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)

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

Pseudotumor cerebri is an idiopathic disorder characterized by papilledema and elevated intracranial pressure without a mass lesion. Most patients are female and young and are either overweight or have a history of recent weight gain. Other disease states, such as systemic lupus erythematosus, and drugs, such as tetracycline, have also been associated with the development of pseudotumor cerebri. The mechanism is unclear, but is likely related to decreased cerebrospinal fluid (CSF) resorption. Almost all patients have headache, but the greatest morbidity of the disorder is visual loss related to optic disc swelling. Common radiographic findings in pseudotumor cerebri include an empty sella, dilation of the optic nerve sheaths and elevation of the optic disc. The CSF, aside from elevated opening pressure, is normal without evidence of infection or inflammation. Treatment of patients with no or mild to moderate visual loss is primarily medical, with acetazolamide as the first-line agent. Acetazolamide decreases CSF production. Furosemide and corticosteroids are secondary choices. Optic nerve surgery is reserved for patients with severe visual loss or progression in visual deficits despite medical management.
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PMID:Pseudotumor cerebri and its medical treatment. 1501 Jul 17

Drug induced autoimmune syndromes have been recognized for many years. The classical presentation is that of drug-induced lupus, a generally milder version of the idiopathic disorder that is associated with production of antihistone antibodies. This pattern is now changing, in part due to the many new drugs that have been introduced into clinical practice for treatment of autoimmune diseases, including both conventional pharmaceuticals and biologicals. The number and complexity of drug-induced autoimmune syndromes has increased, and many are now associated with autoantibodies that have been classically defined as limited to idiopathic disease states. Furthermore, some of these drug-induced syndromes have life-threatening complications, so that recognition of drug-induced disease has become more difficult at a time when it is more urgent to establish a correct diagnosis. Many reports are limited to case descriptions, and few controlled investigations have been carried out. Nevertheless, it is possible to derive an approach to considering possible mechanisms by which these processes may take place. This chapter will consider these proposed mechanisms, using some of the implicated drugs to illustrate possible pathogenetic pathways.
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PMID:Drug-induced autoimmunity. 1545 26

Sweet's syndrome is an acute febrile neutrophilic dermatosis which usually presents as an idiopathic disorder but can also be drug induced, associated with hematopoetic malignancies and myelodysplastic disorders, and more, infrequently, observed in autoimmune disorders. Sweet's syndrome has been reported in three cases of neonatal lupus, three cases of hydralazine-induced lupus in adults, and in nine pediatric and adult systemic lupus erythematosus (SLE) patients. We describe three additional adult cases of Sweet's associated with SLE and provide a focused review on nondrug-induced, nonneonatal SLE and Sweet's. In two of three new cases, as in the majority of prior cases, the skin rash of Sweet's paralleled underlying SLE disease activity. The pathogenesis of Sweet's remains elusive, but evidence suggests that cytokine dysregulation may be central to the clinical and pathological changes in this condition, as well as in SLE. Further research is needed to define the exact relationship between the two conditions.
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PMID:Association of Sweet's Syndrome and Systemic Lupus Erythematosus. 2293 42

Dermal mucinosis is characterized by the deposition of glycosaminoglycans (mucin), either focally or diffusely within the dermis. This may occur as a primary idiopathic disorder or secondary to several dermatoses, most notably lupus erythematous, scleroderma, and dermatomyositis. The authors present an unusual finding of dermal mucinosis in association with chronic sclerodermoid graft-versus-host disease.
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PMID:Dermal Mucinosis in Chronic Sclerodermoid Graft-Versus-Host Disease. 2632 57

Kikuchi-Fujimoto disease, or histiocytic necrotizing lymphadenitis, is an infrequent idiopathic disorder. It has been associated with autoimmune disorders, of which systemic lupus erythematosus is the most outstanding. The basis of its diagnosis relies on the histological examination of lymph nodes, which typically reveals necrosis surrounded by histiocytes with crescentic nucleus, immunoblasts and plasma cells, and absence of neutrophils. We report the case of a 27-year-old Argentinian female patient without any relevant past medical history to demonstrate the correlation between Kikuchi-Fujimoto disease and systemic lupus erythematosus.
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PMID:Kikuchi-Fujimoto disease and systemic lupus erythematosus. 2741 58