Relapse rate and renal prognosis in ANCA-associated vasculitis according to long-term ANCA patterns.

Long-term observation of patients with ANCA-associated vasculitis (AAV) allows the identification of different longitudinal patterns of ANCA levels during follow-up. This study aimed to characterize these patterns and to determine their prognostic significance. All ANCA determinations performed in two university hospitals during a 2-year period were retrospectively reviewed. Patients were included in the analysis if they had high titers of anti-myeloperoxidase (anti-MPO) or anti-proteinase 3 (anti-PR3) antibodies at least once, ≥ 5 serial ANCA determinations and AAV diagnosed by biopsy or American College of Rheumatology (ACR) classification criteria. Patients' time-course ANCA patterns were classified as monophasic, remitting, recurrent or persistent. Associations between ANCA patterns and prognostic variables (relapse rate and renal outcome) were analysed by univariate and multivariate statistics. A total of 99 patients [55 with microscopic polyangiitis (MPA), 36 with granulomatosis with polyangiitis (GPA) and eight with eosinophilic granulomatosis with polyangiitis (EGPA)] were included. Median follow-up was 9 years. Among patients diagnosed with MPA or GPA, recurrent or persistent ANCA patterns were associated with a higher risk of clinical relapse [hazard ratio (HR) = 3·7, 95% confidence interval (CI) = 1·5-9·1 and HR = 2·9, 95% CI = 1·1-8·0, respectively], independently of clinical diagnosis or ANCA specificity. In patients with anti-MPO antibodies, the recurrent ANCA pattern was associated with worsening renal function [odds ratio (OR) = 5·7, 95% CI = 1·2-26·0]. Recurrent or persistent ANCA patterns are associated with a higher risk of clinical relapse. A recurrent ANCA pattern was associated with worsening renal function in anti-MPO-associated vasculitis.

First month prednisone dose predicts prednisone burden during the following 11†months: an observational study from the RELES cohort.

AIM: To study the influence of prednisone dose during the first month after systemic lupus erythematosus (SLE) diagnosis (prednisone-1) on glucocorticoid burden during the subsequent 11 months (prednisone-2-12). METHODS: 223 patients from the Registro Español de Lupus Eritematoso Sistémico inception cohort were studied. The cumulative dose of prednisone-1 and prednisone-2-12 were calculated and recoded into a four-level categorical variable: no prednisone, low dose (up to 7.5 mg/day), medium dose (up to 30 mg/day) and high dose (over 30 mg/day). The association between the cumulative prednisone-1 and prednisone-2-12 doses was tested. We analysed whether the four-level prednisone-1 categorical variable was an independent predictor of an average dose >7.5 mg/day of prednisone-2-12. Adjusting variables included age, immunosuppressives, antimalarials, methyl-prednisolone pulses, lupus nephritis and baseline SLE Disease Activity Index (SLEDAI). RESULTS: Within the first month, 113 patients (51%) did not receive any prednisone, 24 patients (11%) received average low doses, 46 patients (21%) received medium doses and 40 patients (18%) received high doses. There was a strong association between prednisone-1 and prednisone-2-12 dose categories (p<0.001). The cumulative prednisone-1 dose was directly associated with the cumulative prednisone-2-12 dose (p<0.001). Compared with patients on no prednisone, patients taking medium (adjusted OR 5.27, 95% CI 2.18 to 12.73) or high-dose prednisone-1 (adjusted OR 10.5, 95% CI 3.8 to 29.17) were more likely to receive prednisone-2-12 doses of >7.5 mg/day, while patients receiving low-dose prednisone-1 were not (adjusted OR 1.4, 95% CI 0. 0.38 to 5.2). If the analysis was restricted to the 158 patients with a baseline SLEDAI of ≥6, the model did not change. CONCLUSION: The dose of prednisone during the first month after the diagnosis of SLE is an independent predictor of prednisone burden during the following 11 months.

The autoimmune disease-associated IL2RA locus is involved in the clinical manifestations of systemic sclerosis.

