上海单中心住院儿童风湿性疾病谱演变

目的:了解上海单中心风湿性疾病住院患儿疾病谱的变化趋势,提高对儿童风湿性疾病的认识。方法:回顾性分析2005—2016年复旦大学附属儿科医院5950例患者的临床资料,采用χ2检验进行发生率的比较和分析。结果:①住院例数位列前3位的分别是:川崎病2633例(44.3%),过敏性紫癜(HSP)2109例(35.4%),幼年特发性关节炎(JIA)574例(9.6%)。②除外HLP,其余病种住院人数均呈上升趋势。③近6年住院的风湿性疾病种类由原来的17种增长到目前37种。④SLE患者逐年增长(112/2348和197/3602,χ2=1.41,P=0.235),重症狼疮患者数亦较前增多(35/112和55/197,χ2=0.38,P=0.536)。⑤风湿性疾病合并巨噬细胞活化综合征(MAS)的发生率为7.2‰(43/5950),幼年型关节炎伴全身发作(sJIA)中有12.9%(26/201)出现过MAS,占风湿性疾病合并MAS总数的60.5%(26/43)。近6年风湿性疾病合并MAS(χ2=14.1,P<0.01),及sJIA合并MAS均明显增多(χ2=11.2,P<0.01)。⑥1.1%(64/5950)风湿性疾病相关肺部病变,幼年型皮肌炎(JDM)中24.4%(20/82)合并风湿性疾病相关肺部病变,占风湿性疾病相关肺部病变总人数的31.3%(20/64)。近6年风湿性疾病并发相关肺部病变及患者明显增多(χ2=5.66,P=0.017)。⑦儿童风湿性疾病病死率为3.7‰(22/5950),45.5%发生于SLE(10/22)。近6年SLE病死率有所下降(5/112和5/197,χ2=0.34,P=0.558)。结论:风湿性疾病住院患者病种及人数由多到少依次为川崎病、HSP、JIA、SLE及JDM。在每年总患者数相对稳定情况下,少见、疑难、危重病种逐年增多。虽近6年SLE仍是风湿性疾病主要死因,但病死率逐年下降。     

Pulmonary alveolar hemorrhage in patients with rheumatic diseases in Korea

Pulmonary alveolar hemorrhage (PAH) is a rare and often fatal presenting feature of rheumatic diseases, with high mortality rate ranging from 40% to 90%. This study was undertaken to review the clinical manifestations, disease course, prognosis, and treatment of PAH in rheumatic diseases in Korea. A retrospective analysis was performed from October 1995 to March 1999 at the Samsung Medical Center. Ten cases were diagnosed as having pulmonary hemorrhage with rheumatic diseases that comprised the following: 6 systemic lupus erythematosus (SLE), 3 microscopic polyangiitis (MPA), and 1 mixed connective tissue disease (MCTD). In 80% of the patients in the present series, PAH was the first clinical manifestation of rheumatic diseases. The most consistent systemic manifestation occurring in conjunction with PAH was renal involvement (80%). The overall patient mortality rate was 50% (5/10) in the current series. Our study suggests that PAH often occurs as the first clinical manifestation of rheumatic diseases and needs urgent medical treatment including plasmapheresis in addition to cyclophosphamide and methylprednisolone.     

Risk factors and clinical features for tuberculosis among patients with systemic lupus erythematosus in Hong Kong

OBJECTIVE: To determine the risk factors associated with and to describe the clinical course of tuberculosis (TB) in patients with systemic lupus erythematosus (SLE). METHODS: Clinical features of patients with TB during the 1-year period prior to the diagnosis of TB were compared with controls. RESULTS: Of the 526 SLE patients, 57 (11%) had TB. Extra-pulmonary or miliary TB occurred in 67%. Patients with TB were more likely to have organic brain syndrome, vasculitis, and nephritis: and they were also more likely to have received intravenous 'pulse' methylprednisolone or high cumulative dose of prednisolone. The cumulative dose of prednisolone and presence of nephritis were independent risk factors for the development of TB using multivariate regression analysis. CONCLUSIONS: There is a high prevalence of TB, especially extra-pulmonary diseases, among SLE patients in Hong Kong. High cumulative dose of corticosteroid and lupus nephritis are important risk factors for the development of TB.     

