Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 30 year old man admitted with a brain stem infarct presented with intellectual deterioration. A diagnosis of systemic lupus erythematosus (S.L.E.) was based on the presence of five A.R.A. criteria :photosensitivity, arthralgia, leukopenia and thrombopenia, false positive syphilitic serology, antinuclear factors on immunofluorescence. A lupus type circulating anticoagulant (L.C.A.) was no longer detected after corticotherapy. This appears to be a case of the "hematologic" form of S.L.E. in which the presence of L.C.A. predisposes towards thrombosis, not only of veins--which is typical--but also of arteries. These may be isolated and reveal the underlying disease as in the present case. The L.C.A. is an antibody possessing antiphospholipid specificity which explains the anticoagulant action in vitro and the thrombogenic effect in vivo. Some authors consider this to be sufficient justification for administration of anticoagulants and/or antiplatelet agents, but corticotherapy may be effective.
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PMID:[Brain stem infarction, systemic lupus erythematosus and circulating lupus anticoagulant]. 392 92

The authors reported a case of systemic lupus erythematosus (SLE) associated with bilateral epidural hematomas which had developed without any recent trauma. A 34-year-old male suddenly suffered from a severe headache and vomited several times. He had no neurological deficits on admission, but CT scans of the head revealed abnormal high density areas over the parieto-occipital regions beneath the calvarium bilaterally. At the operation, fresh epidural clots were removed. There were neither evidence of trauma nor abnormal structures which might have led to the development of the clots. He was discharged a month later, being free from any signs and symptoms. One year later, arthralgia progressed and cutaneous ulceration appeared on his feet. On the second admission, butterfly rash on the face, alopecia, polyarthritis and arthralgia, photosensitivity, systemic purpura and proteinuria were noted. With detailed immunological examinations and renal biopsy, he was diagnosed as SLE. SLE is often associated with neurologic and psychic disorders and there are some cases of intracranial hemorrhage among them. However, the association with epidural hematoma has not been reported to date. We think the degeneration of the dural vessels caused by underlying SLE resulted in the development of these epidural hematomas. We also reviewed the literature about spontaneous epidural hematoma and about bilateral epidural hematomas.
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PMID:[A case of systemic lupus erythematosus associated with spontaneous bilateral epidural hematomas]. 401 Aug 85

Seronegative systemic lupus erythematosus is defined as a SLE devoided of antinuclear factors as well as of LE cells when at least 4 out of the 14 ARA criteria are present. We describe herein two case reports and review previous literature concerning this topic. This kind of SLE is characterized by a high incidence of Raynaud's disease, photosensitivity, oral ulcerations, alopecia and perhaps less frequent kidney and central nervous system involvement. The hypotheses about the absence of ANF are discussed. The awareness of such a SLE ("devoided of ANF rather than" seronegative") is to be kept in mind from a chemical, therapeutic and pathophysiologic point of view.
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PMID:[Seronegative systemic lupus erythematosus (author's transl)]. 626 42

Neonatal lupus erythematosus (NLE) is an inflammatory disorder of neonates characterized by transient cutaneous lesions and/or congenital heart block. The cutaneous lesions usually heal with minimal scarring, but healing may be delayed for many months in occasional cases. Photosensitivity is recognized as a component of this syndrome. A large proportion of this maternal population is asymptomatic, although the mothers' potential risk for developing CTD in the future remains to be determined. Moreover, this maternal group may exhibit a tendency to fetal wastage. La(SSB) and/or Ro(SSA) antibody is almost universally present in the sera of the neonatal lupus mothers and their infants. Since these antibodies may have a pathogenetic role in NLE, screening of infants with isolated CHB and/or cutaneous lesions suggestive of LE, and their mothers, for the presence of Ro(SSA) and La(SSB) antibodies is strongly recommended. HLA studies reveal that infants of Ro-positive mothers bearing the HLA, A1, B8, DR3, MB2 and MT2 phenotypes are at increased risk of developing neonatal lupus, in sharp contrast to infants of Ro-positive mothers bearing the DR2 and/or MB1/MT1 phenotypes. Recognition of the protean manifestations of this complex disorder by obstetricians, pediatricians, cardiologists, and dermatologists will undoubtedly lead to increased detection of NLE and afford further opportunity to elucidate more fully the etiology of this syndrome.
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PMID:Neonatal lupus erythematosus. A clinical, serological and immunogenetic study with review of the literature. 639 81