Regulatory T cells (T(regs)) are crucial in the maintenance of the immune tolerance and seem to have an important role in systemic sclerosis (SSc). The interleukin 2 receptor α (IL2RA) is an important T(reg) marker, and polymorphisms of IL2RA gene are associated with a number of autoimmune diseases. Therefore, we aimed to investigate for the first time the association of the IL2RA locus in SSc. For this purpose, a total of 3023 SSc patients and 2735 matched healthy controls, from six European Caucasian cohorts, were genotyped for the IL2RA gene variants rs11594656, rs2104286 and rs12722495 using the TaqMan allelic discrimination technology. The overall meta-analysis reached statistical significance when the three polymorphisms were tested for association with SSc, the limited subtype (lcSSc) and anti-centromere auto-antibodies (ACAs). However, no significant P-values were obtained when the ACA-positive patients were removed from the SSc and lcSSc groups, suggesting that these associations rely on ACA positivity. The strongest association signal with ACA production was detected for rs2104286 (P(FDR)=2.07 × 10(-4), odds ratio=1.30 (1.14-1.47)). The associations of rs11594656 and rs12722495 were lost after conditioning to rs2104286, and allelic combination tests did not evidence a combined effect, indicating that rs2104286 best described the association between IL2RA and ACA presence in SSc.

Clinical presentation and outcome of venous thromboembolism in COPD.

Chronic obstructive pulmonary disease (COPD) is a moderate risk factor for venous thromboembolism (VTE), but neither the clinical presentation nor the outcome of VTE in COPD patients is well known. The clinical presentation of VTE, namely pulmonary embolism (PE) or deep venous thrombosis (DVT), and the outcome at 3 months (death, recurrent VTE or bleeding) were compared between 2,984 COPD patients and 25,936 non-COPD patients included in the RIETE (Registro Informatizado de la Enfermedad TromboEmbólica) registry. This ongoing international, multi-centre registry includes patients with proven symptomatic PE or DVT. PE was the more frequent VTE presentation in COPD patients (n = 1,761, 59%). PE presentation was more significantly associated with COPD patients than non-COPD patients (OR 1.64, 95% CI 1.49-1.80). During the 3-month follow-up, mortality (10.8% versus 7.6%), minor bleeding (4.5% versus 2.3%) or first VTE recurrences as PE (1.5% versus 1.1%) were significantly higher in COPD patients than in non-COPD patients. PE was the most common cause of death. COPD patients presented more frequently with PE than DVT. It may explain the worse prognosis of COPD patients, with a higher risk of death, bleeding or VTE recurrences as PE compared with non-COPD patients. Further therapeutic options are needed.

Comparison of the clinical history of symptomatic isolated distal deep-vein thrombosis vs. proximal deep vein thrombosis in 11 086 patients.

BACKGROUND: The clinical significance of symptomatic isolated distal deep vein thrombosis (DVT) is uncertain. Consequently, this leads to important disparities in its management. OBJECTIVE: To examine the clinical history of isolated distal DVT and to compare it with that of proximal DVT. METHODS: Using data from the international, prospective, RIETE registry on patients with confirmed symptomatic venous thromboembolism (VTE), we compared the risk factors and 3-month outcome in patients with isolated distal DVT vs. proximal DVT. RESULTS: Eleven thousand and eighty-six patients with symptomatic DVT, but without pulmonary embolism, were included between 2001 and 2008; 1921 (17.3%) exhibited isolated distal DVT. Anticoagulant treatment was received by 89.1% (1680/1885) of isolated distal DVT and 91.8% (7911/8613) of proximal DVT patients for the entire follow-up period. Isolated distal DVTs were more associated with transient risk factors (i.e. recent travel, hospitalization, recent surgery), whereas proximal DVTs were more associated with chronic states (i.e. > or =75 years or with active cancer). At 3 months, major bleeding rate was lower in patients with isolated distal DVT (1.0% vs. 2.2%, P < 0.01), whereas VTE recurrence rate was equivalent (2.0% vs. 2.7%, P = 0.07). The mortality rate was lower in patients with isolated distal DVT (2.7% vs. 7.5%; P < 0.001); this was mainly due to a lower rate of non-VTE-related deaths (2.2% vs. 6.3%; P < 0.001). Active cancer was the main predictive factor of death in patients with isolated distal DVT. CONCLUSIONS: Proximal and isolated distal DVT patients differ in terms of risk factors and clinical outcomes, suggesting different populations. In the short term, the life expectancy of patients with isolated distal DVT depended chiefly on their cancer status.

