Epidural abscess due to Salmonella enteritidis in a patient with systemic lupus erythematosus.

Increased susceptibility to infection has long been observed among patients with systemic lupus erythematosus, and still represents a chief cause of morbidity and mortality in these patients. This is due in part to the severity of infection and to the difficulty of early diagnosis owing to the similarity between SLE flares and infection. Epidural infection is an uncommon condition, and a most rare condition caused by salmonella, which accounts for a broad spectrum of human illnesses from gastroenteritis and typhoid fever to the asymptomatic carrier state. We report the first case of epidural abscess caused by Salmonella enteritidis in a female with SLE with protean manifestations treated by intravenous antibiotics and surgery with full recovery of neurological symptoms, illustrating the importance of performing an early diagnosis and prompt treatment.

[Synovial lipoma arborescens]. / Limpoma arborescente sinovial.

Synovial lipoma arborescens is a rare and benign intra-articular pathology, of unknown etiology, characterized by a villous and lipomatous proliferation of synovial tissue. It presents with atypical clinical manifestations, usually located in the knee, represented as recurrent joint effusions and painless swelling joint. The magnetic resonance is the most specific test and can often even avoid the synovial biopsy. We related the case of a female patient with mechanical pain in the knee with indolent evolution for 18 years, clinical and radiological compatible with osteoarthritis. With the finding of a localized unilateral increase of the suprapatellar bursa without perceptible joint effusion and ultrasonographic aspect of an exuberant nodular synovitis, the possibility of villonodular pigmented synovitis had to be discarded by synovial biopsy. Even after this procedure, her diagnosis was not clear, being reported to rheumatology evaluation due to histopathology findings confused with rheumatoid arthritis. The set of clinical, laboratory, magnetic resonance and histological review of synovial tissue confirmed the diagnosis of synovial lipoma arborescens, excluding the possibility of rheumatoid arthritis.

Rituximab in refractory autoimmune diseases: Brazilian experience with 29 patients (2002-2004).

OBJECTIVE: Rituximab, a monoclonal antibody against B-lymphocytes that express CD 20, is already available for the treatment of non-Hodgkin's lymphoma. Due to the increased relevance of B-cell regulation in the pathogenesis of autoimmune diseases, rituximab is being used in the treatment of patients whose condition is refractory to conventional therapy. METHODS: We retrospectively evaluated the short-term efficacy and tolerance of rituximab in patients with various autoimmune diseases who were treated at the Hospital Israelita Albert Einstein in the city of Sao Paulo. RESULTS: During the period 2002-2004, 29 patients with various autoimmune diseases were treated with rituximab 375 mg/m2 for 4 consecutive weeks, or two doses of 1 g 2 weeks apart. We observed remarkable short-term results in all cases, except for one patient with thrombocytopenic purpura. Of note, we describe the results in two patients with diseases not previously treated with rituximab (hypergammaglobulinemic purpura of Waldenstrom and eosinophilic fasciitis with hypergammaglobulinemia). Treatment was well tolerated, with no unexpected adverse events. We also observed a marked reduction in steroid dosage. CONCLUSION: Rituximab seems to be safe and effective in the treatment of patients with a variety of autoimmune diseases that are refractory to other modalities of treatment.

Abnormalities in the biochemical markers of bone turnover in children with juvenile chronic arthritis.

OBJECTIVE: To study the biochemical markers of bone turnover in children with juvenile chronic arthritis (JCA) and to compare these parameters with those in healthy children in order to evaluate the relationships between age, disease activity and biochemical variables. METHODS: Sixty-two children with JCA and 157 healthy children were studied. Serum samples were analyzed for their concentrations of minerals, osteocalcin (OC) and bone-specific alkaline phosphatase (BAP). Urine samples were examined to monitor the excretion of hydroxyproline (HYP) and deoxypyridinoline crosslinks (DPD). RESULTS: OC, BAP, HYP/Cr, DPD/Cr values were decreased in healthy girls more than 12 years of age and in healthy boys more than 14 years of age compared to younger children from the same population. Lower levels of OC and BAP were observed in younger children with JCA (girls < or = 12 yrs.; boys < or = 14 yrs.) compared to healthy children of the same age. Older girls with JCA (> or = 13 yrs.) were found to have increased HYP/Cr and DPD/Cr values compared to older healthy children. CONCLUSION: These results indicate that abnormalities of bone metabolism occur in an age-related fashion in JCA. This was demonstrated by a reduction in the markers of bone formation in younger JCA patients. Moreover, in older girls the markers of bone resorption were found to be elevated. Taken together, these findings suggest that bone formation is reduced from early childhood to mid-puberty, while resorption levels increase in children with JCA after this time.

