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At least 325 records · Page 18Linked to original sources
PubMed1999

Oxidative stress in systemic lupus erythematosus and allied conditions with vascular involvement.

OBJECTIVE: To evaluate the occurrence and clinical significance of lipid peroxidation (oxidative stress) in rheumatic diseases characterized by vascular involvement. PATIENTS AND METHODS: Plasma 8-epi-PGF2alpha (oxidative stress marker) was measured by gas chromatography-mass spectrometry in 36 patients with systemic lupus erythematosus (SLE), 13 with systemic sclerosis (SSc), 13 with systemic vasculitis [Wegener's granulomatosis (WG), n = 4; Churg Strauss syndrome (CSS), n = 3; Behcet syndrome, n = 6], 12 with rheumatoid arthritis (RA) and in 23 healthy controls (n = 23). RESULTS: 8-epi-PGF2alpha levels were higher in patients with SLE (P = 0.007), SSc (P < 0.001) and vasculitis (P = 0.001) than in controls. In SLE, a positive Coombs' test and arterial hypertension independently predicted 8-epi-PGF2alpha concentrations (P = 0.004 and P = 0.001, respectively). SLE patients not taking prednisolone showed higher 8-epi-PGF2alpha concentrations than SLE patients on prednisolone (P = 0.02). In the latter group, a dose response relationship was noted between 8-epi-PGF2alpha and steroid dosage (r = 0.6, P = 0.0003). In WG and CSS, 8-epi-PGF2alpha concentrations correlated with disease activity (r = 0.8, P = 0.01) and were higher than in patients with Behcet disease (P = 0.003). CONCLUSIONS: Oxidative stress may be pathogenetically relevant in some autoimmune rheumatic diseases with vascular involvement. Amelioration of some clinical manifestations of these diseases may be envisaged by targeting lipid peroxidation with dietary or pharmacological antioxidants.

Adult↗
PubMed2001

Unusual presentation of neonatal Behcets disease.

Intrauterine growth retardation and neonatal transient mucocutaneous lesions ("transient Behcet syndrome") have been reported in pregnancies complicated by Behcets disease (BD). Neonatal neurological manifestations have not been reported in such pregnancies. Vascular and neurological involvement is known to worsen the prognosis in adults with BD. The clinical course and outcome of a 34-weeks' gestation neonate born to a mother with BD is reported. Progressive recovery from minimal respiratory distress syndrome was followed by catastrophic presentation on 6th day of life with generalized seizures. Cranial ultrasound revealed multiple hyperechoic lesions in the frontal, parietal, and periventricular regions with a few surrounded by a ring of reduced echogenicity suggesting haemorrhage into ischemic areas. Death occurred after withdrawal of life support on Day 9, after extensive discussions with parents in view of the progressive deterioration in the neonates' general condition and the cranial ultrasound findings. Strong family history of BD, clinical course, and laboratory results (no evidence of disseminated intravascular coagulation, normal levels of protein C and S, absence of factor V Leiden and anticardiolipin antibodies) suggested neurological manifestations of BD as the most probable diagnosis.

Adult↗
PubMed2002

Surgical treatment of Budd-Chiari syndrome induced by Behcet's disease.

