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 13-year-old girl presented with abdominal pain, fever, dysuria, incontinence and pyuria and was subsequently diagnosed as having systemic lupus erythematosus (SLE) with extensive gastrointestinal involvement and an associated interstitial cystitis. Despite aggressive therapy with high dose prednisone and cyclophosphamide she developed a small bowel perforation and subsequently died. The combination of bowel symptoms and interstitial cystitis seems unique to the population with SLE, while the separate complication of bowel perforation carries an extremely poor prognosis in this group of patients.
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PMID:Bowel perforation and interstitial cystitis in childhood systemic lupus erythematosus. 186 24

Chronic relapsing polyneuropathy (CRPN) is an idiopathic disorder characterized by relapsing and remitting course, elevated CSF protein, slow nerve conduction and absence of systemic disease(s). Systemic lupus erythematosus (SLE), however, has been reported to manifest clinical symptoms and signs mimicking CRPN. A few authors described CRPN as a presenting manifestation or more rarely as the only illness of SLE. In these cases diagnosis of SLE was confirmed by some laboratory tests to detect auto-antibodies which were positive in subclinical SLE. We experienced a 24-year-old female whose illness started as CRPN without any auto-antibodies and recurred with autoimmune abnormalities indicating SLE. She noticed muscle weakness in the lower extremities about ten months previous to the first admission. The weakness progressed gradually and was accompanied by urinary incontinence and sensory deficits in limbs. In another hospital lumbar puncture revealed highly elevated CSF protein and she was referred to us. Neurologic examination showed sensorimotor polyneuropathy with normal blood chemistry and negative auto-antibodies. Prednisolone therapy brought out gradual improvement. She was readmitted 2 years after the first admission because of severe motor dominant polyneuropathy. Serological examination revealed positive auto-antibodies including antinuclear (ANA), anti-DNA, anti-RNP and anti-ENA antibodies. CBC showed decreased number of white blood cells. Nerve conduction velocities were markedly reduced. Again prednisolone was administrated successfully. Thereafter, she experienced several relapsing and remitting cycles. It was characteristic that deterioration of symptomatological findings such as motor weakness was always accompanied by an elevated titer of ANA and increased CSF protein in each of the cycles.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Systemic lupus erythematosus presenting chronic relapsing polyneuropathy as the first manifestation without auto-antibodies]. 226 10

At the age of 29, a woman developed central nervous system manifestations of incontinence, psychosis and a grand mal seizure in February 1982. She was diagnosed as having systemic lupus erythematosus (SLE) based on photosensitivity, oral ulcers and elevated antinuclear and anti-DNA antibodies titers. Three years and one month later the patient had episodes of severe headache and vomiting during the course of maintenance treatment. CT examination of the head revealed blood within subarachnoid cisterns, and a small berry aneurysm was found at the distal portion of the basilar artery by cerebral angiography. The possible role of SLE-associated cerebral vascular changes in the development of this aneurysm is discussed.
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PMID:A case of central nervous system lupus associated with ruptured cerebral berry aneurysm. 250 73

A thirty-year-old housewife, who had been diagnosed as SLE, had been hyperkinetic and mute for 13 months after reiteration of manic-depressive episodes. In this condition she walked around without aim, ate and drank only with nursing help, and could not understand the circumstances around her; she was incontinent of urine and feces. Serological examination did not show any abnormal results during psychic exacerbation. She recovered dramatically by electroconvulsive therapy, and was completely amnestic about the past 15 months. Because her mental state was considered to be limited consciousness, it might be possible that her hyperkinetic and mute state is situated between twilight state and akinetic mutism.
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PMID:[Hyperkinetic mutism within the scope of consciousness disorder in a case of systemic lupus erythematosus]. 273 74

The authors report two cases with both true vitiligo and cutaneous lupus erythematosus (LE). The latter was found only on the vitiliginous areas on both exposed and non-exposed skin. A study of the literature shows that this association is very rare and, like the association between vitiligo and antinuclear antibodies, not statistically significant. It is therefore not possible to explain the pathogenesis of this association by a common autoimmune theory, although vitiligo is well known to coexist with other autoimmune disorders and although antimelanocyte antibodies are exceptionally found in certain cases of vitiligo. The coexistence of lupus erythematosus and vitiligo must be clearly distinguished from post lupus depigmentation which is much more common. In the latter there is clinically an irregular hypomelanosis and an atrophic epidermis and, histologically, there is a pigmentary incontinence with an increased number of melanocytes. Even if it is fortuitous, the appearance of LE lesions on light exposed vitiliginous areas may be explained by the common photosensitivity of these two disorders. However, this superimposition does not explain the common points between these two diseases nor the localization on non-light exposed skin. The localizing role of vitiligo in these exceptional cases could represent a more complex disorder of the dermo-epidermal junction. This disorder could affect more than the melanocytic system and subsequently favour the occurrence of the LE. Nevertheless, the mechanism of this coexistence still remains unknown.
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PMID:[Association of lupus erythematosus and vitiligo (author's transl)]. 723 2

The patient is a 63-year-old Chinese man who presented with tetraparesis and urinary incontinence. The initial diagnosis was cord compression from cervical spondylosis. The patient relapsed 3 months after cervical laminectomy. The transverse myelitis picture, left optic atrophy and suggestive brainstem evoked potentials led to treatment of a presumptive demyelinating process. The presence of vitiligo, however, led to detection of high titers of antinuclear antibodies (ANA) and presence of anti-nonhistone antibodies. The patient was then diagnosed to have a lupus (SLE)-like disease, which has not fully evolved. He was prescribed pulsed cyclophosphamide and prednisolone with significant gains both neurologically and functionally up to 1 year of follow-up. This report highlights the befuddling impact the disease process have on the clinicians in terms of diagnosis, treatment, and prognosis. That it can occur in men in the seventh decade of life heightens the need for awareness in our approach to the myelopathic patient.
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PMID:"Great mimicry" in a patient with tetraparesis: a case report. 771 42