Tuberculosis prophylaxis in patients with steroid treatment and systemic rheumatic diseases. A case-control study

OBJECTIVE: The aim of this study was to assess the impact of isoniazid prophylaxis in patients with systemic rheumatic diseases who attended a teaching hospital in Mexico City between 1987 and 1992. METHODS: In this case-control study, patients with systemic rheumatic diseases and tuberculosis (cases) were compared with patients with systemic rheumatic diseases without tuberculosis (controls). The groups were matched by year of hospital admission and rheumatic disease. Clinical charts were reviewed for: 1) isoniazid prophylaxis, defined as the administration of isoniazid 300 mg/day for 6 or more months in patients with exposure to steroids (prophylaxis with isoniazid was defined as complete, incomplete or any prophylaxis); 2) exposure to steroids: defined as the administration of prednisone > 15 mg/day (or its equivalent of another steroid) for 3 or more months before tuberculosis or recruitment into the study; 3) exposure to immunosuppressants, defined as the administration of any dose of azathioprine, methotrexate, cyclophosphamide, and/or 6-mercaptopurine, before tuberculosis in the cases or recruitment date in the controls; 4) reactivity to PPD; and 5) other relevant variables. RESULTS: Twenty cases and 66 controls were studied. A 70% decrease in the risk of developing tuberculosis was found among patients who received any prophylaxis with isoniazid as compared to controls: OR 0.31, 95% CI 0.09-0.98, p = 0.03. A 97% decrease was seen in those patients who received complete prophylaxis: OR 0.034, 95% CI 0.0001-0.216, p < 0.0001. The protective effect of complete prophylaxis persisted even after controlling for other potential confounders, such as age, gender, rheumatic disease, duration of rheumatic symptoms, and exposure to steroids and/or immunosuppressants. CONCLUSION: The results of this study suggest that in countries with a high prevalence of tuberculosis the use of isoniazid (300 mg/day for 6 months) in rheumatic patients with exposure to prednisone (> 15 mg/day for three or more months) may be useful to prevent tuberculosis, independently of the results of the PPD reactivity test. However, a controlled clinical trial will be required to confirm these results.     

风湿免疫性疾病并发结核感染的临床特征分析

目的 分析风湿免疫性疾病患者并发结核感染的状况,探讨风湿免疫性疾病患者并发结核感染的特点。方法 对2011年1月至2015年12月深圳市第三人民医院和北京大学深圳医院诊断为风湿免疫性疾病的1218例患者进行结核筛查,对其中并发结核感染的300例患者进行回顾性分析,其中系统性红斑狼疮(systemic lupus erythematosus, SLE)患者32例,强直性脊柱炎(ankylosing spondylitis, AS)患者174例,类风湿关节炎(rheumatoid arthritis, RA)患者73例,干燥综合征(sicca syndrome, SS)患者18例,皮肌炎(dermatomyositis, DM)患者3例。应用结核菌素皮肤试验 (tuberculin skin test, TST)、X线摄影、CT扫描和病原学检查等方法评价结核感染状况,并进行追踪随访24~36个月。结果 300例风湿免疫性疾病并发结核感染患者中,结核潜伏感染(latent tuberculosis infection,LTBI)发生率为86.33%(259/300),非活动性结核病(inactive tuberculosis,IATB)发生率为9.0%(27/300),活动性结核病(active tuberculosis,ATB)发生率为4.67%(14/300);并发结核感染患者中,男性占54.7%(164/300),女性占45.3%(136/300),差异有统计学意义(χ ²=48.368,P<0.01);在结核筛查的1218例患者中,不同风湿免疫性疾病患者并发结核感染率(包括LTBI+ATB+IATB)[SLE占9.5%(32/335),AS占37.7%(174/461),RA占22.7%(73/321),SS占26.4%(18/68),DM占9.1(3/33)]的构成不同,差异有统计学意义(χ ²=88.766, P<0.01);不同风湿免疫性疾病患者并发结核潜伏感染率(TST方法检测)[SLE占8.0%(27/335),AS占33.6%(155/461),RA占18.7%(60/321),SS占23.5%(16/68),DM占3.0(1/33))差异有统计学意义(χ ²=84.971,P<0.01)。 结论 风湿免疫性疾病患者并发结核感染以LTBI为主,AS患者并发结核感染的比例最高,男性多于女性,临床医生应增强认识。     