We present four patients with coexistent lupus erythematosus (LE) and psoriasis. This is an unusual combination. All four patients had antibodies to Ro, which were absent in twenty-four control psoriatics. Antibodies to Ro occur in only 25% to 30% of unselected SLE patients, but occur in approximately 60% of "antinuclear antibody (ANA)-negative" LE patients, many of whom are highly photosensitive. The increased frequency of anti-Ro in our patients suggests that this may be a specific serologic marker for the LE/psoriasis overlap. Also, since anti-Ro correlates with photosensitivity, LE/psoriasis overlap patients may be at increased risk for photosensitivity, which occurred in two of our patients, one of whom developed severe systemic disease following ultraviolet (UVB) phototherapy. Screening for anti-Ro antibodies may be appropriate in psoriatics prior to UVB phototherapy.
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PMID:Serologic studies in patients with lupus erythematosus and psoriasis. 660 15

Antibodies to SS-A/Ro have been proposed to be a serologic marker for the neonatal lupus syndrome, which is characterized by congenital heart block or cutaneous lupus or both. The antibodies occur in the mother and are transiently found in the child's serum. We examined an unselected series of 12 children with idiopathic CHB, isolated in 10 children and with cutaneous lupus lesions in two. Six of these children and their mothers were studied during the child's neonatal period, and six were studied retrospectively. All six neonates had SS-A/Ro autoantibodies. Nine of 12 mothers had SS-A/Ro autoantibodies. Of the seropositive mothers, one had systemic lupus erythematosus, two had sicca syndrome, one had photosensitivity, one had arthralgias, and four were asymptomatic. We propose that congenital heart block may be related to transplacental passage of maternal SS-A/Ro antibodies and that neonatal lupus may be the most common cause.
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PMID:Autoantibodies to SS-A/Ro in infants with congenital heart block. 664 24

A case of systemic lupus erythematosus-like disease due to occupational exposure to hydrazine is described. The patient had four of the 1982 revised criteria for SLE (malar rash, photosensitivity, antinuclear antibody, and antibody to nDNA) and genetically is a slow acetylator with the HLA DR2,3 phenotype. Many of her healthy family members had antibodies to nuclear constituents. Lymphocytes from the patient and an identical twin sister, but not from three normal control subjects, showed inhibition of pokeweed mitogen-stimulated IgG synthesis after five daily exposures of each subject to hydrazine. Chemicals such as hydrazine in the environment can induce cases of SLE-like disease in predisposed persons.
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PMID:Lupus erythematosus-like disease due to hydrazine. 668 28

The purpose of this paper is to show the relationship of systemic vascular lesions to the clinical manifestations and prognosis in SLE. Thirty-four autopsied cases of SLE formed the subject material for this study. Histopathological tissue studies were made on vascular lesions from almost all organs. Vascular lesions were separated into three groups according to the size of the involved vessel: 1) medium-sized artery (9 cases), 2) small artery (12 cases) and 3) (13 cases) without systemic vascular lesions. Vascular lesions were also separated into five groups (a) fibrinoid degeneration (10 cases), b) intimal thickening (6 cases), c) thrombosis (6 cases), d) sclerosis (7 cases) and e) (13 cases) without systemic vascular lesions. Patients with involvement of medium-sized arteries had a low female incidence, photosensitivity, and positive LE cell incidence, and the cause of death in these patients was cerebral vascular involvement. The cause of death in patients with vascular fibrinoid degeneration and thrombosis was mainly uremia, whereas patients with sclerosis more often died from infection. Regarding systemic vascular lesions in SLE, the prognosis for the patients with thrombosis and vascular involvement of medium-sized arteries was the most grave.
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PMID:Systemic vascular lesions and prognosis in systemic lupus erythematosus. 671 61

An automated antiglobulin test showed that erythrocytes of a patient with an acute episode of Coombs-negative hemolytic anemia were strongly sensitized with nonagglutinating IgM molecules. The bound antibodies, after elution from red blood cell stroma, were found to be monomeric IgM, since they migrated with IgG molecules on an agarose column, although they were proved to be immunoglobulin M, not G, by a sensitive hemagglutination inhibition assay. The hemolysis subsided with steroid therapy, but ANA increased to a titer greater than 1,024 (with peripheral pattern) without other laboratory evidence of systemic lupus erythematosus. In addition, the patient demonstrated photosensitivity and nonscarring alopecia. We consider that this appearance of erythrocyte autoantibodies consisting of monomeric IgM was a symptom of atypical SLE.
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PMID:Erythrocyte sensitization with monomeric IgM in a patient with hemolytic anemia. 674 33

Out of a total of 370 patients with photosensitivity disorders, the light sensitivity had started at an age of 15 years or earlier in 95 cases (26%). Seventy-eight of these suffered from polymorphous light eruptions (PMLE). In half of the PMLE cases the morphology of the light-induced rash was classified as prurigo-like, and in one-fourth as vesicopapular. Other registered photodermatoses with childhood onset were xeroderma pigmentosum, erythropoietic protoporphyria, systemic lupus erythematosus and pellagra. No childhood cases of photosensitivity from external chemicals or internal medication were encountered.
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PMID:Photosensitivity in childhood. 695 15


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