We describe a patient with systemic lupus erythematosus (SLE) with severe organic brain syndrome, who showed diffuse low density areas in the cerebral white matter on computed axial tomography (CAT) scans. A 43-year-old woman with SLE showed disturbances in various intellectual functions, followed by the development of consciousness disturbances and urinary incontinence. CAT scans, single photon emission computed tomography (SPECT) and cerebrospinal fluid (CSF) interleukin 6 (IL-6) were serially examined during the clinical course before and after treatment with high doses of prednisolone. CSF IL-6 activity paralleled the central nervous system (CNS) disease activity most consistently. By contrast, the diffuse low density areas in cerebral white matter on CAT scans as well as the decreased blood flow in the cerebral cortices on SPECT persisted long after the improvement of the CNS manifestations. Magnetic resonance imaging (MRI) scans also revealed the diffuse areas of increased signal intensity in the subcortical white matter on T2 weighted images. The diffuse lesions in the cerebral white matter are considered to reflect the edema as well as other irreversible changes that have resulted from the disturbed cerebral circulation presumably due to the diffuse microangiopathies in the cerebral cortices and not to be necessarily correlated with the CNS disease activities. Moreover, it is suggested that the inflammatory process evidenced by the elevation of CSF cytokines in addition to the disturbed circulation in the cerebral cortices might play an important role in the pathogenesis of CNS lupus in our patient.
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PMID:Systemic lupus erythematosus presenting with diffuse low density lesions in the cerebral white matter on computed axial tomography scans: its implication in the pathogenesis of diffuse central nervous system lupus. 779 63

A 69-year-old woman was admitted to our hospital with a 7-month history of sensory disturbance of the bilateral lower extremities. Since she developed paraplegia of the extremities, urinary incontinence and left hemiplegia several days after admission, neurologic involvement both in the lumbar cord, and in the cervical cord or the brain was suspected. While no abnormalities were noted by computerized tomography of the brain. T2-weighted magnetic resonance imaging (MRI) clearly demonstrated foci in the periventricular and the basal ganglia regions bilaterally. Furthermore, the levels of immunoglobulin G and interleukin 6 were increased in the cerebrospinal fluid (CSF). From physical and other laboratory findings in addition to the MRI and CSF findings, she was diagnosed as having systemic lupus erythematosus with central nervous involvement. The administration of prednisolone resulted in marked improvement in her neurologic symptoms in two months. Thus, it is considered that the MRI and CSF examinations are useful for the diagnosis and treatment of central nervous involvement of systemic lupus erythematosus.
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PMID:[Magnetic resonance imaging and cerebrospinal fluid examinations in a case of central nervous involvement of systemic lupus erythematosus]. 793 61

Involvement of the central nervous system associated with SLE (CNS lupus) is not rare. Two types of CNS lupus are noted clinically; one group manifesting focal neurological symptoms and another group showing mental symptoms. Though it is well known that neurological symptoms are caused by arteritis and thrombus, pathophysiological mechanisms leading to mental symptoms remain obscure and there is no established clinical evidence responsible for these symptoms. A 41-year-old woman was confused and her attention was markedly impaired. Her mental symptoms consisted of disorientation, restlessness, euphoria and emotional incontinence. There were neither focal neurological signs nor meningeal signs. Cerebrospinal fluid (CSF) examination showed increased number of polymorphonuclear cells and permeability of the blood-brain barrier, calculated based on the CSF/plasma protein ratio, was also elevated. Repeated bacteriological examinations revealed to be negative. Gd-DTPA MRI demonstrated diffuse enhancement of the cerebral leptomeninges. Methylprednisolone pulse therapy ameliorated her mental deterioration effectively, and subsequently the leptomeningeal enhancement with Gd-DTPA MRI disappeared in parallel. These radiological and laboratory findings suggest that SLE itself causes inflammation of vessels in the leptomeninges and adjacent cerebral cortex. We consider mental symptoms associated with SLE may be caused, at least in part, by this mechanism. To our knowledge, we could not find similar reports in the literature. Gd-DTPA enhanced MRI seems to be of clinical use for making diagnosis, evaluating clinical activity and understanding of CNS lupus.
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PMID:[Leptomeningeal abnormality on Gd-DTPA enhanced MRI in a case of SLE presenting diffuse organic brain syndrome]. 833 80

The histopathology of the lupus-like skin lesions associated with Bloom syndrome has been sporadically described. Skin biopsies from a 2-year-old boy with the classical features of Bloom syndrome, including lupus-like skin lesions, demonstrated marked interface changes with basal liquefaction degeneration, a moderate superficial mononuclear infiltrate, pigmentary incontinence, and capillary dilation in the papillary dermis. Immunophenotyping of the dermal infiltrate revealed predominance of T-cells. Basement membrane thickening on periodic acid-Schiff examination was not seen. Direct immunofluorescence failed to demonstrate deposits of immunoglobulin other than nonspecific IgM deposition along the basement membrane zone of lesional skin. Ultrastructurally, the most striking findings were disintegration of basal cell cytoplasm and tubuloreticular inclusions in vascular endothelia. Taken together, the histologic and ultrastructural features of lupus-like lesions associated with Bloom syndrome mimic those of cutaneous lupus erythematosus, with the exception of paucity of immune deposits at the dermoepidermal junction.
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PMID:Histopathologic and ultrastructural study of lupus-like skin lesions in a patient with Bloom syndrome. 969 94


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