Analysis of temporal artery biopsies in an 18-year period at a community hospital.

OBJECTIVE: To identify analytical and clinical variables that may improve the effectiveness of temporal artery biopsy (TAB) for the diagnosis of giant cell arteritis (GCA). MATERIALS AND METHODS: A retrospective study of TABs conducted between 1989 and 2007 at the 450-bed Hospital Parc Taulí, Sabadell. Demographic data, clinical manifestations, analytical data prior to the biopsy and final diagnoses were recorded, including only those cases in which these data were reflected in the clinical history. RESULTS: In this period, 278 TABs were conducted in 181 women (65.1%) and 97 men (mean age 74 years). Seventy-nine (28.4%) were positive (GCA+) and 199 (71.5%) negative (TAB-). The most frequent final diagnoses in the TAB- group were: polymyalgia rheumatica (PMR) (18.6%), giant cell arteritis plus negative TAB (GCA-) (13.6%), tension headache (7.5%), infection (7.5%), other vasculitis (7.5%), and neoplasm (6.0%). The GCA+ group was compared with the TAB- group, the GCA- group and the PMR group. In the multivariate analysis only headache (RR 3.6), jaw claudication (RR 2.9) and abnormal temporal artery on palpation (RR 2.5) revealed statistical differences between the GCA+ and TAB- groups. CONCLUSION: One third of the biopsies performed at our centre were positive for GCA. The clinical variables that best predicted a positive TAB in our series were headache, jaw claudication, and abnormal temporal artery on palpation.

Investigation of TLR5 and TLR7 as candidate genes for susceptibility to systemic lupus erythematosus.

OBJECTIVES: The aim of this study was to evaluate the relevance of genetic variants of TLR5 (rs5744168) and TLR7 (rs179008) gene in systemic lupus erythematosus (SLE) in a Spanish population. MATERIAL AND METHODS: Our study population consisted of 752 SLE patients and 1107 healthy controls. All individual were of Spanish Caucasian origin. The TLR5 and TLR7 polymorphisms were genotyped using a PCR system with pre-developed TaqMan allelic discrimination assay. RESULTS: No statistically significant differences were observed when the allele and genotype distribution of TLR5 rs5744168 and TLR7 rs179008 polymorphisms was compared between SLE patients and healthy controls. A significant increase frequency in the CC genotype of the TLR5 rs5744168 polymorphism among SLE patients without nephritis was found (93% vs. 87% in SLE patients with nephritis, p=0.03, OR=2.11 95%CI 0.93-3.51). However, this difference did not reach statistical significance in the allele frequencies (p=0.08). CONCLUSION: These results suggest that the tested variations of TLR5 and TLR7 genes do not confer a relevant role in the susceptibility or severity to SLE in the Spanish population.

No evidence for genetic association of interferon regulatory factor 3 in systemic lupus erythematosus.

The aim of this study was to determine the potential role of three IRF3 gene polymorphisms (rs2304204, rs7251 and rs2304207) with systemic lupus erythematosus (SLE). Our study population consisted of 610 patients with SLE and 730 healthy controls. All individual were of Spanish Caucasian origin. The IRF3 polymorphisms were genotyped using a PCR system with pre-developed TaqMan allelic discrimination assay. No statistically significant differences were found when allele and genotype distribution of rs2304204, rs7251 and rs2304207 polymorphisms were compared between patients with SLE and controls [overall P values: rs7251, P = 0.06; rs2304204, P = 0.26 and rs2304207, P = 0.36, by chi-squared test on a 3 x 2 contingency table. Overall allelic P values: rs7251, P = 0.8, OR (95%CI) = 1.03 (0.87-1.22); rs2304204, P = 0.2, OR (95%CI) = 1.12 (0.93-1.34) and rs2304207, P = 0.8, OR (95%CI) = 1.02 (0.82-1.26)]. In addition, no evidence of association with haplotypes and clinical features of SLE was found. Our data suggest that the IRF3 polymorphisms do not appear to play a major role in the susceptibility or severity of SLE in a Spanish population.

Reticulated platelets as a screening test to identify thrombocytopenia aetiology.