OBJECTIVE: Although Budd-Chiari syndrome in Japanese is usually chronic, and of unknown etiology and idiopathic, Behcet's disease is rare as an underlying disorder of Budd-Chiari syndrome in Japanese. To clarify the Behcet-induced Budd-Chiari syndrome, the clinical course and pathologic findings of patients with Behcet-induced Budd-Chiari syndrome were compared with those of patients with idiopathic Budd-Chiari syndrome. PATIENTS AND METHODS: We treated 45 patients (15 women and 30 men) with our devised surgical procedure. With normothermic partial bypass, the occluded vena cava and hepatic veins were reopened. The age of the patients ranged from 24 to 76 years (mean, 48.9 13.0 years). In two patients, Budd-Chiari syndrome was induced by Behcet's disease (Behcet group). The other 43 patients (control group) had no distinct underlying disorder. The Behcet group was compared to the control group with regards to (1) onset of symptoms and duration of illness prior to medical treatment, (2) preoperative laboratory data including liver function, (3) intraoperative findings, (4) microscopic findings of liver tissue, and (5) postoperative course. RESULTS: (1) In the Behcet group, duration of illness from diagnosis to surgical treatment was markedly shorter (P=0.027, 8.5 months vs. 10.1 10.6 years). (2) The preoperative laboratory data of liver function were similar in both groups with moderately impaired hepatic function. (3) The Behcet group had no patent hepatic vein (P=0.025 vs. 1.22 0.57). (4) Microscopic examination of the liver tissue showed liver cirrhosis or liver fibrosis in the control group, and centrilobular marked congestion only in the Behcet group. (5) During hospitalization, one patient of the control group died due to preoperative severe hepatic failure. One patient with Behcet's disease underwent reoperation due to reocclusion by Behcet-induced vasculitis, and the other died of peritonitis by intestinal Behcet's disease. CONCLUSION: In Budd-Chiari syndrome in Japanese, the Behcet-induced Budd-Chiari syndrome had an acute clinical course, and its postoperative prognosis depends on the prognosis of the Behcet's disease.

Adult↗
PubMed2004

Neuro-ophthalmologic manifestations of systemic disease: rheumatologic/Inflammatory.

Rheumatologic and inflammatory systemic diseases often cause similar neurologic and neuro-ophthalmologic manifestations. A wide variety of conditions can be included in these categories. This article summarizes the most common of these conditions, including sarcoidosis, idiopathic orbital inflammation, inflammatory bowel disease, Wegener's granulomatosis, polyarteritis nodosa, Churg-Strauss syndrome, Behcet's disease, systemic lupus erythematosis, scleroderma, Sjogren's syndrome, and rheumatoid arthritis.

Eye Diseases↗
PubMed2003

Conn's syndrome associated with Behcet's disease.

A 39-yr-old woman, who had been treated for Behcet's disease for 4 yr, was admitted for further investigation of recently identified hypokalemia and hypertension. Suppressed plasma renin activity with elevated plasma aldosterone concentration and an anomalous postural decrease in plasma aldosterone were observed. An abdominal CT scan revealed a right adrenal mass. The patient was diagnosed with Conn's syndrome. The association of Conn's syndrome with Behcet's disease was thought to be coincidental. To our knowledge, this is the first case of Conn's syndrome associated with Behcet's disease.

Adenoma↗
PubMed2001

Antiphospholipid syndrome and dystonia-parkinsonism. A case report.

Background: Although clinically evident and MRI confirmed, basal ganglia involvement, is usual in primary antiphospholipid syndrome, extrapyramidal disorders such as parkinsonism and dystonia are very rare. We were unable to find any report in the literature on dystonia-parkinsonism in patients with primary antiphospholipid syndrome. Here we report an adult patient with dystonia-parkinsonism and primary antiphospholipid syndrome.Case report: A 60 year old, right-handed man came to our attention due to writer's cramp, bradykinesia and stiffness of his right hand. Neurological examination revealed constant, marked dystonic posturing, rigidity and bradykinesia of the right hand. Hyper-gammaglobulinemia was demonstrated on electrophoresis-serum IgG was increased. Anticardiolipin antibodies were examined by counterimmunoelectrophoresis (ELISA): IgG was negative, while IgM was positive. There was also slight thrombocytopenia. Magnetic resonance imaging brain scan axial T2W/UTSE revealed several hyperintense lesions in the basal ganglia and in the periventricular white matter and diffuse hyperintensity of the subcortical white matter bilaterally in the parietal regions. There was asymmetric parenchimal atrophy, more prominent in the left hemisphere. No clinical improvement was achieved by levodopa, dopamine agonists or anticholinergics. According to the criteria for primary antiphospholipid syndrome our patient had thrombocytopenia and high levels of IgG and IgM anticardiolipin antibodies so he was presumed to have a primary antiphospholipid syndrome.Conclusion: Various movement disorders may appear secondary to stroke, antiphospholipid syndrome, Behcet's disease or brain tumor. These cases may help in the understanding of pathophysiology of movement disorders. Dystonia and parkinsonism as well as other movement disorders may be associated with primary antiphospholipid syndrome.