Autoantibodies to lipoprotein lipase and dyslipidemia in systemic lupus erythematosus

OBJECTIVE: To demonstrate the binding of bovine lipoprotein lipase (LPL) by IgG from sera obtained from patients with systemic lupus erythematosus (SLE) and other rheumatic diseases, and the relationship of anti-LPL to triglyceride levels in SLE. METHOD: Binding of LPL by IgG from sera obtained from patients with SLE and other rheumatic diseases was measured by an enzyme-linked immunosorbent assay technique. Lipid profiles for fasting blood samples obtained from SLE patients and control subjects were determined. RESULTS: Sera obtained from 105 patients with SLE were assessed for reactivity with LPL, and 49 (47%) of the results were positive. Sera obtained from patients with rheumatoid arthritis (RA) (n = 80), Sj?gren's syndrome (n = 30), polymyositis and dermatomyositis (n = 30), and progressive systemic sclerosis (n = 31) were also studied, and 10 (13%), 3 (10%), 12 (40%), and 13 (42%), respectively, were positive for reactivity with LPL. It was determined that all affinity-purified anti-double-stranded DNA (dsDNA) antibodies and 4 of 5 monoclonal anti-dsDNA antibodies bound to LPL. The binding of IgG depleted of anti-dsDNA to LPL indicates a second anti-LPL activity in SLE. Measurements of fasting lipid levels in SLE patients with anti-LPL revealed a strong positive correlation of antibody levels and total serum triglycerides, apolipoprotein B, and apolipoprotein E concentrations. CONCLUSION: Antibodies to LPL occurred in 47% of SLE patients and in a similar percentage of patients with polymyositis or systemic sclerosis. The prevalence of these antibodies was less in patients with RA or Sj?gren's syndrome. It is hypothesized that the elevated triglyceride levels in SLE patients are in part attributable to anti-LPL, and this lipid abnormality could contribute to the premature atherosclerosis known to be present in patients with SLE.     

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.     

Serum isoamylases in patients with autoimmune rheumatic diseases

We studied sera of 107 patients with autoimmune rheumatic diseases (46 with classical rheumatoid arthritis (RA), 36 with systemic lupus erythematosus (SLE) and 25 with primary Sj?gren's syndrome (SS). None of these patients had abdominal pain or gastrointestinal symptoms at the time of blood collection. We used as controls 81 normal age and sex matched volunteers. The presence of hyperamylasemia i) of P-type in 6 of 46 patients (13%) with RA and ii) of P-type and S-type in 11 of 36 patients (30.5%) with SLE and 6 of the 25 patients (24%) with primary SS suggests that asymptomatic pancreatic damage in autoimmune rheumatic diseases may occur frequently especially in patients with SLE. We conclude that the hyperamylasemia in these patients probably reflects a slow, subclinical, inflammatory process of the exocrine glands.     

Adult respiratory distress syndrome in systemic lupus erythematosus: causes and prognostic factors: a single center, retrospective study.

