One of the most prominent features in patients with APS is headache. This symptom, a common complaint of APS patients in clinical practice, can vary from classic intermittent migraine to almost continuous incapacitating headache. The association of migraine and aPL is controversial with widely varying results from different series.
One of the major problems is that headaches, often non-migrainous, have been loosely termed "migraine," and these headaches may precede or accompany TIAs or CVAs.
The available data suggest an association between the migraine-like phenomena and aPL, but not between migraine headache and aPL. Thus, aPL should be included in the investigation of all young individuals with migraine irresponsive to conventional treatment.
With regard to the treatment, it usually responds to conventional therapy. In some cases it has been reported to improve remarkably with low-dose aspirin; very resistant cases may require heparin or warfarin as alternative therapy.
7.2 Cardiac Manifestations
Around 40% of patients with APS may develop cardiac manifestations, but significant morbidity appears in less than 10% of these patients (Table 7.2).
Heart valve lesions are the most common cardiac manifestations described in patients with aPL. According to a review performed by Nesher et al., 36% of PAPS patients and 35% of SLE patients had valvulopathy. The same review showed that 48% of aPL-positive SLE patients had valvulopathy, compared with only 21% of aPL-negative SLE patients. Although most cases are symptomless, some cases evolve to severe valvular dysfunction resulting in cardiac failure, sometimes requiring valve replacement.
Thickening of the valve leaflets is the most common lesion detected by echocardiography in both SLE and PAPS patients (Fig. 7.4). The mitral valve is involved most commonly, followed by the aortic valve. Most thickened valves develop hemody-namic abnormalities, occurring roughly in 25% of all patients with SLE or PAPS. The pathogenesis of valvular abnormalities in APS is not entirely clear. It has been postulated that
Table 7.2. Cardiac manifestations in APS.
Coronary bypass graft and angioplasty occlusions. Other: Intracardiac thrombus
Acute/chronic cardiomyopathy (due to microangiopathy)
aPL could cause straightforward valvular or endothelial injury unrelated to clinical severity of the disease.
Most cases are clinically silent and detected by either chest auscultation, echocardiography, or at autopsy. Nevertheless, 5% of all SLE and APS patients develop severe mitral or aortic regurgitation (with symptoms such as fatigue, shortness of breath, and orthopnea), and valve replacement surgery is required in half of these patients.
Although infective endocarditis has been described in several patients with SLE, it is a very uncommon complication of this disease. However, several SLE patients have been reported presenting with the following combination of signs and serology: (1) fever, (2) cardiac murmurs with echocar-diographic demonstration of valve vegetations, (3) splinter hemorrhages (Fig. 7.5), (4) serological evidence of SLE activity (e.g., high titers of antibodies to dsDNA and low serum complement levels), (5) moderate to high elevations of aPL, and (6) repeatedly culture-negative blood samples. All these manifestations are explicable on the basis of SLE activity and complications associated with the APS.
A further problem in patients with aPL and valve lesions is the development of embolic cerebrovascular complications. High levels of IgG aCL has been associated with the development of severe valvular regurgitation and with a high incidence of valvular surgery and thromboembolic events.
With regard to long-term treatment, it is of note that anticoagulant or antiaggregant therapy does not contribute to the disappearance of vegetations or other valve lesions, and data about response to corticosteroids are contradictory. Moreover, surgical excision of uninfected valvular vegetations may not prevent recurrence. Nevertheless, prophylactic antiplatelet therapy may be amend to asymptomatic patients, while anticoagulation may be the best choice for patients with val-vulopathy who have had any evidence of thromboembolic disease. In some cases, valvular damage may result in significant hemodynamic compromise, requiring surgery and further full anticoagulation.
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