BACKGROUND: Thrombocytopenia is a common haematological abnormality and no simple diagnostic test is available to diagnose thrombocytopenia pathogenesis. AIM: To evaluate sensitivity and specificity of reticulated platelets (RP) as a diagnostic test for thrombocytopenia with increased thrombopoietic activity. DESIGN: Prospective observational study in thrombocytopenic patients. METHODS: A direct, whole-blood, dual-labelling flow cytometric method was used. Direct, whole-blood double coverage was achieved using a monoclonal anti-glycoprotein (GP)-III antibody (CD61 PerCP) for platelet identification and thiazole orange (Retic-count) as platelet mARN stain. RESULTS: RP were measured in 101 thrombocytopenic patients and 104 non-thrombocytopenic controls. The mean RP percentage in 60 thrombocytopenic patients with no increased thrombopoietic activity was 7.5% (CI for 95%: 5.2-9.7) and RP absolute number was 3.2 x 10(9)/l (CI for 95%: 2.1-4.3). The mean RP percentage in 41 thrombocytopenic patients with increased thrombopoietic activity was 30.3% (CI for 95%: 25.1-35.5) and RP absolute number was 6.2 (CI for 95%: 4.8-7.7). The RP percentage cut-off for a diagnosis of thrombocytopenia with increased thrombopoietic activity was 11% [sensitivity 93%, specificity 85%, positive predictive value (PPV) 83%, negative predictive value (NPV) 95%]. CONCLUSION: RP measurement by flow cytometry, directly from whole-blood, is a useful screening test to differentiate between thrombocytopenia with high or low thrombopoietic activity. A RP percentage in excess of 11%, has a high sensitivity and good specificity for a diagnosis of thrombocytopenia with increased thrombopoietic activity.

Fatal pulmonary embolism and fatal bleeding in cancer patients with venous thromboembolism: findings from the RIETE registry.

OBJECTIVES: To examine the clinical characteristics and outcomes of cancer patients with venous thromboembolism (VTE) in order to identify factors that place these patients at an increased risk for fatal pulmonary embolism (PE) or fatal bleeding. PATIENTS AND METHODS: Registro Informatizado de la Enfermedad Trombo Embólica (RIETE) is a prospective registry of consecutive patients with symptomatic, objectively confirmed, acute VTE. RESULTS: Up to January 2006, a total of 14 391 patients with symptomatic acute VTE were enrolled in RIETE, of whom 2945 (20%) had cancer. During the 3-month follow-up period the frequency of fatal PE in cancer patients was 2.6%, and that of fatal bleeding 1.0%. These frequencies were significantly higher than in VTE patients without cancer (1.4% and 0.3%, respectively). In patients with cancer, abnormal renal function, metastatic disease, recent major bleeding and recent immobility for >or= 4 days (42% of the 108 patients who died from PE or bleeding had recent immobility) were factors independently associated with an increased risk for both fatal PE and fatal bleeding. In addition, PE diagnosis on admission was an independent risk factor for fatal PE, while body weight < 60 kg was an independent risk factor for fatal bleeding. CONCLUSIONS: Both fatal PE and fatal bleeding are more common in cancer patients with VTE than in those patients without cancer. In cancer patients, abnormal renal function, metastatic disease, recent major bleeding and recent immobility for >or= 4 days are associated with an increased risk for both fatal PE and fatal bleeding.

Antimalarial myopathy: an underdiagnosed complication? Prospective longitudinal study of 119 patients.

OBJECTIVES: To evaluate the prevalence and incidence of antimalarial myopathy in patients with rheumatic diseases treated with antimalarial drugs. METHODS: Over a three year period, all patients with rheumatic diseases who were taking antimalarial drugs were studied. Serum muscle enzymes were assessed at the time of inclusion and every six months thereafter. Muscle strength, electromyography (EMG), and muscle biopsy were assessed in patients with a persistent muscle enzyme disturbances. RESULTS: 119 patients were included (111 chloroquine, eight hydroxychloroquine). Of these, 22 (18.5%) had a persistent muscle enzyme disturbance: lactate dehydrogenase 19/22 (86%); creatine kinase 7/22 (32%), and aldolase 3/22 (14%). Findings of antimalarial myopathy were detected in 3/15 biopsied patients (20%) by light microscopy and in all 15 by electron microscopy. Eleven patients had myopathy at the time of inclusion (prevalence 9.2%) and four patients developed muscle injury during follow up (annual incidence 1.2%). Muscle weakness was observed in 8 of 15 patients with biopsy proven myopathy, giving a prevalence of clinical antimalarial myopathy of 6.7%. All these patients also had a myopathic pattern on electromyography. CONCLUSIONS: The prevalence of antimalarial myopathy is higher than previously recognised when muscle enzyme determination is used as a screening method. When a persistent muscle enzyme disturbance is observed, clinical and electromyographic studies should be undertaken periodically to detect the development of clinical myopathy. In cases of clinical myopathy, an anatomical-pathological tissue study, including an ultrastructural study, is mandatory to confirm the diagnosis.

