Biomedical subjects
Publications and source records attributed to R J Lionarons.
A 30 year old man presented with symptoms of constrictive pericarditis. Echocardiography and computed tomography showed a mass extending from the pericardium to surround the heart and penetrating the left ventricular apex. An unresectable pleomorphic liposarcoma arising from the pericardium was found at thoracotomy.
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The heterogeneous HLA-B27 antigen is closely associated with post-infectious or reactive arthritis (ReA) and is comprised of two serologically defined variants: B27M1+M2+ and B27M1+M2-. An outbreak of dysentery (n = 120) caused by a Shigella flexneri 2a strain, which possessed cell envelope antigens with epitopes resembling B27M2, resulted in five B27M1+M2+ patients with ReA. The remaining seven B27M1+M2+, one B27M1+M2- and all but three B27-negative patients remained free of joint symptoms; the latter three displayed arthralgia. IgM, IgG and IgA serum titers were statistically raised in all patient groups, but were exceptionally and persistently high in the B27M1+M2+ patients with ReA, especially IgA, as determined in acute-phase sera and sera sampled 1 year after dysentery. B27M1+M2+ thus appears to be a marker for a subset of disease, characterized by a high immune response. It is concluded that the B27M2 epitope is not unequivocally disease-related to Shigella ReA, that B27M1+M2+ is not likely to be the only immune-response-regulating gene involved in this form of ReA and that cross-reactivity between bacterial antigenic epitopes and B27 can only be part of a multifactorial process leading to ReA and in itself not sufficient to produce ReA. The intensity of the immune response appears to be another important factor.
Forty-two male patients with reactive spondyloarthropathies in a Dutch military hospital are described with mean age of onset 21.9 years. Peripheral arthritis or sacroiliitis was present in all, eye symptoms in 21 (50%), and genitourinary disease in 15 (35.7%). Evidence of antecedent sexually acquired or enterocolitic infection was found only in three (7.2%). HLA-B27 antigen was detected in 34 (81%) of 42 patients. Additional data suggest that reactive spondyloarthropathies are the most common form of inflammatory arthritis in young men.
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A recent outbreak of bacillary dysentery in The Netherlands revealed that, despite the close association of HLA-B27 with post-dysenteric or reactive arthritis (ReA), not even in one family did all HLA-B27 positive patients infected by an arthritogenic bacterium, develop ReA. This dissociation shows that additional factors beside B27 may determine susceptibility to ReA.