of additional manifestations such as painful rashes on the lower limb similar to erythema nodosum and polyarthralgia on her feet, shoulder and finger joints. She was initially treated with an anti-inflammatory drug (NSAID) for polyarthritis but the symptoms did not improved. In addition, the serum level of anti-streptolysin O antibody (ASO) was elevated at the second visit more than that at the first visit. She was diagnosed to have rheumatic fever (RF) based on the polyarthritis, inflammatory data and an increase of the ASO level. She was treated with 10 mg a day of prednisolone (PSL) and sultamicillin tosilate. However, a systolic murmur that had been never noticed by previous auscultation appeared after the third hospital day and the mitral regurgitation was also detected on echocardiogram. She was then treated with 40 mg a day of PSL because of an appearance of the carditis due to RF. The increased PSL dose promptly improved the systolic murmur as well as the arthritis. This report presented an RF case with carditis detected by an development of the systolic murmur in an adult female.     

High-producing MBL2 genotypes increase the risk of acute and chronic carditis in patients with history of rheumatic fever

Rheumatic fever (RF) and its most severe sequela, chronic rheumatic heart disease (CRHD), are mediated by an abnormal immunological host response following a Streptococcus pyogenes oropharyngeal infection. Mannan-binding lectin (MBL), a collectin that activates complement, binds to N-acetylglucosamine, a molecule present on the streptococcus cell wall and on human heart valves. As high levels of MBL and MBL2 associated genotypes have previously been seen to be associated with CRHD, we investigated the association between MBL2 polymorphisms and the presence of acute carditis and arthritis in patients with a history of RF. Polymorphisms in exon 1 and in the X/Y promoter region of the MBL2 gene were determined by PCR-SSP in 149 patients with a history of RF and 147 controls. Genotypes associated with the high production of MBL (YA/YA and YA/XA) were more frequent in the patients with acute (26/35, 74%) and chronic carditis (79/107, 74%) when compared to the controls (79/147, 54%; OR 2.48, 95% CI 1.09-5.67, p=0.035 and OR 2.42, 95% CI 1.41-4.16, p=0.001, respectively). Logistic regression analysis showed that MBL levels >2800ng/ml increased the risk of CRHD (OR 2.91, 95% CI 1.41-6.03, p=0.003). Among the RF patients without cardiac sequela, YA/YA and YA/XA genotypes were significantly associated with acute carditis when compared to the patients without this clinical manifestation (26/28, 93% vs. 9/14, 64%, OR 7.22, 95% CI 1.18-43.98, p=0.031); on the other hand, arthritis was more frequently observed in those patients presenting MBL2 genotypes related to the low production of MBL (10/14, 71% vs. 10/28, 36%; p=0.048, OR 0.22, 95% CI 0.05-0.89). We concluded that MBL2 genotypes associated with the high production of MBL seem to be involved in the pathogenesis of rheumatic carditis and its progression to CRHD.     

Usefulness of echocardiography in detection of subclinical carditis in acute rheumatic polyarthritis and rheumatic chorea

Detailed echocardiographic analysis was performed in 10 children with first episode of acute rheumatic fever who presented with acute rheumatic polyarthritis or rheumatic chorea and had no clinically detectable evidence of active carditis. Significant changes were observed in the form of mitral valve prolapse with regurgitation in 3, aortic valve prolapse with regurgitation in 1 and mitral valve billowing without regurgitation in 1 patient each. A significant (p < 0.001) anterior mitral chordal elongation was observed in both the groups--rheumatic polyarthritis and chorea when compared with age and sex matched control subjects. Mitral annular diameter was found to be increased (p < 0.001) in patients presenting with polyarthritis alone. These observations of clinically silent but echocardiographically detectable element of carditis forms the basis of how patients of acute rheumatic fever develop permanent valvular deformities in their latter lives without revealing any cardiac affection earlier.     

Sphingomonas paucimobilis bacteraemia and shock in a patient with rheumatic carditis

Acute rheumatic fever (ARF) carditis is treated with steroids, which can cause changes in the cellular immune response, especially decreased CD3 (+) T cells. Nosocomial infections due to steroid use for treatment of ARF carditis or secondary to the changes in the cellular immune response have not been reported in the literature. Sphingomonas paucimobilis is a Gram-negative bacillus causing community- and hospital-acquired infections. It has been reported as causing bacteraemia/sepsis, pneumonia or peritonitis in patients with malignancies, immunosuppression or diabetes. We present a case with S. paucimobilis bacteraemia/sepsis and shock after administration of steroids for treatment of ARF carditis. We suggest early identification of the causative agent and appropriate adjustments of the treatment plan to avoid shock and possible mortality. This is the first reported case of S. paucimobilis bacteraemia/sepsis in the setting of steroid use for ARF carditis.