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)

Immunopathologic studies were performed on ocular tissue obtained at autopsy in five patients with systemic lupus erythematosus (SLE). The immunopathologic findings were correlated with histopathologic and clinical features of SLE. Immune reactants were demonstrated by direct immunofluorescence in all patients in a granular pattern suggesting immune complex aggregates; control ocular tissues from four patients without SLE were negative. Ultrastructural studies demonstrated corresponding electron-dense aggregates in a majority of samples. Histologic and gross anatomic evidence of inflammation were generally more focal and less frequent than the distribution of immune reactants. Most of the immune deposits were localized in the walls of blood vessels of the conjunctiva, ciliary body, retina, choroid, and sclera. Diffuse deposits were also found in association with basement membranes in ciliary body (two of five patients) and along the epithelial basement membrane in the cornea (two of five). Immune deposits in peripheral nerves of the ciliary body and conjunctiva in one patient with visual symptoms contained immunoglobulins A and E. The most intense and widespread ocular immune deposits were observed in patients with persistently increased serologic and clinical activity of their systemic disease. These results suggest a role for immune complex localization in the pathogenesis of the ocular lesions of SLE.
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PMID:Ocular immune reactants in patients dying with systemic lupus erythematosus. 388 22

Noninfectious ulceration of the peripheral cornea remains a major diagnostic and therapeutic challenge. The pathogenesis in most of these disorders is unclear, however, on the basis of systemic connective tissue diseases, autoimmune mechanisms are most likely involved. The peripheral cornea has distinct morphological and immunological characteristics that predispose for inflammatory reactions. Major differences exist regarding humoral and cellular components of the immune system. In the peripheral cornea there is more high-molecular IgM and initial complement component C1 than in the central cornea and may predispose for immune complex formation. The close contact to the conjunctival vasculature provides the basis necessary to generate an immune response. Langerhans cells and macrophages as important antigen presenting and processing cells are present in higher number in the peripheral cornea. Autoimmune diseases that affect the peripheral cornea include collagen vascular diseases and Mooren's ulcer. Although this association is obvious in advanced rheumatoid arthritis more subtle forms of polyarteritis nodosa or systemic lupus erythematosus require careful medical evaluation and workup. Ocular manifestations may present as the initial clinical signs and require careful workup in these potentially lethal disorders.
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PMID:[Autoimmune diseases of the peripheral cornea. Immunopathology, clinical aspects and therapy]. 864 90

Systemic lupus erythematosus (SLE) is an autoimmune disease occasionally involving the conjunctiva, sclera or cornea. The immunopathology of the active epibulbar lesions has not been studied in detail. Conjunctival biopsies from 11 SLE patients with active epibulbar lesions and from 12 age-matched individuals undergoing cataract surgery were analysed by light microscopy, immunofluorescence and immunoperoxidase techniques. SLE patients presented with scleritis (3 cases), peripheral ulcerative keratitis (5 cases) or progressive cicatrising conjunctivitis (5 cases). Histologically, SLE specimens showed moderate subepithelial and perivascular mononuclear cell infiltration or granuloma formation in the substantia propria, and squamous metaplasia; thrombosis was not seen. Immunoreactant deposition was present at the epithelial basement membrane in 4 of 5 cases with cicatrising conjunctivitis. Vascular immunodeposits wer detected in 4 cases. The epithelium showed increased T helper cells (CD4+), granulocytes and natural killer cells (CD67+), dendritic cells (CD1+), and an increase in HLA-DR expression compared with normal tissue. In the substantia propria, B cells (CD22+), macrophages (CD14+), dendritic cells, activated T cells (CD25+, CD3+), the T helper (CD4+)/T suppressor (CD8+) ratio and HLA-DR expression were all increased. These observations suggest that the rare epibulbar manifestations in SLE result from immune-complex-mediated reactions.
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PMID:Histology and immunopathology of systemic lupus erythematosus affecting the conjunctiva. 894 91

Wilson's disease is an autosomal recessive disorder related to the copper metabolism. The clinical symptoms are due to copper deposition in various tissues, including liver, brain, kidney, cornea and others. The key strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering both copper-chelating agents and a low copper diet. D-Penicillamine is considered to be the first choice as a copper-chelating agent. Patients require 15-25 mg/kg daily in the early stages of treatment and this drug should also be given more than 2 h before meals. Some undesirable or serious side-effects, such as systemic lupus erythematosus (SLE) and nephrotic syndrome, do occur in 20-25% of all patients. In such cases, trienthylene tetramine (trientine) appears to be as effective as penicillamine. This drug is usually used when D-penicillamine has to be withdrawn. It is also sometimes administered to patients with neurological symptoms as a first-choice drug. It is given in doses of 40-50 mg/kg daily, in the same manner as for D-penicillamine. Zinc salt administration has also emerged as an interesting supportive therapy for both treatments. A dose of 5-7.5 mg/kg daily is given before meals. The copper content of the diet should be less than 1 mg/day in the early stages of treatment. Thereafter, it can be increased to 1.0-1.5 mg/day during well-controlled periods. Liver transplantation is now performed in many countries for patients with either the fulminant or chronic progressive types of Wilson's disease.
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PMID:Treatment and management of Wilson's disease. 1045 98

Sensorineural hearing loss may occur in SLE, but aortic insufficiency has been very rarely reported. We are describing two patients with well-established SLE who developed bilateral hearing loss and aortic insufficiency, associated with serological evidence of active lupus. Neither patient had evidence of keratitis, and thus did not satisfy criteria for Cogan's syndrome. The aortic insufficiency in one patient stabilized after treatment with high doses of steroids while in the second patient, who refused medical treatment, it progressed requiring surgical valve replacement. Our observations suggest that the aortic valve and the inner ear may share some antigenic crossreactivity not shared by the cornea. In SLE patients, with sensorineural hearing loss, echocardiography should be performed looking for evidence of aortic insufficiency, which may be steroid responsive.
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PMID:Sensorineural hearing loss in conjunction with aortic insufficiency in systemic lupus erythematosus. 1125 92

