Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Epidermal nuclear deposition of immunoglobulins (in vivo ANA) was observed in 45 out of 252 skin biopsies (17.8%). It occurred in 19% of cases with systemic lupus erythematosus, in 32% of the mixed connective tissue disease, in 22% of the scleroderma, in 20% of the cutaneous vasculitis, in 18% of the polymyositis, in 33% of the Sjogren's syndrome, but it was absent in cases with rheumatoid arthritis. The in vivo ANA showed a significant association with serum antibodies to an extractable nuclear antigen (ENA), with speckled pattern of immunofluorescent antinuclear antibody (FANA) and with antibody to a fraction of ENA sensitive to ribonuclease termed ribonucleoprotein (RNP). Indirect evidence was obtained suggesting that the epidermal nuclear deposition of immunoglobulins is a true in vivo phenomenon: some patients with serum antibodies to ENA do not display in vivo ANA and contrariwise, no difference was detected between diseased and normal skin for the occurrence of in vivo ANA and also no association was observed between this phenomenon with immune deposits at dermoepidermal junction or in subepidermal vessels.
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PMID:Epidermal nuclear immunoglobulin deposition in connective tissue diseases. 213 25

Characterization of clinicoimmunological subtypes of systemic lupus erythematosus was based on a detailed serological investigation of 90 SLE patients. Association between excessive formation of circulating immune complexes and the development of some clinical and serological manifestations of systemic lupus erythematosus including active lupus nephritis without the nephrotic syndrome, systemic vasculitis and Sjogren's syndrome was established.
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PMID:[Circulating immune complexes and clinico-immunologic subtypes of systemic lupus erythematosus. II]. 278 88

Sjogren's syndrome (SS) is an autoimmune disorder characterized by lymphocytic infiltration of salivary and lacrimal glands. To determine whether Epstein-Barr virus (EBV) might play a role in the pathogenesis of this disorder, we used monoclonal antibodies and DNA probes to detect evidence of viral gene products and genomes in these patients' tissue biopsies and saliva. Cytoplasmic staining of epithelial cells (i.e., ductal and/or acinar cells) with monoclonal antibody against the EBV-encoded early antigen (EA-D) was noted in 8/14 salivary gland biopsies from SS patients. This antibody did not react with normal salivary glands or salivary gland tumors, nor with other tissues from SS patients. The reactive antigen in SS biopsies had a m.w. of 52,000 on the basis of immunoblotting experiments, similar to the EA-D antigen found in lymphoblastoid cells lytically infected with EBV. EBV DNA was detected in parotid biopsies from two SS patients in amounts ranging from 0.1 to 3 pg per 20 micrograms of cellular DNA. Southern blotting was used to demonstrate the reactivity with Bam V, Eco D, and Bam M probes. Parotid saliva samples from 8/20 SS patients contained EBV DNA detectable by slot blot hybridization. EBV DNA was not detected in saliva of age-matched controls, rheumatoid arthritis patients lacking sicca symptoms, or patients with benign parotid tumors. The presence of EBV in salivary gland and saliva samples was associated with clinically more severe SS, as manifested by extraglandular symptoms such as vasculitis, occurrence of "pseudolymphoma", and marked abnormalities in immunoglobulin levels. These results demonstrate an elevated content of EBV in salivary glands of SS patients, and suggest that this virus may play a role in pathogenesis.
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PMID:Detection of Epstein-Barr virus-associated antigens and DNA in salivary gland biopsies from patients with Sjogren's syndrome. 302 47

A patient who presented with urticarial vasculitis, recurrent angio-oedema and gastro-intestinal symptoms in association with a 'lupus-like' disorder is documented. Other 'auto-immune' phenomena such as Sjogren's-like syndrome and polyarthritis were present in association with antibodies to RNP, low C4 and a null allele was demonstrated at the C4B locus. Response to varied therapies was poor and unsustained.
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PMID:Hypocomplementaemic urticarial vasculitis, angio-oedema and 'lupus-like' disease: association with C4B null allele. 349 4

A 35-yr-old woman with coexistent primary biliary cirrhosis and Sjogren's syndrome developed recurrent transverse myelitis. The histopathologic appearance was that of an angiitis associated with necrotizing myelopathy involving the cervical and thoracic spinal cord.
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PMID:Primary biliary cirrhosis, Sjogren's syndrome, and transverse myelitis. 394 Feb 47

The most typical clinical features of polymyositis (PM), the criteria of diagnosis and principles of treatment are outlined. An inflammatory disease of muscle, PM also frequently affects other organs such as the skin and hence the name dermatomyositis. The principal cardiac symptom is a peculiar disturbance of atrioventricular conduction, correlated with a specific anti-Ro autoantibody, present in 25% of patients. The etiology of PM is as yet unknown, although there is evidence for an autoimmune pathogenesis. It is frequently found in association with other immune-mediated diseases such as myasthenia gravis, pemphigus, immune-complex vasculitis and Sjogren syndrome. Laboratory investigations show hypergammaglobulinemia, a decrease of complement factors C3 and C4 and the presence of circulating immune complexes in 70% of patients. Very frequent, especially in cases of dermatomyositis, is a histologically detectable accumulation of IgG and complement in the walls of the intramuscular venous vessels. Cell-mediated hypersensitivity, emphasised formerly as highly significant in PM, has not been confirmed. The presence of specific antimyoglobin lymphocyto-toxicity, once considered to be the hallmark of muscle degeneration in PM, has been excluded by a number of laboratories. In a personal series of patients with various clinical forms of PM a severe loss of suppressor/cytotoxic lymphocytes was found in the peripheral blood and a relative increase in the first subset. These results support the hypothesis that a serious disturbance of immunoregulation is present in PM and is the cause of a multitude of immunological anomalies, the characterisation of which is under study.
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PMID:Recent advances in polymyositis. 620 72