[40-year-old man with primary antiphospholipid syndrome]. / Varón de 40 años de edad con síndrome antifosfolópido primario.

We report the case o a 40-year-old male patient with "primary antiphospholipid syndrome" who developed ischemic cerebral infarctions and renal microangiopathy with infarction. A review of the literature on renal involvement in the primary antiphospholipid syndrome disclosed the differences from the antiphospholipid syndrome in the systemic lupus erythematosus. We describe the evolution of the patient at eight years, and we emphasize the importance of the treatment with warfarin. Also, we review the pathophysiology of severe secondary arterial hypertension.

[Procalcitonin in the early diagnosis of invasive bacterial infection in febrile infants]. / Procalcitonina para el diagnóstico precoz de infección bacteriana invasiva en el lactante febril.

BACKGROUND: Procalcitonin (PCT) it is a new marker of bacterial infection. Because of its shorter half-life and earlier ascent it offers advantages over C-reactive protein (CRP). OBJECTIVE: To compare the diagnostic performance of PCT in the early detection of invasive bacterial infection in infants with that of CPR. MATERIAL AND METHODS: Between January of 1998 and February of 2000 we performed a prospective observational study in the emergency department of infants aged between 1 and 36 months who had been treated for fever and for whom PCT and CRP plasmatic values had been obtained. Plasmatic PCT and PCR values were evaluated and correlated with the final diagnosis. ROC curves for both markers were calculated. RESULTS: One hundred infants with a mean age of 8.8 months (SD 7.59) were included in four groups of 25 patients each (viral infection, localized bacterial infection, invasive bacterial infection and control group). The mean PCT and CRP values in invasive bacterial infections [PCT: 14.45 ng/mL (SD 27.95) and CRP: 95.10 mg/L (SD 7 2.77)] were significantly higher than in non-invasive infections [PCT: 0.27 ng/mL (SD 0.19) and CRP: 25.67 mg/L (SD 33.04)] but the diagnostic performance of PCT was better. The area under the curve for PCT was 0.95 (SD 0.03), which was significantly higher (p < 0.001) than that obtained for CRP [0.81 (SD 0.05)]. The optimal cut-off for PCT was > 0.4 ng/mL (sensitivity: 95.5 %; specificity: 86.4 %) and that for CRP was > 42.9 mg/L (sensitivity: 75 %; specificity: 81.8 %). In infants who had fever for less than 12 hours (n 30) the area under the curve for PCT was 0.90 (SD 0.06), which was higher (p < 0.001) than that for PCR [0.64 (SD 0.11)]. The optimal cut-off for PCT in this group was > 0.4 ng/mL (sensitivity: 90 %; specificity: 94 %) and that for CRP was > 26.6 mg/L (sensitivity: 60 %; specificity: 77.8 %). CONCLUSIONS: The diagnostic performance of PCT was higher than that of CRP in the early detection of invasive infection in febrile infants, even when evolution was less than 12 hours.

Metal extraction from road sediment using different strength reagents: impact on anthropogenic contaminant signals.

