Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The therapeutic history of sodium diethyldithiocarbamate (dithiocarb) is briefly reviewed. Dithiocarb was discovered serendipitously in our laboratory 35 years ago for the specific treatment of nickel carbonyl poisoning. Since that time, the therapeutic efficacy of dithiocarb has been reported for many disorders, including: nickel, cadmium, thallium, copper, and mercury poisonings, experimental nickel carcinogenesis, protection against radiation damage to bone marrow, treatment of candidiasis in experimental animals, hepatolenticular degeneration (Wilson's disease), systemic lupus erythematosis, and human immunodeficiency virus infection (HIV). It has been used as an antagonist to cisplatin and cyclophosphamide toxicities, and as an antidote to hepatotoxicity induced by chloroform, carbon tetrachloride, and halothane. Most recently, it has been observed that the progression of HIV-1 infection is inhibited by dithiocarb administered intravenously or orally to patients with acquired immunodeficiency syndrome (AIDS). Attention is directed to the interactions of divalent cations to viral infections and to metal chelators (e.g., dithiocarb) as potential antiviral agents.
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PMID:Therapeutic properties of sodium diethyldithiocarbamate: its role as an inhibitor in the progression of AIDS. 184 85

D-Penicillamine is effective in the treatment of Wilson's disease, cystinuria and rheumatoid arthritis. However, it may have adverse side-effects by inducing a spectrum of diseases such as myasthenia gravis, lupus-like disease, IgA deficiency and pemphigus vulgaris. A case of D-penicillamine-induced pemphigus is presented. The clinical aspects, pathogenesis, immunology and therapy of D-penicillamine-induced diseases are discussed.
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PMID:[Penicillamine-induced pemphigus]. 331 68

Although D-penicillamine has been used effectively in the management of a variety of diseases such as cystinuria, Wilson's disease, rheumatoid arthritis, and progressive systemic sclerosis, several toxic drug reactions have been observed with prolonged administration of this agent. One of the most serious side effects is the renal changes that occur following several months of use. We now report two patients with scleroderma who developed serologic evidence of lupus and crescentic glomerulonephritis during treatment with D-penicillamine. Both patients responded to pulse methylprednisolone and subsequent daily steroids. We also review the current information available on the variety of penicillamine nephropathies.
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PMID:Penicillamine-induced rapidly progressive glomerulonephritis in patients with progressive systemic sclerosis: successful treatment of two patients and a review of the literature. 375 71

The clinical features and investigations of 17 patients were analysed. Thirteen of them were Chinese and the rest Indians. Their ages at presentation ranged from 8 to 63 years (mean 18.35 years). Thirteen patients (76%) were symptomatic; 8 with predominantly hepatic manifestations and 5 with neurological features. Four were asymptomatic siblings. At diagnosis, however, 10(59%) had features of liver involvement singly, 3 (18%) had neurological involvement alone and 4 (27%) had mixed presentations. Family histories were available in 15 patients; 26.9% of siblings had Wilson's Disease. Serum ceruloplasmin was low in 82% of the patients. 24-hour urinary copper was measured in 16 patients and was raised in all of them. About half the patients (41%) had evidence of concomittant renal tubular dysfunction with hypouricaemia and aminoaciduria. Three patients (18%) had joint involvement at presentation. All 17 patients were treated with Penicillamine. Complications due to therapy included pemphigus in one and toxic epidermal necrolysis and later a lupus like syndrome in another. The features of clinical improvement included fading of K-F rings, improvement of neurological signs and the normalisation of serum transaminases. One patient developed primary hepatocellular carcinoma 5 years after presentation. Delay in diagnosis was encountered in half of the patients reviewed. Being a treatable condition, Wilson's Disease, although rare, should always be thought of in patients with haemolysis, liver diseases or extrapyramidal disorders.
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PMID:Wilson's disease revisited in the tropics. 375 94

D-Penicillamine is a chelating agent which is effective in the treatment of Wilson's disease, cystinuria, and lead poisoning. In recent years, it has also been used to treat patients with rheumatoid arthritis with good results. The adverse effects of D-penicillamine are many. These include loss of taste, nephrotic syndrome, lupus erythematosus--like syndrome, polymyositis, dermatomyositis, myasthenia gravis, and agranulocytosis. Beginning in 1969, D-penicillamine was reported to induce a pemphigus eruption. We present a patient with D-penicillamine--induced pemphigus erythematosus and review previously reported cases.
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PMID:D-Penicillamine--induced pemphigus syndrome. 706 61

Eight normal volunteers and 32 patients with a variety of neurological disease were studied with a nuclear magnetic resonance (NMR) scanner using repeated free induction decay (RFID), inversion-recovery (IR) and spin-echo (SE) sequences. The results were compared with X-ray computed tomography (CT). RFID sequences which produce images that reflect changes in proton density displayed very little grey-white matter contrast and relatively small changes in disease. IR sequences which produce images that are dependent on T1 showed a high level of grey-white matter contrast and demonstrated changes in a variety of pathological processes. Although SE scans, which have a strong T2 dependence, had shown no abnormality in previous studies of patients with neurological disease, sequences of this type with longer values of tau displayed abnormalities in cerebral infarction, haemorrhage, herpes encephalitis, multiple sclerosis, cerebral oedema, hydrocephalus, tumours and Wilson's disease. All of these conditions were associated with an increase in T2. Abnormalities were demonstrated in cases of multiple sclerosis and brainstem infarction with NMR scans where no abnormality was seen with CT. More extensive changes were seen with NMR in cases of hemisphere infarction, systemic lupus erythematosis, herpes encephalitis, hydrocephalus (periventricular oedema) and Sturge-Weber disease. The margin between malignant tumour and surrounding oedema was better defined with contrast enhanced CT in four of eight malignant tumours, equally well defined in one, and better defined with NMR in three cases. NMR spin-echo sequences provide a sensitive technique for detecting abnormalities in a variety of neurological disease.
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PMID:NMR imaging of the brain using spin-echo sequences. 708 39

Several drugs have been suggested to cause lupus-like syndrome. However, penicillamine-induced lupus-like syndrome has only rarely been reported in patients with Wilson's disease. We describe a 6- year-old Taiwanese girl, with a diagnosis of Wilson's disease in November, 1997, who developed lupus-like syndrome 17 months after penicillamine treatment. After treatment with prednisolone and decrease in the dose of penicillamine, her symptoms subsided gradually. This is the first such case reported in a Taiwanese patient. Because the symptoms of drug-induced lupus (DIL) are nonspecific, subjective and variable, the diagnosis of DIL requires awareness of DIL-inducing potential of chronic medication.
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PMID:Penicillamine induced lupus-like syndrome: a case report. 1104 86

Catatonia is an infrequent but severe condition in young people. Organic diseases may be associated and need to be investigated though no specific recommendations and guidelines are available. We extensively reviewed the literature of all the cases of organic catatonia in children and adolescents from January 1969 to June 2007. We screened socio-demographic characteristics, organic diagnosis, clinical characteristics and treatment. We found 38 cases of children and adolescents with catatonia due to an organic condition. The catatonic syndrome occurred in 21 (57%) females and 16 (43%) males. The mean age of patients was 14.5 years (+/-3.39) [range=7-18 years], and three died from their condition. The organic conditions included infectious diseases (N=10), neurological conditions (N=10), toxic induced states (N=12) and genetic conditions including inborn errors of metabolism (N=6). The onset was dominantly acute, and the clinical presentation most frequently stuporous. Although benzodiazepines were recommended as primary symptomatic treatment, they were rarely prescribed. In several cases, therapeutic approach was related to organic cause (e.g., plasma exchange in lupus erythematosus; copper chelators in Wilson's disease). Based on this review and on our own experience of catatonia in youth, we proposed a consensual and multidisciplinary diagnostic strategy to help practitioners to identify underlying organic diseases.
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PMID:Multidisciplinary approach of organic catatonia in children and adolescents may improve treatment decision making. 1841 62

Wilson's disease (WD) is a rare disease, defined as an autosomal recessive disorder characterised by release of free copper and dramatic accumulation of intracellular hepatic copper with subsequent hepatic and central nervous system abnormalities. Mutations of the ATP7B gene are responsible for the metabolic dysfunction. Small open studies have reported spinal radiological abnormalities including scoliosis, diffuse bone demineralisation, osteochondritis and occasionally fracture. Prevalence of osteoporosis in young adult patients is debated, ranging from 10%, with normal mean Z-score values, to 43% in adults. Past history of spinal or peripheral fractures might be present in 50% of patients. Articular disorders include arthralgias of large joints, such as knee pain, rare effusions, early onset of radiological features of osteoarthritis and associated osteochondritis of the knee joint. Radiological chondrocalcinosis, an unusual feature in young adults, has to be confirmed. Few patients may develop drug-induced lupus with arthralgias, positive anti-nuclear and anti-histone antibodies, secondary to D-penicillamine, the major copper chelator used in WD. In this orphan disease, small retrospective studies cannot allow ascertaining definite WD-related articular and bone manifestations. However, such clinical and radiological abnormalities are occasionally the first symptoms leading to diagnosis. Physicians should be aware that unexplained joint pain and effusion, or even radiological features of osteoarthritis, of the large joints in adolescents could suggest WD and lead to copper survey.
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PMID:Miscellaneous non-inflammatory musculoskeletal conditions. Musculoskeletal conditions associated with Wilson's disease. 2214 43

Systemic lupus erythematosus (SLE) has been reported to be associated to Wilson's disease, as a complication of treatment with penicillamine. Even though drug-induced lupus erythematosus (DILE) has some features in common with SLE, they are distinct entities. We report the case of a young girl who at the age of five had a diagnosis of Wilson's disease and she started therapy with penicillamine. Eight years after the beginning of therapy, she developed proteinuria, which was considered to be related to penicillamine. Two years later, she developed arthritis, malar rash and laboratory findings suggestive for lupus erythematosus. At the beginning her symptoms, due to the known association between penicillamine and DILE, were thought to be related to this treatment. In this hypothesis, she was referred to the Rheumatology Centre; zinc acetate was substituted for penicillamine and she started naproxen for the treatment of arthritis. Anyway, the subsequent clinical course and laboratory findings led us to a diagnosis of idiopathic SLE. A renal biopsy detected massive mesangiocapillary proliferation with subendothelial deposits (wire loops) and duplication of glomerular basement membrane (active diffuse global proliferative lupus nephritis, class IV G A). To our knowledge, this is the first report of an association between Wilson's disease and SLE.
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PMID:Wilson's disease treated with penicillamine and lupus erythematosus: related or distinct entities? 2235 45


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