1/11. A case of Graves' disease associated with autoimmune hepatitis and mixed connective tissue disease.The patient was a woman of forty-eight. liver dysfunction was pointed out at the age of forty-five. She was admitted to hospital because of her hyperthyroidism. Her palmar skin was wet and her fingers were swollen like sausages. She had a diffuse and elastic hard goiter with a rough surface. The serum levels of free T3 (9.6 pg/mL) and free T4 (3.76 ng/dL) were high and that of TSH (0.11 microU/mL) was low. The activity of TSH-binding inhibitory immunoglobulin (TBII) was 89%. The uptake rate of 123I to the thyroid was 55.1% and the uptake pattern was nearly diffuse. The goiter was proved to contain several nodules by ultrasonography, but aspiration cytology showed no malignant cells. She was diagnosed to have Graves' disease with adenomatous goiter. She also had high ALT (34 IU/L) and gamma-globulin (1.97 g/dL). She had positive antinuclear antibody (speckled type), positive anti-ribosomal nuclear protein antibody, and positive LE cell phenomenon. The liver biopsy revealed mononuclear cell infiltration with fibrosis in the portal area. These data indicated that she also had autoimmune hepatitis (AIH) and mixed connective tissue disease (MCTD). The analysis of human leukocyte antigen (HLA) showed positive A11 which had been reported to relate to Graves' disease, and positive DR4 which had been reported to relate to AIH and MCTD. These results suggested that HLA would determine susceptibility to three distinct autoimmune diseases in this case.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/11. Clinical significance of antibodies to TS1-rna in patients with mixed connective tissue disease.OBJECTIVE: To investigate the clinical significance of anti-TS1-rna antibodies in patients with mixed connective tissue disease (MCTD). methods: Anti-TS1-rna antibodies were detected by immunoprecipitation using 32P-UTP labeled TS1-rna as the antigen source. In total, 104 patients with MCTD, 30 with sjogren's syndrome, 30 with systemic lupus erythematosus (SLE), 25 with systemic sclerosis, 23 with polymyositis or dermatomyositis, and 10 with rheumatoid arthritis were examined. Specificity of anti-TS1-rna antibodies was analyzed by immunoprecipitation using HeLa cell extracts. RESULTS: The frequency of anti-TS1-rna antibodies was 31.7% in patients with MCTD, significantly higher than in SLE (p < 0.05). In anti-TS1-rna positive patients, the incidence of hypertension and proteinuria and the frequency of anti-Sm and anti-dsDNA antibodies associated with SLE were higher than those of anti-TS1-rna negative patients. Clinical features of SS such as sicca complex, the serum level of IgA, and anti-SSA antibodies were also elevated. The frequency of anti-TS1-rna antibodies was significantly higher in SLE patients with anti-U1-RNP antibodies (p < 0.01); however, anti-TS1-rna positive sera did not precipitate the specific rna including U1 rna in immunoprecipitation using HeLa cell extracts. In longitudinal studies, the level of anti-TS1-rna antibodies changed in parallel with disease activity. CONCLUSION: We found that the level of anti-TS1-rna antibodies was possibly correlated with the disease activity of lupus-like clinical features in patients with MCTD.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/11. mixed connective tissue disease associated with protein losing enteropathy: successful treatment with intravenous cyclophosphamide therapy.A patient with mixed connective tissue disease who developed protein losing enteropathy (PLE) is described. The PLE and other symptoms improved dramatically after monthly intravenous administration of 700 mg/day cyclophosphamide three times combined with oral prednisolone, while they were ineffective to the treatment with intravenous methyl-prednisolone 500 mg per day for 3 days. The serum level of CA125 antigen paralleled the severity of symptoms, signs and laboratory data associated with PLE. Thus, pleural effusion, ascites, edema and hypoalbuminemia improved along with the decrease in the level of CA125, suggesting that CA125 might be a marker of the activity of PLE.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/11. Autoimmune disease and HTLV-1 infection.We describe two patients who presented with mixed connective tissue disease. Both had antinuclear antibodies and antibodies to the extractable nuclear antigen U1RNP, and both were found to be seropositive for HTLV-1 infection. We found no evidence of HTLV-1 infection in 20 other patients known to have antibodies to U1RNP or in 36 British and West German patients with idiopathic adult polymyositis. In addition the serum from 20 anti-HTLV-1 positive patients did not contain antinuclear antibodies or antibodies to U1RNP. We conclude that rheumatological disease is associated with HTLV-1 through geographical rather than aetiological means.