Review Article

Rheumatic Fever Associated with Antiphospholipid Syndrome: Systematic Review

Table 1

11 cases published in the literature about the association between antiphospholipid syndrome and rheumatic fever.

References Number of casesAge, yearsFemale sexColorDisease duration, yearsRF manifestationsAPS manifestationsSLE associatedASOAntiphospholipid antibodyRF prophylaxis

Soeiro et al., 2012 [6]130100%Brown 1.5 RF-APSCarditis: + (with mitral valve replacement, right bundle branch block) 
Chorea: + 
Arthritis: −
Arterial: embolic stroke, AMI 
Venous: − 
Obstetric: − 
Thrombocytopenia: + 
Livedo: − 
Chorea: +
Yes786 UI/mLaCL IgM: + (14 MPL)Penicillin G benzathine 100%

Camargo et al., 2012 [7] 73 APS, 5 RF-APS37.4100%100% white5.6 RF
5.6 APS
Carditis (ECO abnormal): 100% 
Chorea: 2/5 
Arthritis: −
Arterial: stroke 4/5 
Venous: DVT 2/5 
Obstetric: − 
Thrombocytopenia: 1/5 
Livedo: − 
Chorea: 2/5
NoIncreased in acute phase: 100%aCL IgG: 2/5 
aCL IgM: 3/5 
LAC: 2/5 
Anti-β2GPI(IgG): 3/5
Penicillin G benzathine 100%

Alcock et al., 2011 [8]144100%27 RF
24 APS
Carditis: + (ECG: block first degree 
ECO: mitral thickening) 
Chorea: + 
Arthritis: +
Arterial: stroke 
Venous: − 
Obstetric: abortion, fetal and maternal hypertension 
Thrombocytopenia: + 
Livedo: − 
Chorea: +
No883 UI/mL, Anti-DNAase B: 960 UI/mLaCL: + 
LAC: + 
anti-β2GPI (IgG): 117 SGU/mL
Penicillin 100%

Izhevsky et al., 2004 [9]1290% -RF9 APSCarditis: + 
(block AV first and third grades) 
Chorea: − 
Arthritis: + 
Subcutaneous nodule: +
Arterial: AMI (with CRA and successful resuscitation) 
Venous: DVT 
Thrombocytopenia: − 
Livedo: − 
Chorea: −
NoIncreasedaCL IgG: + 
LAC: +
Penicillin G benzathine 100%

Amital et al., 1999 [10]145100%35 RF
12 APS
Carditis: + (admitted for mitral valve replacement) 
Chorea: + 
Arthritis: −
Arterial: stroke, splenic infarction 
Venous: − 
Obstetric: − Thrombocytopenia: − 
Livedo: − 
Chorea: +
YesLAC: +Prophylaxis with antibiotic

Fung et al., 1998 [11]2Case  1: 22
Case  2: 31
100%1 white, 
1 not described
Case  1: −
Case  2: 15 RF 
2 APS
Case  1:
Carditis: + (ECO: rheumatic valve disease, systolic murmur) 
Chorea: + 
Arthritis: − 
Case  2:
Carditis: + (mitral murmur) 
Chorea: + 
Arthralgia: +
Cases  1 and 2: 
Arterial: − 
Venous: − 
Obstetric: − 
Thrombocytopenia: − 
Livedo: − 
Chorea: +
Case  1: anti-dsDNA positive (35%)
Case  2: ANA positive
Case  1: normal, anti-DNAase B: 1280 UI/mL
Case  2: 451 UI/mL
Case  1: aCL IgM: + (14 MPL) 
Case  2: aCL: +
Case  1: oral penicillin
Case  2: sulfadiazine

AMI: acute myocardial infarction; AV: atrioventricular; CRA: cardiorespiratory arrest; DVT: deep vein thrombosis; ECG: electrocardiogram; ECO: echocardiogram.