1/12. 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) |
2/12. Digital ischemia and gangrene preceding renal neoplasm. An association with sarcomatoid adenocarcinoma of the kidney.A 63-year-old woman had acute onset of rapidly progressive Raynaud phenomenon and digital gangrene. Prior to the detection of a sarcomatoid renal carcinoma, prominemt hypergammaglobulinemia, microhematuria, and weight loss were noted. Following nephrectomy, the patient showed improvement of the Raynaud phenomenon, with complete healing of digital ulcers and decrease of gama-globulin levels. Immunofluroescence studies demonstrated substantial deposits of IgG that lined the tumor cells in a linear and diffuse pattern. Electron-dense deposits were seen in the endothelium of arterioles int the tumor by electron microscopy. These findings suggest that antibodies to tumor antigens may have participated in the induction of digital vasculitis and Raynaud phenomenon.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/12. Antibodies to glycyl-transfer rna synthetase in patients with myositis and interstitial lung disease.OBJECTIVE. We have previously described anti-EJ antibodies, and provided evidence that these antibodies react with glycyl-transfer rna (gly-tRNA) synthetase. The aim of the present study was to identify patients with anti-EJ antibodies and describe the clinical associations of the antibody, in particular, whether it is associated with the syndrome of myositis and interstitial lung disease (ILD) that has been previously associated with autoantibodies to the aminoacyl-tRNA synthetases for histidine, threonine, and alanine. methods. Sera from patients with suspected or proven polymyositis or dermatomyositis (DM), sera with anticytoplasmic patterns, and control sera were tested for anti-EJ antibodies by immunoprecipitation (IPP). Positive sera and controls were tested for the ability to inhibit gly-tRNA synthetase by preincubation of the enzyme source with the serum. RESULTS. Anti-EJ antibodies were demonstrated in the sera of 5 patients, by IPP of characteristic tRNAs and protein. Original serum EJ and each of the new sera significantly inhibited the enzymatic activity of gly-tRNA synthetase but not histidyl-tRNA synthetase. All 5 of the new patients had inflammatory myopathy, a typical DM rash, and ILD. One, who had an overlap syndrome with systemic lupus erythematosus, had anti-EJ at least 4 months before the development of clinical myositis. Arthritis and Raynaud's phenomenon, other features associated with antisynthetases, were also seen. CONCLUSION. Anti-EJ is associated with the syndrome of myositis and lung disease that is seen in association with other antisynthetases. The finding of specific inhibition of gly-tRNA synthetase by all anti-EJ-positive sera strongly supports the identification of EJ antigen as gly-tRNA synthetase.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/12. Raynaud's phenomenon with digital necrosis as the first manifestation of undifferentiated connective tissue syndrome.A patient with severe Raynaud's phenomenon (RP) associated with the presence of antibodies against extractable nuclear antigen (ribonucleoprotein) in the context of undifferentiated connective tissue syndrome is presented. The rapid course of digital necrosis in her case resulted in the surgical amputation of 9 of her fingers. We conclude that massive digital necrosis accompanied by severe RP could constitute the first manifestation of a connective tissue disease.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/12. Coexistence of crest syndrome and primary biliary cirrhosis. Serological studies of two cases.Two patients with characteristic features of crest syndrome and primary biliary cirrhosis are reported. Sera of both patients contained autoantibodies to centromere and mitochondrial antigens. immunodiffusion analysis identified the specificities of precipitating antibodies to mitochondria of the first case as anti-M-A and M-C, and of the second case as anti-M-A and M-B antibodies. Simultaneous occurrence of 2 marker antibodies in an individual patient indicates that the 2 patients reported here have crest syndrome and primary biliary cirrhosis, both of which are considered as a distinct clinical entity.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/12. A novel antinuclear antibody associated with a lupus-like paraneoplastic syndrome.The association of malignancy with lupus-like syndromes is rare, and the relation between these two processes is uncertain. A 71-year-old woman who presented with serositis, Raynaud phenomenon, and positive results on an antinuclear antibody test was initially thought to have systemic lupus erythematosus but was found to have ovarian adenocarcinoma. A unique sparsely speckled antinuclear antibody pattern was seen. The patient's sera reacted to novel antigens with six bands of 100, 80-78, 48, and 17 kD on Western blots not typical of reactivity for sera from patients with systemic lupus erythematosus.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/12. Neurologic complications of primary sjogren's syndrome.Although peripheral nervous system disease has been well documented in Sjogren's syndrome (SS), central nervous system (CNS) involvement is considered distinctly uncommon. Sixteen patients with primary SS and CNS disorders not attributable to other causes were the subjects of this study. Cerebral manifestations, both focal and diffuse, as well as spinal cord disease, were observed. Peripheral vasculitis occurred in 12 patients (75%), 83% of whom had anti-Ro(SSA) antibodies. The high proportion of patients with concomitant peripheral vasculitis, and the observed association with antibodies to the Ro(SSA) antigen system which, in other studies, has been linked to vasculitis in SS, suggest that an immune vasculopathy may play a role in the pathogenesis of the CNS disease of SS.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/12. The crest syndrome: a distinct serologic entity with anticentromere antibodies.The crest syndrome is a variant of systemic sclerosis characterized by the presence of calcinosis. Raynaud's phenomenon, esophageal motility abnormalities, sclerodactyly and telangiectasia. The serums of 27 patients with the crest syndrome have been examined for the presence of antinuclear antibodies. Twenty-six of 27 (98 percent) serums contained high titers (> one:80) of an antibody that produces a discrete speckled pattern of immunofluorescence on a human laryngeal carcinoma cell line (HEp-2). The antibody has been shown to react with the centromeric region of metaphase chromosomes. This antibody was also found in three of 14 patients with Raynaud's disease, in one of 60 patients with systemic lupus erythematosus, in three of 26 patients with systemic sclerosis with diffuse scleroderma and in one of 15 patients with mixed connective tissue disease. The antibody was not detected in the serums of patients with rheumatoid arthritis. Sjogren's sicca complex or linear scleroderma. patients with osteoarthritis who were age- and sex-matched to the group with the crest syndrome did not have anticentromere antibodies. autoantibodies found in other connective tissue diseases (anti-dna, anti-RNP, sjogren's syndrome antigen B (anti-SS-B) were not found in serums from patients with the crest syndrome. A case report illustrating the appearance of the anticentromere antibody at a time when Raynaud's phenomenon antedated the clinical diagnosis of crest syndrome is presented.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/12. Three siblings with scleroderma (systemic sclerosis) and two with Raynaud's phenomenon from a single kindred.A kindred is reported which contains 3 siblings with scleroderma, 2 siblings with Raynaud's phenomenon, and 2 first-degree relatives with histories suggestive of connective tissue syndromes. Studies of microvascular morphology and flow, serum endothelial cytotoxic activity, antinuclear antibodies, and HLA haplotypes in 18 relatives and 6 spouses revealed that 4 of 5 affected siblings expressed the HLA-DRw4 antigen, which was also present in 2 of 3 asymptomatic relatives whose serum contained endothelial cytotoxic activity. The evidence for an inherited susceptibility to scleroderma is reviewed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/12. Raynaud's phenomenon and initially seronegative mixed connective tissue disease.We report 2 young women with Raynaud's phenomenon who later developed the laboratory and clinical features of mixed connective tissue disease (MCTD). Antibodies to extractable nuclear antigens were initially negative and remained so far several years before becoming positive. Raynaud's phenomenon is common in MCTD, but laboratory and clinical evidence of the latter may not be present initially.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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