OBJECTIVES: To determine the causes and prognostic factors of Adult Respiratory Distress Syndrome (ARDS) in patients with systemic lupus erythematosus (SLE). METHODS: Among 544 Korean SLE patients, who were followed in the Lupus Clinic of the Catholic Medical Center from 1993 to 1997, patients diagnosed as ARDS were examined retrospectively. During the study period, non-SLE patients with ARDS were investigated and then compared to SLE patients with ARDS in terms of clinical variables. RESULTS: Nineteen patients with SLE were revealed to have a history of ARDS (3.5%) and 13 (68.4%) of 19 patients died. Death related to ARDS was found in 34.2% of all deaths (n=38) from SLE during the study period. The frequency and causes of ARDS were as follows; 9 sepsis or bacteremia (47.4%), 2 miliary tuberculosis (10.5%), 2 invasive pulmonary aspergillosis (10. 5%), 2 acute pulmonary alveolar hemorrhage syndrome (10.5%), 1 acute lupus pneumonitis (5.3%), 1 massive hemorrhage due to placenta previa (5.3%), 1 aspiration pneumonitis (5.3%), 1 disseminated intravascular coagulation associated with systemic vasculitis (5.3%). The main organisms in sepsis were gram negative bacilli (61.5%) The median steroid dose administered 1 month before ARDS was significantly higher in patients (n=13) with infectious ARDS compared to those (n=6) with ARDS due to other causes (P=0.038). Comparison of the laboratory and clinical variables between the survivors (n=6) and the deceased (n=13) showed that the survivors had lower SLAM indices at presentation (P=0.004) and APACHE (Acute Physiology, Age, Chronic Health Evaluation) III scores within 24 h after diagnosis of ARDS (P=0.024) than the deceased. The APACHE III scores correlated well with the SLAM indices (r=0.615, P=0.007). Non-SLE patients with ARDS during the study period were selected for comparison to SLE patients with ARDS. Age at the onset of ARDS was younger in SLE (n=19) compared to non-SLE (n=190) (P<0.001). Duration from ARDS onset to death was shorter in SLE patients (P<0. 001). The mortality from ARDS tended to be higher in SLE patients (P=NS). The first-day APACHE III score was significantly higher in deceased SLE patients (n=13) compared to deceased non-SLE patients (n=105) (P=0.001). CONCLUSIONS: ARDS was a common premortem event of SLE and showed a high fatality rate in SLE. The most common cause of ARDS in Korean patients with SLE was sepsis by gram negative bacilli. ARDS in SLE developed at a younger age, and progressed more rapidly compared to ARDS in general. The SLAM index and APACHE III score could be useful to predict the prognosis of ARDS in SLE.     

Elevation of a tumor associated antigen CA 19-9 levels in patients with rheumatic diseases

We investigated the incidence and characteristics of an elevated tumor associated antigen CA 19-9 in patients with rheumatic diseases. Serum concentration of CA 19-9 was increased in 13 of 39 patients (33.3%) with definite or classical rheumatoid arthritis (RA), in 6 of 19 patients (31.6%) with systemic lupus erythematosus (SLE), in 3 of 9 patients (33.3%) with progressive systemic sclerosis (PSS) and in 9 of the other 35 patients (25.7%). Malignant neoplasm was not detected in any of the patients with rheumatic diseases. Pretreatment of mouse serum with patients' sera did not reduce the measured CA 19-9 values obtained by the conventional assay. The CA 19-9 antigen found in sera from patients with RA was present in a non-IgG fraction, and had the same molecular weight as that in one patient with pancreatic cancer, as determined by gel filtration. These results demonstrated that serum CA 19-9 levels were increased in some patients with rheumatic diseases.     

Central nervous system involvement as the presenting manifestation of autoimmune rheumatic diseases: an observational study using the American College of Rheumatology nomenclature for neuropsychiatric lupus

OBJECTIVE: We sought to describe CNS involvement as initial presentation of autoimmune rheumatic diseases using a standardized nomenclature. PATIENTS AND METHODS: A 6-year observational study (1999-2005) was conducted in the University Hospital of Heraklion Crete, a regional referral secondary/tertiary care academic center. Patients presenting with new neurological symptoms of acute/subacute onset underwent clinical and laboratory screening for systemic autoimmune disorders. The diagnosis of an autoimmune rheumatic disorder was based upon the American College of Rheumatology (ACR) classification criteria, whereas for primary antiphospholipid syndrome (PAPS) we used the Sapporo preliminary criteria. In order to describe the neurological syndromes we used the ACR nomenclature for neuropsychiatric lupus. RESULTS: During this period fourteen patients (ten females and four males) were recorded. Eight patients had systemic lupus erythematosus (SLE), four had primary APS and the remaining two had systemic vasculitis. Four out of the eight SLE patients had secondary APS. Two of them presented with movement disorder (chorea). The other two and all four patients with primary APS presented with cerebrovascular disease (CVD). These six patients comprised the 5.7% of young adults under < 45 years old with cerebrovascular accident admitted over the 6-year period. CONCLUSION: SLE and APS either primary or secondary to SLE were the most common underlying systemic autoimmune rheumatic diseases, in patients presenting with a neurological event of acute onset. Young adults (< 45 years old) with CVD should undertake screening for SLE/APS.     

