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)

Treatment of severe hand ischemia a associated to progressive systemic sclerosis and other disorders is controversial. We studied prospectively 45 patients, 41 female and 4 males, over an 11 year period. Age ranged from 16 to 73 years, mean 46. Underlying disease was systemic sclerosis in 30, CREST in 8, overlapping syndrome in 4, systemic lupus in 1 and non rheumatic vasculitis in 2 patients. Treatment consisted of intravenous injection of reserpine, 1 mg, at a superficial arm vein after controlled local circulatory block for 15 min. Regional anesthesia was required in 38 patients. Adequate follow up was obtained in 32 females and 2 males, receiving a mean of 3.1 therapy sessions (range 1 to 13). Morphologic improvement from 3.09 +/- 0.16 to 1.57 +/- 0.13 and functional improvement from 3.6 +/- 0.12 to 1.75 +/- 0.14 (5 grade scoring system), was observed (p < 0.001). These results correlate with adequate rehabilitation confirmed clinically.
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PMID:[Severe ischemia of the hand. Treatment with regional intravenous sympathicolysis with reserpine]. 184 85

Episodic cerebro or retinovascular ischemic events without apparent cause occur in patients with cancer. We report a patient in remission from lymphoma whose multiple episodes of presumed ocular ischemia occurred in the setting of a circulating lupus anticoagulant. Symptoms resolved following therapy with Warfarin.
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PMID:Visual scotomata resulting from lupus anticoagulant in a patient with lymphoma in remission. 191 49

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon pathologic finding associated, in most cases, with rheumatic and hematologic diseases. Although its pathogenesis remains unclear, NRH probably results from liver regeneration to maintain its functional capacity after ischemia-induced injury. An intrahepatic microvascular occlusive mechanism has been considered most likely pathogenetically. NRH may lead to portal hypertension. Thus, the diagnosis of Felty's syndrome must be considered with caution in patients with rheumatoid arthritis (RA) and NRH of the liver. We report seven additional cases of NRH in patients with rheumatic disorders and review the literature to determine the patterns of clinical presentation and natural history of this condition. We also report four patients (three systemic lupus erythematosus [SLE] and one primary antiphospholipid syndrome [PAPS]) in whom antiphospholipid antibodies may have played a role in the genesis of NRH.
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PMID:Nodular regenerative hyperplasia of the liver in rheumatic diseases: report of seven cases and review of the literature. 194 1

We describe an 18-year-old white male who developed lower extremity ischemia requiring amputation. He presented at 14 with pulmonary infiltrates, hepatosplenomegaly, fever, rash, adenopathy, uveitis, and arthralgias; clinical and laboratory findings were consistent with Mycoplasma pneumoniae infection. Despite adequate treatment with antibiotics, he developed chronic arthralgias and fevers, with rash and pericardial effusion. Criteria for the diagnosis of systemic lupus erythematosus were not met; juvenile rheumatoid arthritis was diagnosed presumptively. Over the subsequent 4 years he developed lymphadenopathy with biopsy-proven nonnecrotizing granulomas, chronic leg ulceration with granulomatous histology, and acute-onset impending gangrene of the left foot. A biopsy of the posterior tibial artery demonstrated giant cell arteritis. Although the histologic features were consistent with Takayasu's arteritis, complete aortic arteriography was normal. Examination of the amputated leg showed multifocal segmental giant cell arteritis. Clinicopathologic features suggested, but were not fully consistent with, juvenile systemic granulomatosis. His disease may represent a separate sarcoid-like entity in the broad spectrum of vasculitis.
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PMID:Giant cell vasculitis with extravascular granulomas in an adolescent. 205 10

Bone affectation in systemic lupus erythematosus (SLE) is caused both by the disease itself and by the treatment used. We report the case of a woman diagnosed of SLE, who, in the course of her illness, develops multiple aseptic osteonecrosis (AON) and laminar periostitis, radiologically compatible with the diagnosis of hypertrophic osteoarthropathy (HOA), with no evidence of acropaquia. In this case, the patient shows all the risk factors involved in the pathogenesis of the development of ischemia in bone microcirculation.
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PMID:Laminar periostitis and multiple osteonecrosis in systemic lupus erythematosus. 208 52

