Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We sought to explore immunological factors in patients who died with rapidly fatal fibrosing lung diseases (Hamman-Rich syndrome). A retrospective review of cases of interstitial lung disease showed 12 recent deaths from Hamman-Rich syndrome. The mean age was 62, men outnumbering women 3 : 1. Five patients had proved collagen vascular disease (rheumatoid arthritis three, lupus two). Four others had a history of allergic disorders, syphilis, chronic eosinophilia, or hypersensitivity reactions. One patient showed disappearance of immunofluorescence as fibrosis advanced, which has not previously been reported. The study suggests a possible aetiological link between disorders of immunity and Hamman-Rich syndrome. The evidence also supports the notion that Hamman-Rich syndrome is an accelerated variant of the more indolent interstitial pneumonias.
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PMID:Rapidly fatal pulmonary fibrosis: the accelerated variant of interstitial pneumonitis. 16 92

The authors sought serum antinuclear antibodies by the indirect immunofluorescent method in 3,260 patients, and found these antibodies in 293 of them. 90 patients had a level equal or greater than 1/50. Among the latter, 54 had some form of collagen disease. The following facts are emphasized: -- antinuclear antibodies are always present in lupus erythematosus. Their levels fall under the influence of treatment when nephritis occurs; -- their frequency is greater (13 cases out of 16) during scleroderma, with often a hazy appearance; -- they are lower during rheumatoid arthritis (23% of cases had a significant level), and their presence is not a sign of worse prognosis; -- they were absent in other forms of collagen diseases.
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PMID:[Anti-nuclear antibodies in collagen diseases]. 16 88

Of 22 children with congenital complete heart block (CCHB) available for study, 14 (63.6%) were born to 11 mothers with clinical or laboratory evidence of connective tissue disease, primarily lupus erythematosus (LE). Seven mothers had both clinical and laboratory evidence of disease while four had only positive laboratory studies including fluorescent antinuclear antibody, rheumatoid factor, and depressed complement levels. In adults with systemic LE, pathologic changes in the collagen surrounding the conduction system have led to the fibrosis and death from heart block. Antinuclear antibodies of the IgG class cross the placental barrier and newborn infants have been reported with transient skin lesions of lupus. Placental transmission of such antibodies may affect the fetal cardiac conduction system, surrounding collagen, and myocardium, leading in some cases to CCHB. This is probably one important etiologic factor in CCHB even though the mother is asymptomatic during her pregnancy.
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PMID:Congenital heart block in newborns of mothers with connective tissue disease. 30 Oct 70

The mixed connective tissue disease syndrome has been described in the medical literature. The clinical and serological characteristics of the syndrome are defined in this paper. The hands of these patients differ from the hands of patients with systemic lupus, rheumatoid arthritis, or systemic sclerosis. In 10 patients there were no erosive changes on radiological examination and all 10 patients had Raynaud's phenomenon. The most striking finding was tightness in the flexors. Mild cases of flexor tightness improved with systemic steroids. One patient with severe flexor tightness required surgical release of adhesions from a chronic inflammatory process of fascia, muscle, and tenosynovium. Biochemical studies showed an abnormal collagen pattern that may be distinct for mixed connective tissue disease.
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PMID:The hand in mixed connective tissue disease. 30 13

Electron microscopic examinations of the skin from foci of lesions in scleroderma, psoriasis, parapsoriasis, and lupus erythematosus revealed two types of unusual collagen structures consisting of loosely packed fibrils 5--8 nm in diameter with a cross-striation period of 150--170 and 80--90 nm. The former were localized in the epi- or perineurium of pulpless nervous fibers of the derma, the latter among collagen fibers. These structures appear to occur rather widely in collagen metabolism disorders (tumours of neurogenic origin, some skin tumours, collagen diseases).
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PMID:[Unusual forms of collagen fibrils in dermatoses]. 44 89