Washoff of road deposited sediment into storm drain systems is one of the major contributors to nonpoint source pollution in urban fluvial networks. These sediments contain a variety of potentially toxic organic and inorganic contaminants. Road sediment from 13 locations in an urban (non-industrialized) drainage basin, and soil from 10 background (control) locations were collected to assess total and labile fractions of Al, Co, Cu, Fe, Mn, Ni, Pb and Zn. Four digestions, of varying strength, were used to assess contaminant levels, these included: a total four-acid digestion, a microwave-assisted digestion with concentrated nitric acid (USEPA Method 3051), a 0.5 M 'cold' HCl, and a 0.05 M EDTA (pH 7). Road sediment data indicate that Al, Co, Fe, Mn and Ni were primarily lithogenic in origin, while Cu, Pb and Zn showed very significant anthropogenic signals, most probably from vehicle-related sources. Median Pb concentration enrichment ratios for the EDTA extraction were about 42, indicating an extreme anthropogenic signal. The weak extractants (HCI and EDTA) are considered in this study to be superior in their ability to characterize the degree of anthropogenic contamination and should be utilized more widely in environmental contaminant studies.

Idiopathic pylephlebitis and idiopathic sclerosing peritonitis in a man with protein S deficiency.

Idiopathic pylephlebitis and primary sclerosing peritonitis are two highly unusual entities. To our knowledge, the association of the two diseases has not been described previously. We report a 42-year-old patient with a protein S deficiency who presented with fever and chills, in whom idiopathic pylephlebitis was diagnosed. A year later, the patient was readmitted because of recurrent vomiting and weight loss. An exploratory laparotomy yielded diagnosis of sclerosing peritonitis, which resolved after surgery. The short time interval between the processes suggests that they were related to each other, and also to the protein S deficiency.

[Graves-Basedow's disease in childhood and adolescence: clinical and biologic features in 49 patients]. / Enfermedad de Graves-Basedow en la infancia y la adolescencia: aspectos clinicobiológicos en 49 casos.

INTRODUCTION: Graves' disease is rare in childhood and adolescence. PATIENTS AND METHODS: Six boys and 43 girls between the ages of 2 and 18 years were evaluated. All presented diffuse goiter and symptomatology of hyperthyroidism. Concentrations of T4, FT4, T3 and TSH were determined by RIA with various sensitivities during the study. Thyroid antibodies were determined by hemagglutination. All patients were treated with antithyroid drugs. RESULTS: In five patients, growth velocity increased. In a further five patients association with other autoimmune diseases was detected. Quetelet's index was between the 90th and 97th percentiles in five patients and between 3rd and 10th in eight. Biochemical results were as follows: T4: 282.41 +/- 1.1 nmol/1; FT4: 62.98 +/- 1.1 pmol/l; T3: 4.92 +/- 0. 07 nmol/l) and TSH: > 0.002-0.001 mU/l. Values of TSI/TRAb were 33. 23 +/- 1.95 U/l. Four total remissions and 1 partial remission were observed. Four patients underwent surgery and eight received radioiodine. CONCLUSIONS: Few remissions were obtained with medical treatment. Radioiodine seems to be a therapeutic alternative.

Characterization of selected element concentrations and enrichment ratios in background and anthropogenically impacted roadside areas.

Previous research by the National Contaminant Biomonitoring Program found fish from Manoa Stream, Hawaii, to have the highest concentrations of Pb in the nation. To explore possible source linkages we sampled background (uncontaminated) soil, roadside soil, and road deposited sediment in Manoa watershed and analyzed them for total and extractable concentrations of Al, Co, Cu, Fe, Mn, Ni, Pb, and Zn. Our concentration and enrichment ratio data indicate that Al, Co, Fe, Mn, and Ni were controlled by natural source variations, and Cu, Zn, and particularly Pb were anthropogenically enhanced. Labile Pb concentrations, extracted with either dilute HCl or EDTA, reached a maximum of 3,560 mg/kg compared to background values of <10 mg/kg. Additionally, 25% of the 169 contaminated samples analyzed had EDTA-extractable Pb enrichment ratios > or =11.5, where a value of 1 separates uncontaminated from contaminated media. Given the proximity of most samples to roadways it is reasonable to conclude that automotive emissions plus vehicle wear were the primary contributors of trace metals to the roadside system. These data are discussed in context with previous work on the fluvial bed sediments in this watershed in an attempt to examine the potential linkage with the biological accumulation of Pb in fish from Manoa watershed.

Multi-element analysis of road-deposited sediment in an urban drainage basin, Honolulu, Hawaii.

