Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ten percent of 667 consecutive systemic lupus erythematosus (SLE) patients were considered to have definite antiphospholipid syndrome (aPLS) because they had two or more antiphospholipid (aPL)-related clinical manifestations and aPL titers more than 5 SD above the mean of normal controls. Another 14% had either one aPL-related manifestation but high titers of the antibody or two manifestations and low aPL titers (probable aPLS). One fourth of the patients had no manifestations but high titers, one manifestation and low titers, or two or more manifestations and negative aPL titers ("doubtful" aPLS); the other half were considered negative for aPLS. In patients with high-titer aPL, the number of aPL-related manifestations was influenced by disease duration and number of pregnancies, indicating potential mobility of category with time or with risk of recurrent pregnancy loss. Patients with two or more manifestations but variable aPL levels differed in immunosuppressive treatment and in the number of times they had been tested, indicating potential mobility of category with lower treatment and/or further aPL testing. Patients with definite aPLS had increased risk of cutaneous vasculitis, peripheral neuropathy, seizures, psychosis, transient ischemic attacks, and leukopenia. In 11 of 52 SLE patients with definite aPLS the initial manifestation was related to aPL, and in 16 it concurred with an unrelated one. Only two patients fulfilled criteria for aPLS before having other evidence of SLE. The authors conclude that aPLS occurring within SLE is part of the disease rather than an associated condition and propose the use of definite and probable classification categories. These criteria, with appropriate follow-up and clinical and serological exclusion clauses for potential primary conditions, could also be applied to primary aPLS.
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PMID:Preliminary classification criteria for the antiphospholipid syndrome within systemic lupus erythematosus. 160 24

Brain pathology was documented in 10 patients with systemic lupus erythematosus (SLE), 7 of whom had clinical neuropsychiatric SLE prior to death. This was manifested by seizures (5 patients), organic brain syndrome (3 patients) and psychosis (2 patients). Multifocal cerebral cortical microinfarcts, associated with microvascular injury, were documented in 4 patients and in our study constituted the predominant histopathologic abnormality attributable to SLE. Changes of a healed vasculitis in medium sized leptomeningeal vessels were seen in 1 case. Agreement between the clinical and pathologic classification of neuropsychiatric SLE was observed in 7/10 (70%) cases and the strongest association was between the presence of cerebral microinfarcts and seizures (4/5 patients, p less than 0.05). No correlation was observed with anticardiolipin, anti-P, lymphocytotoxic and antineuronal antibodies.
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PMID:Brain pathology in systemic lupus erythematosus. 161 3

The central nervous system (CNS) is clinically involved in approximately 40% of all systemic lupus erythematosis (SLE) patients. Minor psychiatric symptoms and abnormalities on neuropsychological testing are being detected with increasing frequency. This review summarizes current thinking concerning the diagnosis and pathogenesis of CNS lupus. The main symptoms of CNS lupus can be diffuse (generalized seizures, psychosis) or focal (stroke, peripheral neuropathies). Neuropsychiatric symptoms often occur in the first year of SLE, but are rarely the presenting symptoms of the disease. In studies on the pathology of CNS lupus, vasculopathy, infarcts and haemorrhages are often observed, whereas vasculitis is rare. Endocardial lesions and mural thrombi have also been reported in 33-50% of CNS lupus patients. In diagnostic imaging of the CNS, magnetic resonance imaging (MRI) scans often provide evidence for edema or small infarcts, both in focal and diffuse CNS lupus, whereas computerized tomography (CT) scans only show gross abnormalities. The first reports on position emission tomography (PET) scans in CNS lupus patients show decreased glucose uptake in the brain. The cerebral blood flow decreases during active diffuse and focal CNS lupus. The blood-brain barrier is somewhat more frequently impaired in diffuse CNS lupus. Intrathecal IgG and IgM production is observed in 25-66% of all CNS lupus patient. Various specificities of autoantibodies have been observed in CNS lupus. Of these, anticardiolipin (ACA) antibodies show a well-documented association with focal involvement of the CNS in SLE. These antibodies could cause thrombosis by interfering with the protein C pathway of fibrinolysis. In addition, they are associated with endocardial and valvular heart disease, which is often observed in SLE and which could cause embolism. The relation between ACA and diffuse CNS lupus is not yet clear. Low-avidity anti-DNA antibodies are also found in CNS lupus, possibly because of their cross-reaction with cardiolipin. Antineuronal antibodies and lymphocytotoxic antibodies have been associated with diffuse CNS lupus and abnormalities on neuropsychological testing. However, the population of these antibodies is rather heterogeneous and it has not been possible to assess a common target antigen. Therefore, it is still obscure whether there is also a second immune-mediated mechanism responsible for the development of the diffuse form of CNS lupus.
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PMID:Diagnosis and pathogenesis of CNS lupus. 186 69