Evaluation by dual X-ray absorptiometry (DXA) of bone mineral density in children with juvenile chronic arthritis.

OBJECTIVE: To study the relationship between bone mineral loss and disease subtype, disease duration and corticosteroid use in children with juvenile chronic arthritis (JCA). METHODS: Bone mineral density (BMD) was evaluated by dual X-ray absorptiometry (DXA), using a Hologic QDR 1000 densitometer. Sixty-two children with JCA and 157 healthy children, aged 5-18 years, were studied. Bone mass was measured in the lumbar spine at the L1-L4 level (LS), in the femoral neck (FN) and in the distal one-tenth radius (DR). RESULTS: A decrease in bone mineral density was observed in 50-60% of the JCA patients in the three regions studied. Those patients who had undergone corticosteroid treatment showed significant bone loss in the DR and LS (trabecular bone), but not in the FN (cortical bone). Bone mass loss was seen for all three disease subtypes, being higher in the patients with polyarticular JCA (particularly in the DR), although this different was not significant. There was a significant difference in disease duration between the children with decreased BMD and those with no BMD decrease in the same regions. CONCLUSION: A decrease in bone mineral density was found in 50-60% of all the JCA patients in this series, regardless of the disease subtype. Corticosteroid use apparently had an effect on the BMD in the trabecular bone. The data also show a correlation between the loss of BMD in both cortical and trabecular bone and a long disease duration.

[Hormonal and biochemical parameters in postmenopausal osteoporosis]. / Parâmetros bioquímios e hormonais na osteoporose pós-menopausa.

In the recent years, many authors had studied the relationship between the calciotropic and gonadotropic hormones actions on postmenopausal bone loss, named calcium negative balance, with different results (Riggs et al 1983; Prince et al, 1995). We evaluated 187 female patients, aged 40 to 80 with the following distribution: 24 normals, 49 patients with osteopenia and 114 patients with osteoporosis, according to WHO classification. The aim of this study was to analyse the relationship between biochemical parameters (seric and urinary calcium), gonadotropic (seric FSH-foliculi stimulant-hormone and seric oestradiol) and calciotropic hormone (PTH) and postmenopausal bone loss. The results had shown the diminution on bone mineral density was related with elevated levels of FSH (p < 0.00001), lower levels of oestradiol (p < 0.00001) and, however, no differences on seric and urinary calcium (respectively, p > 0.70 and p > 0.52) or PTH (p > 0.70) were demonstrated.

Behçet's syndrome/AIDS/cerebral toxoplasmosis: an unusual association.

Few cases of AIDS associated to manifestations suggesting Behçet's syndrome have been reported. This case is of a young married woman who presented, during a period of 7 years, clinical manifestations consistent with the late diagnosis of Behçet's syndrome, when she developed recurrent lymphomonocytic meningoencephalitis. At this time, she was found to be infected by HIV-1. Immunosuppressive doses of glucocorticoid produced an unsatisfactory response and she evolved to death due to CNS toxoplasmosis. The latter diagnosis was presumed on the basis of magnetic resonance imaging findings and proved by necropsy after her third hospital stay. One of the factors hindering the appropriate diagnosis was the low level of CD4 and the CD4/CD8 ratio, sometimes observed in active Behçet's syndrome and higher than those observed in patients with this severe opportunistic infection. No information about the exact period of time she had been infected with HIV-1 is available. So, we do not know whether both diseases were overlooked, if the patient, infected by HIV-1, developed an unusual clinical feature consistent with Behçet's Behçet's syndrome, and subsequently evolved to AIDS.