Value of anticardiolipin antibodies for monitoring disease activity in systemic lupus erythematosus and other rheumatic diseases

Summary The prevalence of anticardiolipin antibodies in active systemic lupus erythematosus (SLE) was compared with that in inactive SLE and other rheumatic and non-rheumatic diseases to determine the value of these autoantibodies in monitoring rheumatic diseases. Pairs of IgG- and IgM-aCL were measured by ELISA in 173 consecutive hospitalised patients, including 141 with rheumatic diseases (18 active SLE, 21 inactive SLE, 19 rheumatoid arthritis, 13 reactive arthritis, 7 other spondyloarthropathies, 16 vasculitis, 47 other autoimmune diseases) and 32 non-rheumatic controls. A further 101 aCL pairs were determined during follow-up in 19 patients with SLE. Serum concentrations were analysed with respect to SLE activity and compared between the different patient groups. IgG- and IgM-aCL levels in excess of 10 GPL and 9 MPL respectively were considered positive. 30.6% of all patients (53/173) were found to be positive for IgG-aCL, as against only 9.8% (17/173) for IgM-aCL. IgG-aCL serum levels in active SLE differed significantly from all other groups, including inactive SLE (all p<0.005). Median IgM-aCL levels were below the cut off point in all groups, although measurable values were obtained almost exclusively in active SLE and RA. In this study IgM-aCL measurement was of less value in monitoring rheumatic diseases. IgG-aCL positivity in SLE was associated with a significantly higher odds ratio (OR) for active disease (OR 16.0, 95% confidence interval: 2.8–90.0). The results show that disease activity in SLE was accompanied by significantly increased IgG-aCL, whereas no elevation was found in other diseases. This parameter may therefore be useful in monitoring SLE activity.     

Autoantibodies to tissue transglutaminase in Sjögren's syndrome and related rheumatic diseases

OBJECTIVE: Sj?gren's syndrome (SS) has been reported in up to 15% of patients with biopsy proven celiac disease (CD). The diagnosis of CD in the setting of SS and other systemic rheumatic diseases can be difficult because they are often associated with a number of gastrointestinal symptoms and diseases. Although the diagnosis of CD is often confirmed by a small bowel biopsy, marker autoantibodies directed against the endomysium of transitional epithelium (EMA) and tissue transglutaminase (tTG) are highly correlated with biopsy-proven disease and serve as a valuable screening test. We used an IgA-anti-tissue transglutaminase antibody (anti-tTG) ELISA to assess the prevalence of anti-tTG in an unselected cohort of patients with SS and other systemic rheumatic diseases. METHODS: Sera from 50 patients with SS, 50 with systemic lupus erythematosus (SLE), 50 with rheumatoid arthritis (RA), 30 with systemic sclerosis (SSc), and 50 healthy controls were tested for autoantibodies to tTG. A comparison group of 40 sera from patients with biopsy-confirmed CD was also included. IgA anti-tTG was measured by a commercially available ELISA kit (Inova, San Diego, CA) that employs purified tTG. RESULTS: Six of the 50 (12%) IgA sufficient SS patients had anti-tTG compared to 2 (4%) normal sera, 3 (6%) SLE, 2 (7%) SSc, and 1 (2%) RA. By comparison, in the CD cohort, 33 (83%) had anti-tTG. Five of 6 SS patients with anti-tTG had symptoms, signs, or small bowel biopsy findings consistent with a diagnosis of CD. IgA anti-tTG and EMA were accompanied by other IgA autoantibodies in SS sera. CONCLUSION: Anti-tTG ELISA is a reliable method to indicate a coexisting diagnosis of CD in patients with SS. Interestingly, the frequency of false positive tTG tests in any of the systemic rheumatic diseases is not significantly greater than in controls. Further, our study shows that anti-tTG is more prevalent in SS than in other systemic rheumatic diseases. The tTG ELISA may be used as a screening test to identify patients with SS who are at risk and require further evaluation for the presence of CD.     

Elevated serum B lymphocyte stimulator levels in patients with systemic immune-based rheumatic diseases.