The importance of a prothrombotic state as a cause of ischemic stroke in young adults is ill defined. We examined 46 unselected patients under age 50 years with cerebral ischemia for anticardiolipin antibody (aCL) and lupus anticoagulants (LA), over a 3-year-period. Age- and sex-matched patients with other neurologic diseases served as a noncerebral ischemia comparison group to test whether (1) stroke/transient ischemic attacks (TIA) in young people is associated with aCL and/or LA, and (2) their presence is specific to cerebral ischemia. In the stroke/TIA group, 21 patients had aCL or LA and 25 had neither, whereas in the control group, 2 patients had aCL and 24 had neither. Equal numbers of stroke/TIA patients with and without antiphospholipid antibodies (aPL) had other stroke risk factors. Patients with aPL and cerebral ischemia, however, had a more frequent history of multiple events than those without them. These antibodies occur with undue frequency in young patients with stroke/TIA and are not associated with a concurrent diagnosis of systemic lupus in most cases. A coexistent aPL-associated prothrombotic state may be a key determinant of whether patients with atherosclerosis, mitral valve prolapse, or other structural lesions experience recurrent ischemia.
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PMID:Antiphospholipid antibodies and cerebral ischemia in young people. 211 4

New insights about the following aspects of lupus erythematosus are developed: (1) thrombotic and neurological manifestations associated with the presence of antiphospholipid antibodies; (2) cardiac complications, especially coronary ischemia, conduction abnormalities and valvular involvement; (3) pregnancy, with particular attention to fetal and maternal prognoses, Soulier-Boffa syndrome and congenital atrioventricular heart blocks; (4) subacute lupus erythematosus, bullous lupus and cutaneous manifestations associated with the lupus anticoagulant.
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PMID:The multiple clinical aspects of lupus. 212 36

More than a dozen primary hematologic disorders have been associated with ischemic stroke. Inherited deficiencies of antithrombin III, protein C, and protein S have been linked with stroke in case reports; optimal screening requires functional as well as antigenic assays. Antiphospholipid antibodies and lupus anticoagulants are the most frequently identified acquired states associated with ischemic stroke. Polycythemia vera, sickle cell anemia, sickle-C disease, and essential thrombocythemia are the major disorders of formed blood elements causing stroke. Special, step-wise screening for occult prothrombotic entities in stroke patients is recommended for young persons with stroke of uncertain cause, for those with prior venous thrombosis, for those with a family history of unusual thrombosis, and for those with no other explanation for recurrent stroke. Acquired, perhaps transient, abnormalities of platelets, coagulation inhibition, and fibrinolysis may contribute importantly to brain ischemia in synergy with other mechanisms, but at present these remain ill-defined. The contribution of prothrombotic diatheses to stroke is probably underrecognized and warrants further investigation.
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PMID:Hematologic disorders and ischemic stroke. A selective review. 186 63

Magnetic resonance imaging (MRI) was assessed in the management of neuropsychiatric manifestations occurring in 6 SLE patients. The MRI scans were normal in 3 cases and was associated with remission of the symptoms except for a patient who experienced a chorea at the time of the examination. Abnormal MRI scans always revealed more lesions than CT scan. 2 different patterns of abnormalities seem to correspond to 2 specific disorders. In 2 patients with clinical presentation suggesting a cortical ischemia by vascular thrombosis, both MRI scans showed areas of abnormal high signal intensities located in the subcortical white matter. In one last patient, MRI scan revealed multiple focal areas of high signal intensities (on T1 weighter scans) disseminated not only in the deep white matter but also in the gray one. These lesions could be depend upon demyelinisation which may occur by a local vascular process. This serie confirm the interest of MRI in the management of SLE brain involvement as well as it points out some problem of interpretation. This suggest further comparative studies especially at the real onset and during the course of neuro-psychiatric manifestations. At last, the coronal sections may be more informative for the diagnosis and pathophysiology than the horizontal ones.
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PMID:[Magnetic resonance imaging and lupus erythematosus of the nervous system]. 229 Oct 69

Over 2 years, 104 patients underwent clinical evaluation and laboratory screening for the presence of abnormal anticardiolipin antibodies to determine the profile of laboratory and clinical findings in patients with stroke and other neurologic disorders. Seven with incomplete or ambiguous data were excluded; of the remaining 97 patients, 31 were greater than or equal to 65 years old. Nine patients suffered systemic lupus erythematosus, 45 suffered brain ischemia, and 43 suffered other nonischemic neurologic disorders. Cardiac arrhythmia, myocardial infarction, and cardiac valvulopathy were grounds for exclusion. The presence of anticardiolipin antibodies was not influenced by age. In the 88 patients without lupus, anticardiolipin antibodies were significantly more common in the group suffering brain ischemia than in the group with nonischemic neurologic disorders (29% versus 5%, p less than 0.01, chi 2 test). These controlled data demonstrate an association between the presence of circulating anticardiolipin antibodies with stroke, but not with other neurologic conditions.
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PMID:Prospective study of anticardiolipin antibodies in stroke. 230 7


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