Road-deposited sediment (RDS) and its associated contaminant load play a critical role in degrading receiving water bodies. Few quantitative multi-element RDS studies exist, and there are none from Hawaii. This lack of baseline data combined with concerns with high concentrations of Pb and Cu in fish and enrichment of Cu, Pb and Zn in bed sediments from Manoa Stream, Hawaii, lead to a detailed characterization of RDSs in Manoa basin. Data for a total analysis of 23 elements using inductively coupled plasma atomic-emission spectrometry and instrumental neutron activation analysis and 16 elements using a 0.5 M HCl partial leach are presented for RDSs and background soils. Concentration data, comparisons with environmental guidelines, and concentration enrichment ratios (CERs) all indicate that RDS in Manoa has a significant degree of anthropogenic pollution. The most environmentally important elements were Pb, Sb and Zn. Concentrations of these elements, primarily vehicle contributed, compare favorably with those from other studies of RDS. The high mean concentration of Pb (151 mg/kg) compared to background soils (13 mg/kg) indicates remobilization of Pb previously stored in soils and transported to road surfaces by water erosion processes. The higher Pb CER(Total) in RDSs compared to bed sediments from Manoa Stream suggests a potential link via ubiquitous storm drains and subsequent dilution with less contaminated fluvial sediments. Data from the HCl leach also support Pb and Zn as having significant anthropogenic signals, and Cu having a moderate signal. These data indicate that RDSs in Manoa basin are generally contaminated with certain potentially toxic elements and the legacy of past use of leaded gasoline is still a concern in this urban drainage system.

Giant cell (temporal) arteritis and secondary renal amyloidosis: report of 2 cases.

Renal involvement is unusual in giant cell (temporal) arteritis (GCA). Microscopic hematuria and minimal proteinuria responsive to steroid therapy have been described as the most frequent renal alterations. The nephrotic syndrome in GCA has been rarely reported. We describe 2 cases of GCA with nephrotic syndrome, one at the time of diagnosis, the other 2 years after treatment. Percutaneous renal biopsy in both cases disclosed secondary renal amyloidosis confirmed by permanganate sensitivity and immunohistochemical analysis. To our knowledge, cases of GCA and secondary amyloidosis have not been reported.

Survival prognostic factors and markers of morbidity in Spanish patients with systemic sclerosis.

OBJECTIVE: To identify survival prognostic factors and markers of morbidity among patients with systemic sclerosis (SSc). PATIENTS AND METHODS: The study included 72 patients diagnosed with SSc. According to the extent of skin involvement, three groups of patients were established: group 1, without sclerosis and with sclerosis of fingers and neck; group 2, with sclerosis of face and distal to elbows and knees; group 3, with generalised sclerosis including the trunk. All patients were included in a study protocol to determine visceral involvement. Cumulative survival after first symptom has been estimated according to the Kaplan-Meier method. The association between a hypothetical prognostic factor and cumulative survival after first symptom was assessed by log rank test. The association between a hypothetical risk factor and the prevalence of severe morbidity was assessed by the odds ratio. Multiple logistic regression models were used to identify the main predictors of severe morbidity. RESULTS: Survival was estimated to be 85% 10 years after first SSc symptom. Survival was higher among SSc patients with skin involvement distal to elbows and knees than among the rest of patients; a forced vital capacity (FVC) on spirometry lower than 70% of expected value was associated with a shorter survival, even after adjustment for diffuse SSc. Skin involvement proximal to elbows or knees was associated with a higher prevalence of severe morbidity (OR = 46.57; p < 0.001). According to a multiple logistic regression, severe morbidity was higher among patients with skin involvement proximal to knees or elbows (OR = 40.92; p < 0.001) or among patients with pulmonary hypertension detected by Doppler echocardiography (OR = 23.66 p < 0.001). CONCLUSIONS: In patients with SSc the extent of skin sclerosis was found to be a determining factor on the prognosis. According to skin sclerosis extent two main subsets of SSc patients with different survival incidence and degree of morbidity could be clearly established: limited SSc, formed by patients with no skin sclerosis or with sclerosis distal to elbows and knees and diffuse SSc, formed by patients with skin sclerosis distal and proximal to elbows and knees. Moreover, lung involvement (FVC < 70% on survival study and pulmonary hypertension on morbidity study) was an important and independent prognostic factor.