Phenylpropanolamine (PPA) is the major ingredient in more than 70 over-the-counter preparations including diet pills, nasal decongestants, and the legal "look-alike" stimulants. Structurally and functionally similar to amphetamine and ephedrine, PPA has recently been associated with several neurological manifestations including psychosis, stroke, severe headache, seizures, and intracerebral hematoma. We report a case of intracerebral hematoma and subarachnoid hemorrhage in a young woman with angiographic and biopsy-proven vasculitis of the central nervous system (CNS) induced by PPA in her diet pills. From review of the literature, we distinguish drug-induced vasculitis as a separate entity from primary CNS vasculitis, both clinically and pathologically. This report should alert physicians, in general, to this potentially fatal side effect of PPA, a commonly used over-the-counter drug. Also, neurosurgeons in particular should consider the possibility of drug-induced vasculitis when faced with cases of intracerebral or subarachnoid hemorrhage without apparent cause.
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PMID:Phenylpropanolamine: an over-the-counter drug causing central nervous system vasculitis and intracerebral hemorrhage. Case report and review. 295 31

A rare case of systemic vasculitis with second component of complement (C2) deficiency was documented in a patient who developed colonic ulcerations, jejunal edema and dilatation, cutaneous ulcers, peripheral neuropathy, and psychosis. Colonoscopy revealed typical features of ischemic colitis and radiological examination showed ischemic changes in the jejunum and ileum. Histopathological examination of the cutaneous biopsy revealed typical necrotizing vasculitis. It is very likely that multiorgan involvement, including ischemic changes of the intestine, developed secondary to vasculitis associated with C2 deficiency.
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PMID:C2 deficiency vasculitis: complication of enterocolitis, cutaneous ulcers, and neuropsychiatric disorder. 629 37

A 66-year-old woman presented with a recurrent psychotic disorder with central manifestations that initially suggested a delusional disorder. Peripheral stiffness was at first attributed to a dystonic reaction secondary to neuroleptic treatment. The atypical presentation led to suspicion of collagen vascular disease. Both the psychotic disorder and peripheral involvement responded to cortisone. Findings are discussed in light of recent information on the pathogenesis and neuropsychiatric manifestations of systemic lupus erythematosus, progressive systemic scleroderma and limited scleroderma variants (CREST, unclassifiable connective tissue disease) with positive anticentromere titers, including a discussion of the vasculitis hypothesis of collagen vascular cerebral involvement.
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PMID:Psychotic episodes in an elderly woman with an anticentromere-positive scleroderma variant, IgA deficiency, and hypothyroidism. 791 4

The clinical features and autoantibody profile of 111 black South Africans (103 females and 8 males) with systemic lupus erythematosus was retrospectively analysed. The mean age of the patients was 35.1 years and mean duration of disease 3.5 years. The commonest clinical and laboratory features noted were arthritis (62.2%), hypocomplementaemia (61.2%), haematological abnormalities (60.5%) and malar rash (55%). The serological abnormalities included antinuclear antibodies (98.2%), anti-dsDNA (66.2%), anti-Sm (44.2%), anti-RNP (65.5%), anti-Ro (60.5%), anti-La (28.4%) and rheumatoid factor (10.1%). Positive clinicoserological associations observed included: combination of anti-dsDNA antibodies and low C4 levels with renal disease; anti-dsDNA antibodies with cutaneous vasculitis; anti-Sm antibodies with psychosis; anti-RNP antibodies with Raynaud's phenomenon; anti-Ro antibodies with renal disease, psychosis and malar rash. Anti-La antibodies showed a weak negative association with serositis and Raynaud's phenomenon. Most of these clinical correlates are consistent with past studies. The high frequency of anti-Sm and anti-RNP antibodies is similar to the observations in African-Americans and Afro-Caribbeans.
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PMID:Autoantibodies in black South Africans with systemic lupus erythematosus: spectrum and clinical associations. 877 47