We report the optical and ultrasonic biomicroscopy and confocal microscopy findings in bilateral stromal keratitis (keratoendotheliitis), a rare ocular manifestation of systemic lupus erythematosus (SLE). Examination revealed deposits with polyrefringent crystals. Topical corticosteroid produced regression of the corneal edema, but there was an increase in corneal opacity. Ultrasound biomicroscopy images confirmed the deep location of the corneal opacities, and confocal microscopy showed a disruption of the corneal stroma and crystal-like bodies.
Cornea 2004 Mar
PMID:Bilateral deep keratitis caused by systemic lupus erythematosus. 1507 93

MRL/Mp mice bearing the Fas deletion mutant gene, lpr (MRL/lpr), spontaneously develop polyarthritis, sialoadenitis and dacryoadenitis, resembling rheumatoid arthritis (RA), and also corneal involvement such as keratopathy and scleritis, which is a major complication in RA patients. In this study, we found that the expression levels of IL-1beta and MMP-1 mRNAs in cornea were high in both MRL/lpr and MRL/Mp-+/+ strains of mice at an age younger than when they develop any inflammatory lesions. This was not true of other inbred strains, even those bearing the lpr gene, and also not of (NZB x NZW) F1 lupus mice. There was no significant difference in the expression of IL-1alpha and TGFbeta in cornea in these strains. Using crosses between MRL/lpr and C3H/HeJ-lpr/lpr (C3H/lpr) mice, at least the expression of IL-1beta was found to be under the control of the MRL genetic background, likely with a recessive mode of inheritance. Considering that IL-1beta in cornea was detected particularly in the epithelial layer, the high expression of IL-1beta in cornea is most likely involved in the genetic predisposition for corneal involvement and possibly also for arthritis in an MRL strain of mice.
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PMID:High expression of interleukin-1beta in the corneal epithelium of MRL/lpr mice is under the control of their genetic background. 1508 86

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease, the exact etiology of which is unknown. It is characterized by the production of pathological autoantibodies which adhere to cellular surfaces or form immune complexes which deposit in tissue, leading to end-organ damage via inflammatory mechanisms including complement activation. SLE may manifest itself in any organ system. In the eye, keratoconjunctivitis sicca is the most common finding. Other ophthalmic sites of involvement include the cornea, conjunctiva, episclera, sclera, uveal tract, retina, vasculature, optic nerve, and orbit. Therapy varies based on the disease manifestation and severity.
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PMID:Clinical mini-review: systemic lupus erythematosus and the eye. 1551 79

Four patients (age range 54-82, 1F 3M) diagnosed with non-arteritic ischemic optic neuropathy experienced acute worsening of visual function after instillation of phenylephrine for dilated funduscopic examination. They experienced decreased visual function immediately or shortly after administration of topical mydriatic drops given in preparation for funduscopy. In all cases one drop each of 2.5% phenylephrine and 0.5-1% tropicamide was used. Three patients had classical risk factors such as hypertension, diabetes, and had a contralateral "disc-at-risk". The female and youngest patient had ischemic optic neuropathy presumed secondary to lupus erythematosus. The time from acute visual loss to presentation to neuro-ophthalmic care ranged from 1-6 days. The time of onset of the decline in visual function varied from 45 minutes (patient with lupus) to 12 hours after instillation of mydriatic drops. Visual acuity at diagnosis ranged from 20/40-20/400. Phenylephrine is a mydriatic with vasoconstrictive properties, which may be absorbed through the cornea, thus yielding non-negligible intraocular concentrations. Vasoconstriction of the watershed posterior ciliary capillary beds may result in further precipitating infarction of already compromised circulatory territories in edematous optic nerves. Because phenylephrine is a known vasoconstrictor in vivo and in vitro, it is more likely to cause deleterious vasoconstriction and an acute decline in visual function in patients with acute ischemic optic neuropathy than tropicamide. The routine practice of using phenylephrine to prepare patients for funduscopic assessment should be re-examined, particularly in patients with ischemic optic neuropathy.
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PMID:Topical phenylephrine may result in worsening of visual loss when used to dilate pupils in patients with vaso-occlusive disease of the optic nerve. 1551 9

Toll-like receptor (TLR) 2 and TLR4 are major receptors of Aspergillus fumigatus. Aspergillus fumigatus signaling in cornea induces the production of many pro-inflammatory molecules. In this study, we have shown that exposure of telomerase-immortalized human corneal epithelial cells (HCECs) to A. fumigatus antigens resulted in up-regulation of TLR2 and TLR4, and release of IL-1beta and IL-10 in HCECs, effects that could be inhibited by treatment with TLR2, and TLR4 antibodies. In addition, the A. fumigatus antigens-induced production of IL-1beta and IL-10 in supernatants of corneal epithelial cells was also attenuated by NF-kappaB inhibitor. Aspergillus fumigatus keratitis developed in Wistar rats, as evidenced by high SLE scores, influx of polymorphonuclear leukocytes (PMNs), activation of TLR2 and TLR4, and production of IL-1beta and IL-10 over controls. These findings indicate that the cornea has functional TLR2 and TLR4, and activation of TLR2 and TLR4 through NF-kappaB may contribute to pathogenesis of keratomycosis.
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PMID:Activation of Toll-like receptors 2 and 4 in Aspergillus fumigatus keratitis. 1947 9


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