Journal Article↗
PubMed2001

Tacrolimus in dermatology.

BACKGROUND: Tacrolimus (FK 506), a metabolite of the fungus Streptomyces tsukubaensis, is an anti-T-cell drug. It acts by inhibiting the production of IL-2, IL-3, IL-4, TNFa, and GM-CSF. More potent and with slightly less secondary effects than cyclosporine, it has been the object of considerable interest, especially in conditions that could benefit from the latter. OBJECTIVE: In psoriasis, a placebo-controlled double-blind study has shown oral tacrolimus at 0.1 mg/kg/day to be effective in controlling recalcitrant lesions. In human, small studies have reported tacrolimus ointment to be effective in controlling acute contact dermatitis. Short-term trials of topical tacrolimus in the treatment of atopic dermatitis have recently shown excellent results in both adults and children. In animal studies of hair growth disorders, topical tacrolimus induces anagen and protects from chemotherapy-induced alopecia. Animal studies with the ointment for the prevention of skin graft rejection, lupus dermatoses, and skin papilloma formation have also shown to be promising. CONCLUSIONS: There are case reports of pyoderma gangrenosum, Sezary's syndrome, and Behcet's disease successfully treated with oral tacrolimus but, because of their small number, they remain anecdotal at this point.

Administration, Oral↗
PubMed2005

Potential off-label use of infliximab in autoimmune and non-autoimmune diseases: a review.

TNF-alpha is a crucial cytokine in the establishment and maintenance of inflammation in multiple autoimmune and non-autoimmune disorders. A number of large placebo-controlled trials have shown that infliximab, a chimeric monoclonal antibody against TNF-alpha, is effective and well-tolerated in patients with Crohn's disease and rheumatoid arthritis (RA) and has become a widely used treatment for these diseases. More recent controlled trials have also shown the effectiveness of TNF-alpha blockers in psoriasis, psoriatic arthritis, and ankylosing spondylitis. The results of clinical trials, open-label studies, and case studies indicate that TNF inhibitors (alone or in combination with other protocols) look very promising for the treatment of a variety of other conditions, including uveitis, sarcoidosis, Sjögren's syndrome (SS), Behcet's syndrome, vasculitis, and graft versus host disease. There is a rationale for using TNF blockade even in systemic lupus erythematosus, a prototype of autoantibody-mediated disease, and a pilot study seems to confirm this potential effective approach. The neutralisation of TNF might therefore play a role in the treatment of many autoimmune and non-autoimmune disorders other than Crohn's disease or RA. We here review the current and prospective roles of infliximab in the treatment of autoimmune diseases and other conditions that do not currently have FDA or EMEA approval.

Animals↗
PubMed2003

Therapy of neurological disorders in systemic vasculitis.

Significant progress has been made in our understanding of the underlying cellular and molecular mechanisms associated with vasculitis. Simultaneous involvement of the nervous system and systemic organs points to shared antigenic targets or immune mechanisms operative at both sites. Although specific immunotherapy is not available, the old and new immunomodulatory therapies have been instrumental in providing effective therapy in most of these chronic autoimmune disorders. The treatment of giant cell arteritis, neurosarcoidosis, Wegener's granulomatosis, systemic lupus erythematosus, polyarteritis, Sjögren's syndrome, and Behcet disease will be reviewed.

Adjuvants, Immunologic↗
PubMed2002

Catastrophic antiphospholipid syndrome in a patient with Behçet's disease.

We report on a patient who had a life-threatening relapse of Behçet's disease associated with a catastrophic antiphospholipid syndrome. The patient experienced over a short time a recurrent acute myocardial infarction, multiple venous thromboses, uveitis, and erythema nodosum. Search for thrombophilic factors was positive only for lupus anticoagulant (LAC) and criteria for the diagnosis of the antiphospholipid antibody syndrome were fulfilled. LAC was not found three months after the discharge. At that time the patient had no evidence of clinically active disease or thrombosis. We suggest that LAC was the main triggering factor for the repeated thromboses in this patient.

Acute Disease↗