OBJECTIVE: To determine whether serum levels of B lymphocyte stimulator (BLyS) are elevated in patients with systemic immune-based rheumatic diseases and correlate with serum Ig levels and/or autoantibody titers. METHODS: Sera from 185 patients with various systemic immune-based rheumatic diseases (95 with systemic lupus erythematosus [SLE], 67 with rheumatoid arthritis [RA], 23 with other diagnoses) were assayed for BLyS and Ig. In 7 patients who required arthrocentesis of a swollen knee, coincident serum and synovial fluid samples were assayed for BLyS. Medical charts were retrospectively reviewed for elevated autoantibody titers and proteinuria within a 1-month period before or after collection of sera for BLyS and Ig determination. Sera concurrently collected from 48 normal healthy subjects served as controls. RESULTS: Serum BLyS levels were elevated in 38 of 185 patients (21%) and correlated significantly with serum IgG levels. Serum BLyS levels did not correlate with the patients' age, sex, race, or medications, but correlated positively with anti-double-stranded DNA antibody titers among SLE patients and with rheumatoid factor titers among seropositive RA patients. In contrast, serum BLyS levels correlated inversely with nephrotic-range proteinuria among SLE patients. In every case tested, BLyS levels in clinically inflamed synovial fluids were greater than those in simultaneously obtained sera. CONCLUSION: BLyS may be an important factor in driving polyclonal hypergammaglobulinemia and elevated autoantibody titers in patients with systemic immune-based rheumatic diseases. Local production of BLyS in the joints may contribute to joint pathology. Patients with elevated serum BLyS levels may be ideal candidates for therapeutic targeting of BLyS.     

Anti-telomere antibodies in systemic lupus erythematosus (SLE): a comparison with five antinuclear antibody assays in 430 patients with SLE and other rheumatic diseases

Objective: To investigate the prevalence and diagnostic significance of antibodies against telomeric DNA in systemic lupus erythematosus (SLE) and other autoimmune rheumatic diseases, and to make comparisons with five conventional anti-DNA or anti-nuclear antibody (ANA) assays. Methods: Antibodies to telomeres, which are highly repetitive sequences of DNA (TTAGGG/CCCTAA) at the end of eukaryotic chromosomes, were measured by an enzyme linked immunosorbent assay (ELISA) in 305 patients with SLE and 125 patients with other autoimmune rheumatic diseases (78 rheumatoid arthritis, 32 primary Sjögren''s syndrome, eight mixed connective tissue disease, seven miscellaneous rheumatic diseases). Other assays used were two commercial ELISA assays for anti-dsDNA using calf thymus as antigen, Crithidialuciliae immunofluorescence, and radioimmunoassay (RIA) for anti-dsDNA and immunofluorescence using Hep-2 cells for ANA. Results: The prevalence of anti-telomere in SLE was 60%, v 5% in rheumatoid arthritis and 18% in other autoimmune rheumatic diseases. Specificity of anti-telomere for SLE was 91%; positive and negative predictive values were 95% and 46%, respectively. For anti-dsDNA by two ELISA assays using calf thymus as antigen, sensitivities were 69% and 29% and specificities 66% and 96%, respectively. Other anti-dsDNA assays had low sensitivities (RIA 43%, Crithidia immunofluorescence 13%). The association of anti-telomere with a history of nephritis in patients with SLE was stronger (p = 0.005) than by any other assay (p = 0.006–0.999). The correlations between the different assays were good (p<0.001 for all comparisons). Conclusions: The new ELISA for anti-telomere antibodies using standardised human dsDNA as antigen is a sensitive and highly specific test for SLE.     

Severe clinical course of systemic lupus erythematosus in the first year of life

Systemic lupus erythematosus (SLE) very rarely occurs before the age of 5. Herein we describe the clinical features of infantile SLE (iSLE) with onset during the first year of life. The clinical and laboratory characteristics of iSLE patients followed at the Department of Pediatrics of Padua were analyzed. They were combined with those collected from the literature by performing a systematic literature search on PubMed using the following keywords: SLE, infant, laboratory, therapy, and outcome. A total of 13 patients with iSLE, 2 from our Institution and 11 from the literature, are included in this review. Seven (53.8%) were females and 6 were males (46.2%). The age at disease onset ranged from 6 weeks to 11 months. In comparison with juvenile systemic lupus erythematosus (jSLE), iSLE showed a higher prevalence of positive family history for autoimmune diseases, systemic symptoms at presentation, internal organs involvement, and shorter time between symptoms onset and diagnosis. Anemia and thrombocytopenia were present in the majority of the patients at diagnosis, whereas leukopenia was rarely observed. The overall prognosis in iSLE was very poor: 5/13 infants died between 2 and 31 months after the onset, and 5/13 had severe disease course with residual organ damage. SLE can start as early as during the first year of life and is more severe than in the later age groups.     