[Superposition syndrome of connective tissue diseases: current view with special focus on mixed connective tissue disease]. / Síndrome de superposição de conectivopatias: visão atual com enfoque especial para a doença mista do tecido conectivo.

Attention is called to mixed connective tissue disease which, twenty years after it's original description, has now reached the syndromic individualization with important therapeutic and prognostic implications. In particular the discussion concerns the pulmonary complications (hypertension and fibrosis) responsible frequently for fatal outcome.

Antibody to phosphatidylethanolamine in a patient with lupus anticoagulant and thrombosis.

Most patients with lupus anticoagulant (LA) activity have coincident antibodies to a group of negatively charged phospholipids, and its is suggested that LA and anticardiolipin tests detect antibodies with overlapping specificities. Some discordance between the two assays has been described, however. One patient presenting with severe thrombotic disease (recurrent deep vein thrombosis, pulmonary embolism, inferior venocaval obstruction, myocardial infarction, and digital gangrene) showed strong LA activity in February 1987. An enzyme linked immunosorbent assay (ELISA) showed no binding to the negatively charged phospholipids cardiolipin, phosphatidylserine, and phosphatidic acid, but binding to zwitterionic phosphatidylethanolamine (PE) was demonstrated. Inhibition studies and affinity purification confirmed this finding. Interestingly, the serum did not bind to the kaolin cephalin clotting time reagent when used as antigen in an ELISA. The pathogenic significance of anti-PE antibodies and their relation to LA remains to be clarified. Further studies of the occurrence of anti-PE antibodies in patients with LA activity who have negative anticardiolipin tests are suggested.

Efficacy of auranofin as demonstrated by improvement in clinical parameters and decrease in anti-inflammatory usage: a long-term, multicenter study.

An open, noncomparative study at 8 rheumatology centers in Brazil assessed the efficacy and safety of auranofin (AF) when given for up to 24 months. The study enrolled 80 patients with classic or definite rheumatoid arthritis (RA); disease was severe in 20 (25%), moderate in 55 (69%), and mild in 5 (6%). Patients received auranofin, 3 mg twice daily, and varying doses of anti-inflammatory drugs (aspirin, nonsteroidal anti-inflammatory drugs, and corticosteroids). Sixty patients (75%) completed the full 24 months of therapy. No patients were withdrawn from therapy because of insufficient therapeutic effect. There was statistically significant improvement (p less than 0.001) in 9 clinical parameters of disease activity, evident as early as 3 months after beginning AF therapy, increasing steadily over 12 months, and remaining at improved levels for another 12 months. Improvements in some parameters were particularly striking. By 24 months, assessment of well-being had increased by 150%, intensity of pain had decreased by 66%, and duration of morning stiffness had decreased by 78%. The average daily dose of anti-inflammatory drugs also decreased over time. The safety profile of AF was similar to that found in comparable trials. Ten patients (12.5%) were withdrawn because of adverse events: 6 for diarrhea (7.5%), 2 for proteinuria (2.5%), and 1 each for pruritus and anemia (1.25%). Adverse events occurred in 24 of 80 patients; some reported more than one adverse event. The most common adverse events were loose stools (20 patients) or diarrhea (11 patients).(ABSTRACT TRUNCATED AT 250 WORDS)

Open multicentre clinical trial of diftalone in osteoarthrosis.

An open, multicentre trial was carried out in 140 patients with coxarthroxis and/or gonarthrosis to assess the effectiveness of treatment with diftalone. The trial lasted 4 weeks with a drug dosage decreasing from 1000 mg (Week 1) to 750 mg (Week 2), and to 500 mg (Weeks 3 and 4). In almost all of the objective and subjective assessments used for the measurement of effectiveness there was a statistically significant improvement with treatment, which was dose related, the greatest improvement being seen during the first week. Tolerance of treatment was assessed as good in the majority of patients and again, side-effects appeared to be dose related.