Myocardial infarction caused by rheumatoid vasculitis: histological evidence of the involvement of T lymphocytes.

Rheumatoid arthritis (RA) is a chronic joint disease that can be complicated with extra-articular manifestations due to vasculitis. We describe a patient with RA who developed systemic vasculitis and died of myocardial infarction. Autopsy demonstrated vasculitis of the left anterior descendent and circumflex coronary arteries, which were narrowed or occluded with organizing thrombosis. Formation of granuloma with multinucleated giant cells was also observed in media of the circumflex artery. There was no microscopic evidence of atheroma formation in the coronary arteries. Of note, there was a follicle-like infiltration of CD45RO-positive T lymphocytes in interna of the left coronary arteries and the right renal artery. Although not frequently reported, coronary vasculitis as a cause of myocardial infarction should be considered in patients with RA. Moreover, our results suggest that infiltration of T lymphocytes might be involved in the development of rheumatoid vasculitis.

Pleurisy in primary Sjögren's syndrome: T cell receptor beta-chain variable region gene bias and local autoantibody production in the pleural effusion.

Pleurisy with or without effusion has not been considered to be associated with primary Sjögren's syndrome (SS), but rather to represent a manifestation of the underlying disorder, usually rheumatoid arthritis in secondary SS. We describe a patient with primary SS who presented with pleural effusions (PE) as an initial manifestation. Serological studies of paired serum and PE specimens demonstrated the occurrence of local immune reactions in the pleura, including the production of rheumatoid factor and anti-SS-A antibody, the formation of immune complexes, and activation of complement. In addition, the analysis of T cell receptor beta-chain variable (V beta) regions in the PE revealed the overexpression of a number of V beta gene products, including V beta 2 and V beta 13 that have previously been shown to be over-represented in the salivary glands of patients with SS. Thus, our report not only calls for an awareness of pleurisy as an extraglandular manifestation of primary SS, but suggests that a common biased T cell response might play a critical role in the pathogenesis of the glandular as well as extraglandular manifestations.

Cerebrospinal fluid interleukin-6 in progressive Neuro-Behçet's syndrome.

Central nervous system (CNS) involvement in Behçet's disease, usually called neuro-Behçet's syndrome (NB), is one of the most serious complications of the disease. The present study examined cerebrospinal fluid (CSF) interleukin-6 (IL-6) activity in patients with NB to explore its relevance to the progressive CNS disease. Paired CSF and serum specimens were obtained from 11 patients who were diagnosed as having progressive NB based on careful clinical observation and from 11 patients with active Behçet's disease but lacking progressive CNS disease. IL-6 levels in the CSF and sera were determined using IL-6-dependent murine hybridoma MH60.BSF2 cells. All 11 patients with progressive NB showed marked elevation of CSF IL-6 activity [0.18-3.90 U/ml, 1.19 +/- 1.18 (mean +/- SD), normal range, <0.010 U/ml]. In contrast, only 5 of the 11 control patients showed very modest CSF IL-6 activity below 0.10 U/ml, and CSF IL-6 was not detected in the other 6 patients. There was no difference in the serum IL-6 activities of patients with progressive NB and control patients. There was no significant correlation of CSF IL-6 activity with serum IL-6 activity, CSF cell counts, CSF total protein levels, or the CSF/serum albumin quotient. These results indicate that persistent chronic CNS inflammation, as evidenced by the enhanced production of IL-6 within the CNS, plays an important role in the pathogenesis of progressive neuropsychiatric manifestations in Behcet's disease.

[Vasculo-Behçet's disease].

Vasculo-Behçet's disease is a type of Behçet's disease with clinical features based on vasculitis of the arteries or deep veins, and is frequently life threatening. Early diagnosis and treatment are essential for the management of vasculo-Behçet's disease. Recent advances in the diagnosis of vasculo-Behçet's disease have been brought about by the use of radionuclide venography for deep vein thrombosis and computed tomography for arterial lesions. In the treatment of vasculo-Behçet's disease, anticoagulants and fibrinolytic agents are beneficial in controlling deep vein thrombosis. Operative therapy is often recommended for the management of arterial aneurysm because rupture of arterial aneurysm is the leading cause of death in patients with Behçet's disease.

Increased serum alkaline phosphatase activity in Behçet's disease.

Raised serum alkaline phosphatase (Al-P) levels were found in 23 of 213 patients (10.8%) with Behcet's disease (BD). Al-P isoenzyme studies showed an increased liver fraction. Increased Al-P levels were well correlated with the erythrocyte sedimentation rate (ESR) and C reactive protein (CRP). It is concluded that Al-P is a good indicator for the activity of BD.