The clinical features and autoantibody profile of 111 black South Africans (103 females and 8 males) with systemic lupus erythematosus were retrospectively analysed. The mean age of the patients was 35.1 years and mean duration of disease 3.5 years. The commonest clinical and laboratory features noted were arthritis (62.2%), hypocomplementaemia (61.2%), haematological abnormalities (60.5%) and malar rash (55%). The serological abnormalities included antinuclear antibodies (98.2%), anti-dsDNA (66.2%), anti-Sm (44.2%), anti-RNP (65.5%), anti-Ro (60.5%), anti-La (28.4%) and rheumatoid factor (10.1%). Positive clinicoserological associations observed included: combination of anti-dsDNA antibodies and low C4 levels with renal disease; anti-dsDNA antibodies with cutaneous vasculitis; anti-Sm antibodies with psychosis; anti-RNP antibodies with Raynaud's phenomenon; anti-Ro antibodies with renal disease, psychosis and malar rash. Anti-La antibodies showed a weak negative association with serositis and Raynaud's phenomenon. Most of these clinical correlates are consistent with past studies. The high frequency of anti-Sm and anti-RNP antibodies is similar to the observations in African-Americans and Afro-Caribbeans.
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PMID:Autoantibodies in black South Africans with systemic lupus erythematosus: spectrum and clinical associations. 879 57

In humans, chronic cocaine abuse is associated with changes in the central nervous system (CNS). Neuropathological changes include cerebrovascular events, EEG abnormalities, vasculitis, seizures, and decrements in neurobehavioral performance. The acute administration of cocaine is associated with acute psychotic episodes and paranoid states while withdrawal from the drug is often associated with depressed mood. The mechanistic basis of these behavioral states is not known. Given the structural and functional changes associated with cocaine use, we propose that the chronic heavy use of cocaine may result in a neuropsychiatric syndrome which might be associated with neuropsychological changes that are not obvious during routine clinical evaluation of drug-using individuals. This disconnection syndrome, because of its sublety, might have deleterious effects on both acute and long-term therapeutic interventions with these subjects. An approach which deals with cocaine abuse as a neuropsychiatric disorder might be more beneficial to the long-term goal of treating these patients. This approach entails a neurobehavioral evaluation which will be comprised of a thorough neurological and psychiatric examination, neuropsychological testing, and imaging studies. The results of this evaluation would provide a more rational basis for cognitive and/or pharmacological therapies.
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PMID:Chronic cocaine use as a neuropsychiatric syndrome: a model for debate. 882 75

We studied the frequency, location, clinical and histopathological features, associated manifestations, and prognosis of vasculitides in a cohort of 667 SLE patients. Exclusion of patients with previous vasculitis or insufficient information left 540 patients, 194 of whom has vasculitis (incidence density: 0.053 new cases/person/year, cumulative incidence of 0.051 at one year, 0.232 at 5 years and 0.411 at 10 years). Vasculitis was confirmed by biopsy in 46 cases, by arteriography in five, and by both in three. A single episode of vasculitis occurred in 119 and two or more in 75 patients. Vasculitis was cutaneous in 160, visceral in 24, both in 10. In the first episode of cutaneous vasculitides, 111 had punctuate lesions, 32 palpable purpura, 6 urticaria, 6 ulcers, 8 papules, 5 erythematous plaques or macules confirmed with biopsy, 2 erythema with necrosis, and 1 panniculitis (plus small vessel vasculitis). Of 29 with visceral vasculitis in the first episode, 19 had mononeuritis multiplex, 5 digital necrosis, 3 large artery vasculitis of limbs, one mesenteric, and one coronary, more than one type could appear simultaneously or in subsequent episodes. Patients with vasculitis had longer disease duration and followup, younger age of onset of SLE, and were more frequently males than those without. Lupus manifestations associated with vasculitis in univariate logistic regression included myocarditis, psychosis, Raynaud's phenomenon, serositis, leukopenia, lymphopenia and pleuritis. Vasculitis also associated with the antiphospholipid syndrome. The strength of this association increased when patients with vasculitis confirmed by biopsy and/or arteriography were considered separately. Visceral vasculitis associated with increased mortality when controlled for age of onset and nephropathy.
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PMID:Vasculitis in systemic lupus erythematosus. 910 29


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