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/11. Muscle ultrastructure in a case of mixed connective tissue disease and Sjogren's syndrome.A 29-year-old black woman with clinical and serological diagnoses of Sjorgren's Syndrome (SS) and mixed connective tissue disease (MCTD) presented a very important muscular compromise with proximal, bilateral weakness and wasting, an abnormal electromyogram, and elevated muscle enzymes. Ultrastructural study of a muscle biopsy showed different degrees of atrophy and presence of subsarcolemmal loss of sarcomeric arrangement forming regions of contractil material with diverse electron densities. capillaries were proliferative with distortion or occlusion of lumen and thickened basement membranes. An unusual capillary abnormality consisting of thick and convoluted basement membrane intermingled with portions of endothelium and macrophages is described. Mononuclear cell infiltration was formed by lymphocytes, plasma cells, macrophages and scarce mast cells. This work is the first study of the ultrastructural muscle pathology in a patient with diagnoses of MCTD and SS based on clinical findings and the presence of antibodies to RNP, SS-A and SS-B antigens.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/11. The clinical significance of the in vivo antinuclear antibody phenomenon.We describe a patient with systemic lupus erythematosus (SLE) with demonstrated direct immunofluorescent staining of epidermal nuclei in a rim pattern, the second case of in vivo antinuclear antibody (ANA) producing a rim pattern in normal epidermis. A review of the literature showed that 258 cases have been reported of in vivo ANA deposition in epidermal nuclei from nonlesional skin. This phenomenon, which is most common in patients with mixed connective tissue disease, SLE, and scleroderma, correlates frequently with antibodies to extractable nuclear antigens. In vivo ANA have also been described in renal and pulmonary tissue from patients with SLE. In most cases, the pathogenic significance of the phenomenon is unknown.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/11. Membranous glomerulonephritis with extra-renal disorders in children.Thirty of 85 children with membranous glomerulonephritis (MGN) had associated extraglomerular disorders. The relation of these associations to membranous glomerulonephritis (MGN) is discussed. The causal relationship of acute hepatitis (5 cases), persistent hepatitis b antigenemia (6 cases), systemic lupus erythematosus (2 cases) and syphilis (1 case) may be ascertained; in similar conditions a definite antigen (Ag) has been found in MGN deposits. The association with SS or SA hemoglobinopathy (3 cases) ans with a preceding streptococcal infection (4 cases) raises the possible responsibility of renal tubular epithelium (RTE) Ag and of a streptococcal Ag. D-penicillamine therapy (1 case) is a well-known cause of MGN although the acting Ag remains unknown. Four children had serum sickness-like symptoms, two had hematologic disorders and two had proximal tubular dysfunction, one of them with proven anti-tubular and anti-alveolar basement membrane antibodies. A decrease in plasma C4, Clq, and factor B with normal C3 was frequently observed. The multiple Ag previously described as causative of MGN are recalled. The prevalent incidence of HBsAg is stressed, and the necessity for further investigations in patients with MGN in order to find an underlying disease is emphasized.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
8/11. 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) |
9/11. mixed connective tissue disease with fatal pulmonary hypertension.An autopsy case of mixed connective tissue disease (MCTD) with pulmonary hypertension is presented. A 34-year-old woman suffering from arthralgia, Raynaud's phenomenon, and dyspnea of 6-years duration was diagnosed as having MCTD on the basis of a high titer (1:160,000) of serum antibody to the ribonuclease-sensitive component of extractable nuclear antigen. Examination of cardiac function revealed the complication of pulmonary hypertension. autopsy revealed concentric intimal cellular proliferation of the small arteries and arterioles of both lungs. Typical plexiform lesions of these vessels were also observed. These findings coincide with those of plexogenic pulmonary angiopathy of primary pulmonary hypertension (PPH). This is the second autopsy case of MCTD with fatal pulmonary hypertension reported and our observations suggest that some cases with PPH who had immunological abnormalities but could not be classified as cases of classical collagen disease, may have been induced by MCTD.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/11. mixed connective tissue disease complicated by infection with human immunodeficiency virus.A case of HIV-infection presenting as mixed connective tissue disease (MCTD) is reported. A 26-year-old woman from kenya was admitted with the symptoms and laboratory findings of MCTD. Her peripheral CD4 cell count was decreased. She had high titers of antibodies to HIV antigen by a passive agglutination test and an immunofluorescence technique. Moreover, specific IgG and IgM antibodies to hiv antigens were detected by Western blot analysis after complete absorption of the serum in extractable nuclear antigens from calf thymus. While undergoing azidothymidine treatment, she developed AIDS and died of intracranial bleeding. This is the first report of HIV infection associated with MCTD-like symptoms.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
| | Next -> |