1/5. mixed connective tissue disease.Three patients with mixed connective tissue disease (MCTD) had clinical features that included a high incidence of Raynaud phenomenon, arthritis, myositis, and swollen hands. The diagnostic laboratory test result was the presence of high titers of antibody to extractable nuclear antigen. These antibody titers are notably reduced or abolished in patients with MCTD when the tanned red blood cells that are used in the test are pretreated with ribonuclease. Speckled antinuclear antibodies were present in all patients. patients with MCTD have a low incidence of renal disease, are responsive to treatment with prednisone, and have a good prognosis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/5. factor viii related antigen and childhood rheumatic diseases.factor viii related antigen (FVIIIRAg) levels were measured in the plasma of 63 children with rheumatic diseases and 20 controls. High levels were found in patients with systemic juvenile arthritis, systemic lupus erythematosus, dermatomyositis and systemic forms of vasculitis. The amount of circulating FVIIIRAg seemed to be independent of values for erythrocyte sedimentation rate, c-reactive protein and fibrinogen, implying that it was not just another acute phase reactant. Rather, a high level of circulating FVIIIRAg most likely reflects the presence of vascular endothelial injury, and this test may be useful in monitoring disease activity in children with rheumatic diseases in which vasculitis is present.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
3/5. Terminal complement component deficiencies and rheumatic disease: development of a rheumatic syndrome and anticomplementary activity in a patient with complete C6 deficiency.Hereditary deficiencies of early complement components have usually been associated with the development of rheumatic diseases like systemic lupus erythematosus (SLE), while terminal component deficiency is well known to predispose to recurrent neisserial infection. In contrast, only recently have patients been reported with rheumatic disease and hereditary deficiency of a terminal component. The clinical syndrome in these patients has been characterised as 'SLE-like'. We describe here a third patient with complete C6 deficiency and a systemic rheumatic illness characterised by fever, anaemia, lymphadenopathy, hepatosplenomegaly, episcleritis, and asymmetric arthritis. After blood transfusion her serum became anticomplementary; IgG antibody to human C6 was found to be the cause of anticomplement activity. Persistent absence of C6 in this patient and production of anti-C6 antibody after antigenic challenge indicate hereditary C6 deficiency. This case supports an association between hereditary deficiency of a terminal complement protein and the development of systemic rheumatic disease.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/5. Familial palindromic rheumatism: a possible association with HLA.Palindromic rheumatism is very rare in children. The familial occurrence of the disease has been only briefly reported previously. A family is described here in which the mother and her 3 children suffered from typical palindromic rheumatism, which ran a benign course. All 4 members were seronegative and the HLA types of the children were identical. The HLA genotype of the mother was HLA A2, Cw4, Bw41, Bw6, DR5, MT2/Aw32, CX, Bw44, Bw4, DR1, MT1. HLA DR5 has previously been found to be associated with juvenile rheumatoid arthritis, while DR1 in jews is significantly associated with adult-onset rheumatoid arthritis. DR5 was shared by the mother and her 3 children. The presence of the antigen DR5 in this sibship suggests that palindromic rheumatism may be a variant of juvenile rheumatoid arthritis with a relatively late onset and a more benign course. Tissue typing of patients with palindromic rheumatism may give a clue to prognosis. The patients' youth and the familial involvement, with identical HLA characteristics, are the outstanding features of this family.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/5. immunosuppression and the rheumatic diseases.Ignorance of the basic nature of rheumatoid arthritis precludes the introduction of rational schemes for using cytotoxic drugs. It is still plausible that the autoimmune and other immunological abnormalities which accompany this disease are the secondary effects of persistent antigen, for example, related to microbial infections. In this event, cytotoxic drugs may diminish the inflammatory response but their effects on immune responses would be irrelevant or even undesirable. Should rheumatoid arthritis prove to be a primary immunoproliferative disorder, cytotoxic drugs may prove to be of value not because of their conventional immunosuppressive effects but because of their selective action on the proliferating cells. Indeed, current evidence suggests that these drugs enhance rather than depress conventional immune responses, at least in the doses given to patients with rheumatic disorders.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |