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)

Intestinal pseudo-obstruction (IPO) is a rare complication of systemic lupus erythematosus (SLE). We present a 32-year old female with SLE for seven years. She was admitted with profound fatigue, frequent vomiting, colicky abdominal pain, diarrhoea and intermittent dysuria for the past 12 months. Imaging studies revealed dilated small and large bowel loops with thickened intestinal wall and multiple fluid levels. Urinary tract involvement was also demonstrated. The patient responded well to immunosuppressive treatment. IPO in the context of SLE has been described only in anecdotal case reports. Half of the cases developed this complication during the course of lupus as in the present case. Concomitant ureterohydronephrosis was present in approximately two-thirds of the cases. Early recognition of the syndrome is necessary for the institution of the appropriate medical treatment and prevention of inappropriate surgical intervention.
Lupus 2004
PMID:Intestinal pseudo-obstruction and ureterohydronephrosis as the presenting manifestations of relapse in a lupus patient. 1564 52

Patients with systemic lupus erythematosus are prone to various infections, often associated with high mortality. Salmonella species are a rare cause of continuous ambulatory peritoneal dialysis (CAPD) peritonitis, but patients with lupus are susceptible to infection caused by Salmonella species. A 47-year-old woman who had systemic lupus erythematosus with end-stage renal disease and was undergoing CAPD presented with fever, watery diarrhea, abdominal pain, and turbid dialysate effluent. The effluent culture yielded Salmonella enteritidis. Although she was administered antibiotics to which the organism was susceptible in vitro, peritonitis was not cured. Her clinical condition stabilized after removal of the Tenckhoff catheter and prolonged antibiotic use. In Salmonella-related CAPD peritonitis, early catheter removal may be necessary to achieve complete treatment.
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PMID:CAPD-related peritonitis due to Salmonella enteritidis in a patient with SLE. 1611 34

The antimalarials, mainly chloroquine and hydroxychloroquine, derive from the quinoleine core of quinine. Their initial therapeutic indication was the treatment of malaria attacks but, because of anti-inflammatory and immuno-modulatory activities, they have been since used to treat many other pathologies, in particular dermatological ones. For some of these pathologies, lupus or porphyria cutanea tarda for example, the use of these molecules is based on obvious scientific evidence. For other pathologies (cutaneous sarcoidosis, polymyositis, polymorphous light eruption...), the data on the medical literature corroborating the daily clinical practice are extremely poor. Their toxicity is limited. Their most common toxic effects are gastrointestinal (mild nausea or diarrhea) or mucocutaneous (reversible skin or mucosal pigmentation). Their most serious and dreaded side effect, retinopathy, can be largely prevented by using amounts of APS adapted to the weight of the patients. The recommended "safe" daily dose for hydroxychloroquine is 6.5 mg per kilogramme of body weight and for chloroquine 4 mg per kilogramme of body weight. However, at 6- to 12 months intervals, follow-up eye examinations should be performed.
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PMID:[Synthetic antimalarials]. 1623 Sep 16

Gastrointestinal manifestations are common in systemic lupus erythematosus (SLE). Eosinophilic enteritis is a rare disorder of uncertain cause that was recently reported for the first time in association with SLE. This report presents a second case of eosinophilic enteritis in a 47-year-old female patient with SLE. The patient presented with recurrent episodes of abdominal pain, nausea, vomiting, and diarrhea. Complete blood counts on occasion showed elevated eosinophil counts. The patient underwent a comprehensive workup over several weeks, culminating in a small bowel biopsy that showed eosinophil infiltration in the muscularis propria, establishing the diagnosis. The patient was treated with a prolonged taper of prednisone with successful resolution of symptoms.
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PMID:Eosinophilic enteritis with systemic lupus erythematosus. 1629 25

A 26-year-old Indian lady was admitted for lower abdominal pain, diarrhoea, vomiting, fever and cough. The initial diagnosis was that of peritonitis secondary to ruptured or perforated viscus with lobar pneumonia. On laparotomy, she was found to have necrotizing or Kikuchi's lymphadenitis of the abdominal lymph nodes. The initial two antinuclear antibody (ANA) results came back negative. She was diagnosed to have systemic lupus erythematosus (SLE) when the third sample for ANA came back positive and the double-stranded DNA (dsDNA) antibody test was homogenously positive. This case illustrates a need to be aware that necrotizing lymphadenitis can precede the onset of systemic lupus erythematosus.
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PMID:Kikuchi's lymphadenitis (necrotizing lymphadenitis) and systemic lupus erythematosus: a case report. 1632 34

Systemic lupus erythematosus (SLE) is frequently associated with ascites, but rarely without proteinuria. We report a 10-year-old girl with distended, non-tender abdomen with shifting dullness and no pitting edema in the lower legs before admission. Facial rash had appeared 1-2 weeks before admission and became more prominent 3 days prior to admission. Hypoalbuminemia with hypertriglycemia (but no proteinuria or diarrhea) was noticed. The antinuclear antibody titer was 1:2560 (speckle type) and the anti-double-stranded DNA was 1:160. Abdominal echo revealed no cirrhosis change or venous obstruction. Chest X-ray and electrocardiogram revealed no cardiomegaly or pericardial effusion. The serum prealbumin was low on admission day 5, but the liver function tests were within normal range. We deduced that the hypoalbuminemia in SLE without nephritis may be secondary to mesenteric vascular leakage. SLE may present with initial manifestation of painless massive ascites. Careful utilization of history taking, chest X-ray, electrocardiogram, cardiac and abdominal echo, urinary analysis and serum prealbumin is helpful in decision-making while assessing such patients.
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PMID:Painless massive ascites and hypoalbuminemia as the major manifestations of systemic lupus erythematosus. 1644 Jan 28

Haemolytic uraemic syndrome (HUS) is the most common cause of acute renal failure in children. The syndrome is defined by triad of microangiopathic haemolytic anaemia, thrombocytopenia and acute renal failure (ARF). Incomplete HUS is ARF with either haemolytic anaemia or thrombocytopenia. HUS is classified into two subgroups. Typical HUS usually occurs after a prodrome of diarrhoea (D+HUS), and atypical (sporadic) HUS (aHUS), which is not associated with diarrhoea (D-HUS). The majority of D+HUS worldwide is caused by Shiga toxin-producing Esherichia coli (STEC), type O157:H7, transmitted to humans via different vehicles. Currently there are no specific therapies preventing or ameliorating the disease course. Although there are new therapeutic modalities in the horizon for D+HUS, present recommended therapy is merely symptomatic. Parenteral volume expansion may counteract the effect of thrombotic process before development of HUS and attenuate renal injury. Use of antibiotics, antimotility agents, narcotics and non-steroidal anti-inflammatory drugs should be avoided during the acute phase. Prevention is best done by preventing primary STEC infection. Underlying aetiology in many cases of aHUS is unknown. A significant number may result from underlying infectious diseases, namely Streptococcus pneumoniae and human immunedeficiency virus. Variety of genetic forms include HUS due to deficiencies of factor H, membrane cofactor protein, Von Willebrand factor-cleaving protease (ADAMTS 13) and intracellular defect in vitamin B12 metabolism. There are cases of aHUS with autosomal recessive and dominant modes of inheritance. Drug-induced aHUS in post-transplantation is due to calcineurin-inhibitors. Systemic lupus erythematosus and catastrophic antiphospholipid syndrome may also present with aHUS. Therapy is directed mainly towards underlying cause.
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PMID:Haemolytic uraemic syndrome: an overview. 1675 34

The patient was a 13-year-old girl. In August 2000, she presented with a fever, together with diarrhea, vomiting, arthralgia, nasal bleeding and malaise, and was examined by another physician. Because her platelet count was low, and there were positive reactions for anti-nuclear antibodies, anti-DNA antibodies and platelet-associated IgG, idiopathic thrombopenic purpura, and systemic lupus erythematosus (SLE) was suspected. From January 2001, when she caught measles, she reported abdominal pain, and urinalysis indicated urinary protein and occult blood, and the left kidney was found hydronephrotic. At the same time left ureter stenosis and dilatation were demonstrated. Symptoms were disappeared by hydration and treatment with NSAIDs, but 2 months later fever and erythematous patches seen on both cheeks led to the proper diagnosis of SLE, and she was admitted to our hospital. Intravenous pyelography revealed hydronephrosis on left kidney, constriction and dilatation of the left ureter, and intracystic endoscopy showed erythema at the orifice of the left ureter. The pathological examination indicated the presence of vasculitis, and finally lupus cystitis was diagnosed. Intravenous cyclophosphamide (IVCY)-pulse therapy was introduced to a total of 8 times over the period of a year, and maintenance therapy with predonisolone and azathioprin was also used. After completion of the IVCY-pulse therapy, the hydronephrosis and constriction of the ureter were disappeared. No side effects of IVCY-pulses were observed, and the patient is now in remission. We reported a case of childhood SLE complicated with lupus cystitis and successfully treated by IVCY-pulse therapy and maintenance predonisolone and azathioprin.
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PMID:[A case report of childhood systemic lupus erythematosus complicated with lupus cystitis]. 1681 64

Mycophenolate mofetil (MMF) is an immunosuppressant drug being used for induction and maintenance of remission of lupus nephritis in systemic lupus erythematosus. Evidence about its use was sought from full publications and abstracts of randomised trials and cohort studies by using a variety of search strategies. Efficacy and adverse event outcomes were sought. Five randomised trials enrolled patients with World Health Organization (WHO) class III, IV, or V (mostly IV) lupus nephritis, predominantly comparing MMF (1 to 3 g daily) with cyclophosphamide and steroid. Complete response and complete or partial response was significantly more frequent with MMF than with cyclophosphamide, with numbers needed to treat of 8 (95% confidence interval 4.3 to 60) to induce one additional complete or partial response, with wide confidence intervals. Death was reported less frequently with MMF (0.7%, 1 death in 152 patients) than with cyclophosphamide (7.8%, 12 deaths in 154 patients), with a number needed to treat to prevent (NNTp) one death of 14 (8 to 48). Hospital admission was also lower with MMF (1.7% versus 15%; NNTp 7.4 [4.8 to 16]). Serious infections, leucopaenia, amenorrhoea, and hair loss were all significantly less frequent with MMF than with cyclophosphamide, but diarrhoea was significantly more common with MMF. Ten of 18 cohort studies enrolled only patients with lupus nephritis (author-defined or WHO class III to V). Seven of these 10 reported that complete or partial response with MMF (mostly 1 or 2 g daily) with steroid occurred in 121/151 (80%) and that treatment failure or no response occurred in 30/151 (20%). Adverse events were generally similar in cohort studies with and without only patients with lupus nephritis. In all 18 cohorts, gastrointestinal adverse events (diarrhoea, nausea, vomiting) occurred in 30%, infection in 23%, and serious infection in 4.3%. Adverse event discontinuations occurred in 14% and lack of efficacy occurred in 10%. There was a single death with MMF, a mortality rate over the course of 1 year of approximately 0.2%. The results form a basis on which to plan future studies and provide a guide for the use of MMF in lupus nephritis until results of larger studies are available. At least one such study is under way.
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PMID:Systematic review and meta-analysis of randomised trials and cohort studies of mycophenolate mofetil in lupus nephritis. 1716 90

Thalidomide, in development by Celgene, inhibits the effects of elevated TNFalpha and may consequently be of use in a range of diseases including cachexia, bacterial meningitis, rheumatoid arthritis, septic shock, AIDS, tuberculosis, multiple sclerosis, ulcerative colitis, graft-versus-host disease and systemic lupus erythematosus. In July 1998, Celgene received clearance from the US FDA to market and sell Thalomid (thalidomide) for the treatment of erythema nodosum leprosum (a severe and debilitating condition associated with leprosy) [291919], following a recommendation for approval by the FDA advisory committee in September 1997 [261846,263970]. In that same month, Celgene filed an IND for the treatment of the chronic autoimmune disorders Behcet's disease, and aphthosis [264366]. The trial will be conducted by investigators at the Mayo Clinic and Bowman Gray School of Medicine. It will be divided into two phases, the first phase lasting 4 weeks in which patients will receive 100 mg thalidomide or placebo, and a second open-label phase which will call back all patients to receive the same dose of thalidomide over a 24-week period. It will be determined whether the drug significantly reduces existing ulcerations and inhibits the formation of new lesions. Positive results of a National Institute of Allergy and Infectious Diseases trial for aphthous ulceration of the mouth in HIV-infected patients prompted Celgene to commence a pivotal trial for the same indication. A total of 84 patients will be randomized to 100 mg, 200 mg or 400 mg thalidomide/day for 4 weeks. Patients achieving a full response after 4 weeks will be re-randomized on 100 mg thalidomide or placebo for up to another year [248356]. The company has also completed the pivotal phase III trial for AIDS-related cachexia [225437]. Results from a pivotal phase II/III trial showed that the drug significantly increased body weight in AIDS patients, but also increased viral load initially. A total of 99 patients, who had lost more than 10% of their body weight due to HIV infection, received either 100 or 200 mg/day of thalidomide or placebo orally for 8 weeks. Although there was a significant increase in body weight associated with the 100 mg dose (p = 0.025), there was no difference in body weight changes between patients treated with 200 mg doses and those on placebo. There was a 55% dropout rate at the higher dose due to side-effects such as somnolence, rash, neutropenia, neuropathy and dizziness. Viral load was significantly increased after 4 weeks of treatment. However, there was no further increase in viral load at 8 weeks, and patients were not receiving triple combination antiviral therapy [243943]. In April 1996, Celgene initiated a phase II trial of thalidomide in London for the treatment of chronic intractable diarrhea in HIV positive patients. The double-blind, placebo-controlled trial will involve up to 120 patients, aged 18 to 65 inclusive, at three centers for 28 days of therapy; those on drugs will be orally dosed with 100 mg of thalidomide daily at bedtime. The primary endpoint is reduction in the occurrence of diarrhea [205006,206218]. The trial will be conducted in the US, the UK and Mexico [210069]. The company expanded its clinical trial program in June 1996, for use of thalidomide in graft versus host disease and AIDS complications, such as debilitating ulcers of the digestive system [212461]. A phase II trial for the treatment of cachexia in cancer patients was carried out at St George's Hospital, London. Ten patients received thalidomide (100 mg) orally for 8 weeks and ten received placebo. The study was structured to determine the ability of thalidomide to reduce or stabilize the symptoms of cachexia. Quality of life and levels of disease markers will also be assessed. Results showed that after a 3-week treatment period, patients who received thalidomide gained an average 4.5% in overall body weight versus 0.9% with placebo [190161]. Results from a 65 patient multicenter phase II/III trial for cachexia are still awaited [221227]. Celgene is also conducting a double-blind, placebo-controlled pivotal trial for the treatment of rheumatoid arthritis at New York University's Hospital of Joint Diseases. Levels of TNFalpha are increased in patients with rheumatoid arthritis. Indicators for the trial will be joint swelling and pain and levels of serological markers [177618]. A separate study is being conducted by the US National Institute for Allergy and Infectious Diseases, of thalidomide in combination with Chiron's IL-2 for the treatment of HIV infection [192218]. In vitro evidence suggests that thalidomide can inhibit the replication of HIV type 1 [169245]. In addition to the associated patent, WO-09214455, which discloses the use of thalidomide in TNF-related diseases, another Celgene patent, US-05463063, discloses a scaleable process to make high purity thalidomide [194937].
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PMID:Thalidomide Celgene Corp. 1846 84


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