FIGURE 8 Vulvar ulcerations in Behgets disease Source Courtesy of NZ Derm Net From

fibrosis (16,24). One of the most feared, as well as most common, pulmonary complications of Adamantiades-Behget's is the development of pulmonary artery aneurysms, which are due to vasculitis of the vaso vasorum of the pulmonary vasculature (24,25). Clinically, patients with aneurysms may present with mild hemoptysis, although massive fatal hemoptysis has been reported as a presenting symptom (16). As might be expected, rupture of these aneurysms is associated with significant mortality. In patients with Adamantiades-Behget's who present with hemoptysis, a search for an underlying aneurysm should be performed. Computed tomography (CT), angiography, magnetic resonance imaging, and ventilation-perfusion scanning have been used to identify lesions which, if seen early and treated with immunosuppression, may regress, leading to improved outcomes (26). Pulmonary complications of Adamantiades-Behget's usually occur in the setting of the complete syndrome, but can be seen without obvious systemic involvement, and it is suggested that the Hughes-Stovin syndrome of isolated pulmonary artery aneurysms may be a forme fruste of Adamantiades-Behget's (27).

Other. Renal manifestations of Adamantiades-Behget's are rare and are thought to be due to immune complex deposition (28). Cardiac involvement can include conduction abnormalities, endomyocardial fibrosis, vasculitic infarction or myocarditis, pericarditis, or cardiac or aortic aneurysm formation, with histopathology similar to the lesions seen in pulmonary arterial aneurysms. Epididymitis and orchitis may occur in conjunction with genital ulcerations. Nailfold capillary abnormalities are present in a subset of patients with Adamantiades-Behget's (29). Secondary amyloid deposition can occur with uncontrolled disease.

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