Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
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

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

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

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