Upon analysis of 550 cases of different chronic diffuse pulmonary diseases included in a group of interstitial diseases of the lungs (IDL) the authors came to the conclusion that IDL incorporate such variants as alveolitis, pulmonary vasculitis and pulmonary hemorrhages; granulomatosis covers exogenic allergic alveolitis, alveolitis in chronic active hepatitis; vasculitis group includes such rare diseases as necrotizing sarcoid granulomatosis vasculitis and lymphoid granulomatosis; fibrosing alveolitis--secondary alveolitis in sclerodermia systematica, rheumatoid arthritis, Sjogren's disease, chronic active hepatitis. Knowledge of IDL etiology (environmental, occupational, induced by radionuclides, drugs, viruses, fungi) with focus on drug affection of the lungs is thought of value. Biopsy and bronchial lavage findings are compared clinically and morphologically. Mechanisms of pulmonary fibrosis and approaches to inhibition of pulmonary fibrosis progression are outlined.
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PMID:[Interstitial lung diseases--the clinical aspects of the problem]. 763 86

Rheumatoid arthritis affects joints and other systems in the body. Dryness of the eyes and mucous membranes are referred to as Sjogren's Syndrome. The heart is usually spared but the lungs can be affected by pleurisy, scarring, and the formation of nodules in the lungs. The nervous system may be involved by compression neuropathy (e.g. carpal tunnel syndrome), peripheral neuropathy, or occasionally from cervical cord compression. The rare complication of vasculitis (inflammation of blood vessels) may be devastating because of gangrene or severe internal organ damage. Some of the effects of rheumatoid arthritis may be "invisible" such as anemia or abnormalities in the white blood cell count. Osteoporosis is usually asymptomatic until a fracture occurs.
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PMID:Extraarticular manifestations of rheumatoid arthritis. 765 52

The aim of the study was to prove the incidence of antiphospholipid syndrome (APS) in systemic connective tissue disease (SCTD), especially in systemic lupus erythematosus (SLE) and to find out serologic markers of APS. Of 94 patients with SCTD followed up in the last three years 80 had SLE, 7 Sjogren's syndrome, 3 systemic vasculitis, 2 combined systemic connective tissue disease, 1 rheumatoid arthritis, 1 Sjogren's syndrome with vasculitis. Antiphospholipid antibodies (ApSt) were determined by ELISA method. In 31 of 80 patients with SLE (38.07%) antiphospholipid antibodies were proved. In lo of 31 (32.26%) signs of systemic lupus or the presence of APS markers have not been found, while 21 of 31 (67.74%) had some of clinical manifestations of APS (most commonly thrombocytopenia or vascular thrombosis) with evolution of the basic disease. Among our patients with SLE the most common finding was IgG antibody isotype-in 14 or 66.66% of cases. In 5 of 7 patients with Sjogren's syndrome ApAt were proved without clinical evolution of the basic disease and APS. Only 2 of 7 patients of this group showed, together with more increased ApAt, mild evolution of the basic disease and manifestation of APS (livado reticularis and recurrent venous thrombosis).
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PMID:[The antiphospholipid syndrome in systemic connective tissue diseases]. 819 29

In populations such as Northern Europeans in which the HLA-DR4 subtypes DW14 and Dw4 show strong association with rheumatoid arthritis (RA), these alleles and the double allelic dose of the shared epitope are considered severity markers. The clinical expression of RA varies in different populations, which may be determined by variation in the prevalence of these markers. In the present study we analyzed the expression of RA in 112 consecutive Chilean patients and its relation to the prevalence of genetic factors, prompted by our previous observation that DR4 is weakly associated to RA in this population. Mean age was 50 +/- 14 years; 90% were seropositive and 87% were female, with a disease duration of 10 +/- 8 years. Extra-articular manifestations were found in 38% of patients, rheumatoid nodules in 27%, vasculitis in 8%, and Sjogren's syndrome in 29%. Functional capacity (ACR, 1991) I or II: 82%.15% of patients stopped working. Hand radiographs scored according to Steinbrocker in 89 patients: I, 21%; II, 15%; III, 43%; IV, 21%. In this series, patients with less formal education seemed to have more benign arthritis. In 97 controls and in 65 (56%) RA patients the presence of DRB1 alleles corresponding to DR1 and DR4 serotypes, to DR4-Dw subtypes, and homozygocity, were determined by polymerase chain reaction followed by specific oligonucleotide hybridization. The shared epitope was present in 53% of RA patients and in 30% of controls (P = .0048, odds ratio [OR] = 2.64). A double allelic dose of the epitope was present in 15% of RA patients compared with 4% of controls (P = .026, OR = 4.23). In a subgroup of 31 erosive RA patients we did not find a significant association of disease severity with the shared epitope in a single or double allelic dose. None of the DR4 subtypes that associate with RA in other populations was found significantly more prevalent in our patients. The severity of RA in our study compared with published series was intermediate between British patients with severe RA and Greek patients with milder disease. This may be due to the high prevalence of Dwl3*0403 in our population.
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PMID:Clinical expression of rheumatoid arthritis in Chilean patients. 865 May 90


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