The platelet function was studied in 23 patients with systemic lupus erythematosus, all of whom met the diagnostic creteria established by the American Rheumatism Association. They were not under any treatment, especially with any drug that might interfere with platelet function. The same study was performed on a control group composed of volunteers donors at a blood bank. The platelet count was definitely lower in the patients with lupus than in the control subjects (p less than 0.0005), although a clear thrombopenia was observed in only two indivduals (8.7 percent). Anti-platelet antibodies were found in only six cases (26 percent). There was a linear correlation between thrombopenia and the presence of hemorrhagic diathesis and low levels of C4 and CH50 components. Plateler adhesiveness was clearly lower in the lupus group than in the control group (p less than 0.0005). The presence of kidney disease determined a greater impairment of the platelet adhesiveness (p less than 0.0025). A notable defect on platelet aggregation was induced by ADP, adrenaline and collagen. This was more apparent in the group of patients exhibiting a higher degree of clinical activity and in those who showed a serum complement decrease. The mechanism responsible for this thrombopathy appears to be an interference in the platelet function due to the presence of circulating immunocomplexes. They adhere to the platelet membrane blocking its function and inhibiting the release of the necessary thrombocytic components for the second phase of the aggregation. This platelet alteration is not usually manifested clinically; for this reason no relationship was found between this platelet defect and the presence of hemorrhagic symptoms in our patients. The condition is reversible and may disappear after therapy with steroids and/or immunosuppresive agents.
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PMID:[Platelet function in systemic lupus erythematosus (author's transl)]. 45 1

Lupus erythematodes is related to the vegetative nervous system in Raynaud's disease, the butterfly distribution in the face, the involvement of the lateral part of the eyebrows (Hertoghe) and in cerebral attacks. Involvement of peripheral and central animal nervous systems is common and produces primary and secondary syndromes. Prominent are polymyositis or lupus-myopathy, in the brain mainly functional psychoses, epileptiform attacks, and a variety of focal, often very massive, signs. There are very clear differences from the neurologic signs of dermatomyositis, less so from arteritis nodosa and even less from progressive sclerodermia. Peripheral neurologic syndromes in lupus erythematosus are less common and more subtle than in arteritis nodosa. Bio-electric parameters of the petitmal-trias have been found. The relative absence of collagenoses from the spinal cord is also noticed in lupus erythematosus. Signs of involvement here appear to be limited and often subacute. Differential diagnosis has to consider many neurologic diseases, a special problem when MS is simulated just as in sclerodermia. Since cerbral attacks appear early in about 5%, the use of anticonvulsive drugs, particularly of the hydantoin group, provides special problems. Signs of myasthenia demand further attention. The neuropathologic changes are known in essential points and can obviously hardly be mistaken for inflammatory processes due to other causes. Certain basic facts appear to apply to other collagenoses. All 4 "grand" or "classic" collagen diseases are very similar and have much in common, but also show more or less definite differences. This is true for their clinical and anatomical appearance and last but not least to their neurologic aspects. The "roof-concept" of Collagenosis" is once more proved to be justified. As far as is known today the autonomic system plays a decisive and obligatory role only is sclerodermia. This syndrome is also the only one which contains the odd phenomenon of atrophy of which the best-known form is facial hemia-atrophy.
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PMID:[Erythematodes and nervous system (author's transl)]. 58 95

TEM, SEM and X-ray diffraction analysis demonstrate the heterogeneity of the dentinal tissue on Anarhichas lupus, a vascular osteodentine. The disordered aspect of collagen fibres, incompletely mineralized (the periodical striation being still visible), explains the scattered distribution of crystallites since they are responsible for their arrangement. The low degree of mineralization revealed by the visible collagen striation is confirmed by X-ray diffraction analysis (the crystallinity of vascular osteodentine being much lower than that of the peripheral dental tissue) as well as by high resolution TEM, since no lattice planes could be observed. Osteodentine, supporting bone and proper bone have in common a mineral phase, more or less organized, different from the apatite system.
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PMID:Osteodentine and vascular osteodentine of Anarhichas lupus (L.). 63 May 86

An increase of soluble fractions of collagen and a decrease of insoluble ones was found in the skin of guinea pigs with collagen disease-like syndrome, produced by prolonged treatment with hydralazine. Chromatography studies showed that the collagen alpha chains content in the skin increased and beta-chains decreased under conditions of the development of lupus-like syndrome. The obtained results suggested the inhibition of collagen maturation as one of biochemical defects of connective tissue in drug-induced collagen disease-like syndrome.
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PMID:Increased solubility of collagen. A biochemical defect in the experimental hydralizine-induced collagen disease-like syndrome. 63 26

The influence of tuberculosis and chemotherapy of tuberculosis on the immunologic investigations was proved on 136 patients with tuberculosis and some cases with sarcoidosis, over a period of several months. The immunologic investigations should contribute towards the differential diagnosis of tuberculosis. A general control group was established and also followed up for several months. The diagnostic value of the immunologic investigations seems of little importance for the tuberculosis, the chemo-therapy of tuberculosis does not influence the immunologic situation. The relative frequent appearance of antibodies against cell nuclei and the plain muscles shows that in clinically similar cases we must be careful with the diagnosis of collagen diseases or lupus erythematodes.
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PMID:[Immunologic investigations in tuberculosis and chemotherapy (author's transl)]. 79 25


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