Incomplete lupus erythematosus

Thirty-eight patients with incomplete lupus erythematosus (ILE) (defined as the presence of fewer than four of the criteria of the American College of Rheumatology for systemic lupus erythematosus [SLE]) were identified and compared with 42 patients with SLE. Both groups were comparable with respect to age, sex, and race. Patients with ILE had symptoms for an average of 38 months before seeking rheumatologic care and were followed up for a mean of 19 months; patients with SLE averaged 9 months with symptoms before their diagnosis was made and were followed for a mean of 30 months. Characteristic clinical features of patients with ILE included positive antinuclear antibody titers (83%), polyarticular nonerosive arthritis (47%), and cutaneous findings (61%). These were comparable with findings in the the SLE group. However, patients with ILE had significantly fewer systemic manifestations than did those with SLE. Patients with ILE were treated with nonsteroidal anti-inflammatory drugs more frequently (47%) than were patients with SLE, while the latter group received more topical and oral corticosteroids and immunosuppressives. Only two of the patients with ILE went on to have typical SLE. Thus, ILE may be frequent, mild, and relatively stable or benign, apparently evolving slowly if at all into SLE or other rheumatic disease.     

Tuberculosis during infancy.

SETTING: A worldwide re-emergence of tuberculosis has been observed during the last decade. However, few studies of infants with tuberculosis appear in the literature. OBJECTIVE: To describe tuberculosis during infancy. DESIGN: The records of all infants diagnosed with tuberculosis at a tertiary care hospital from 1982 to 1998 were reviewed. RESULTS: Thirty-nine infants with a median age of 10 months were identified, 59% of whom presented during the second half of the study period. Diagnoses included endothoracic tuberculosis (33 patients), meningitis (3), miliary tuberculosis (2) and cervical lymphadenitis (1). Reasons for medical evaluation were the onset of symptoms (25 patients), contact investigation (12) and tuberculin skin test screening (2). Common signs and symptoms included fever (22 patients), cough (7), appetite loss (4) and wheezing/rales (4). Chest X-ray revealed hilar adenopathy (22 patients), infiltrates (16), atelectases (3) and miliary pattern (2). Cultures were attempted in nine patients and were positive in seven. All patients responded promptly to treatment. No complications or deaths occurred. CONCLUSION: Tuberculosis in infants has been diagnosed increasingly during the last decade. Endothoracic tuberculosis predominates. One third of the patients were diagnosed due to contact investigation. As early diagnosis and treatment appears to prevent complications and reduce mortality, pediatricians should be alert for tuberculosis in an infant with an atypical picture suggestive of infection.     

Persistent radiological changes following miliary tuberculosis in miners exposed to silica dust.

SETTING: Silicosis leads to increased susceptibility to tuberculosis, but it has also been suggested that tuberculosis may interact with intra-pulmonary silica to exacerbate fibrotic lung disease. OBJECTIVES: To investigate the possibility that silicosis developed due to or was exacerbated by tuberculosis. METHODS: In a case series of 15 miners presenting with culture-positive miliary tuberculosis, serial radiographs taken premorbidly, at presentation, and after 2 and 6 months of standard anti-tuberculosis treatment were graded for nodularity using the International Labour Organization system. RESULTS: Increased nodule profusion (compared to premorbid film) remained in 13 (87%) and eight (53%) patients after 2 and 6 months of treatment, respectively, despite clinical improvement in all and documented bacteriological cure in eight (53%). These phenomena, observed irrespective of human immunodeficiency virus (HIV) status, were most pronounced in men with minor premorbid changes. Abnormal pulmonary collagenisation related to silica particles was apparent at post-mortem in two men who died of HIV-associated cryptococcosis after completing TB treatment. CONCLUSIONS: Previous silica exposure appears to result in delayed and potentially incomplete radiological resolution of miliary TB. We postulate that the immune response in tubercles may evoke a 'bystander' fibrotic response, as cytokines play a central role in the pathogenesis of both TB and silicosis.