某化工厂3例职业性皮肤病的调查

目的调查某化工厂(生产左羟丙哌嗪)3例职业性皮肤病即"硬皮病"样改变的发生的原因。方法根据现场职业卫生调查情况、患者的病历资料及职业病诊断原则进行综合分析。结果病例均发生在产品左羟丙哌嗪预试验期间,因接触大量的苯胺及其化合物导致发生职业性硬皮病样综合征,病程迁延,彻底脱离致病环境者病情好转,严重者呈不可逆性倾向。正式投产运行后职业卫生管理完善,自动化程序提高,防护措施加强,未发生新的病例。结论结合患者的职业接触史及车间发病的情况和临床表现,并参考相关的资料,可以确认该化工厂发生的硬皮病样改变为苯胺及其化合物所致的职业性皮肤病——硬皮病。     

Asymmetric scleroderma in a CVA patient

We describe a systemic sclerosis and cerebral vascular accident case in which the cutaneous manifestation and the distal acroosteolysis occurred in an asymmetrical way in the non-paretic limb. The subsequent sclerodermic alterations and the acroosteolysis acquired an asymmetric pattern, sparing the patient's hemiparetic side. Although a number of definitions of this protective effect may be found in other rheumatic diseases, such as rheumatoid arthritis and gout, we found in the literature only one previous case describing the protective effect of the hemiplegia in scleroderma.     

A study of scleroderma in South Australia: prevalence, subset characteristics and nailfold capillaroscopy

Background: Scleroderma is a systemic rheumatic disorder seen in a wide range of clinical specialties. Aims: To establish the prevalence and mortality rates of scleroderma in South Australia (SA), to determine the relative frequency and characteristics of the three principal subsets (diffuse, limited and overlap), and to examine the role of nailfold capillaroscopy in subset identification and implied prognosis. Methods: Outpatient and discharge diagnostic indexes from five major teaching hospitals in SA were reviewed between February 1987 and November 1993. A total of 215 patients with scleroderma were identified. Case notes of 115 of these patients were reviewed in order to validate scleroderma diagnosis, and subset characteristics such as sex, mean age at diagnosis, extent of skin involvement, internal organ involvement and serology were analysed. Fifty-two of these patients were then examined prospectively to confirm positive discharge diagnosis, and nailfold capillaroscopy was performed on these patients. Results: The point prevalence of scleroderma in SA for 1993 was estimated to be 208/10⁶. This figure is a conservative estimate and is higher than most other reported series. The female to male ratio was 4:1. The majority of patients had limited disease with a ratio of 6:1:1.6 limited vs diffuse vs overlap. Systemic involvement excluding the oesophageal component in limited disease was found predominantly in the diffuse group. Autoimmune serology was positive in 90% of patients, with Scl-70 being more common in diffuse scleroderma, anti-centromere antibody (ACA) in the limited form and anti-ribonucleoprotein (RNP) in the overlap form. Nailfold capillaroscopy was useful in predicting disease-subtype as capillary dilatation was observed predominantly in limited disease, and capillary dropout in diffuse disease. Conclusions: Scleroderma is more common in SA than previously recognised. Limited disease is more common than diffuse or overlap disease, carries a better prognosis and is associated with ACA. Nailfold capillaroscopy is a useful tool in disease assessment and may provide useful diagnostic and prognostic information.     

A case report and literature review of cutaneous intravascular large B-cell lymphoma presenting clinically as panniculitis: a difficult diagnosis,but a good prognosis

Intravascular large B-cell lymphoma is a rare, non-mass-forming, extranodal large B-cell lymphoma subtype characterized by the presence of tumor cells in the lumens of vessels. It is divided into two major types: classical and Asian. Patients presenting only with skin involvement are mostly female, at a younger age than classical intravascular large B-cell lymphoma patients, and have a better prognosis. Since the diagnosis of cases with isolated skin involvement is difficult, keeping this entity in mind, performing a careful microscopic examination, and applying new, effective treatment regimens will make it possible to achieve better clinical outcomes in these cases.     

Clinical features of 405 Japanese patients with systemic sclerosis

Abstract

We aimed to clarify the clinical features of Japanese patients with systemic sclerosis (SSc), especially with reference to organ involvement and autoantibodies. A cohort of 405 patients with SSc who attended our institution from 1973 to 2008 was identified retrospectively. Data on clinical features, including autoantibodies, organ involvement, and overlap of other connective tissue diseases, were obtained by following the medical records until 2009. The percentage of male patients during or after 1990 was greater than that before 1990 (3.9 vs. 10.6%, respectively). Limited cutaneous SSc (lSSc) was twice as frequent as diffuse cutaneous SSc (dSSc). About half of the patients had lung involvement (50.4%), while only 3.2% had scleroderma renal crisis. Male gender was associated with lung involvement, and dSSc was associated with most organ involvements except for pulmonary arterial hypertension (PAH). Anti-Scl-70 antibody was associated with lung or heart involvement, while anti-U1-RNP antibody was only associated with PAH. Conversely, patients with anti-centromere antibody had less organ involvement. SSc–Sjögren overlap syndrome was related to lSSc, further overlapping systemic lupus erythematosus (SLE), and less lung or heart involvement. In conclusion, these results not only confirmed previous reports but revealed several other findings, such as the increased proportion of male patients in recent years and the relationships between clinical features.     

Systemic sclerosis and risk of venous thromboembolism: A systematic review and meta-analysis

Background. Several chronic inflammatory disorders, such as rheumatoid arthritis, inflammatory myositis, and systemic vasculitides, have been linked to an increased risk of venous thromboembolism (VTE). However, the data on systemic sclerosis (SSc) remains unclear.

Methods. We conducted a systematic review and meta-analysis of observational studies that reported odds ratio, relative risk, hazard ratio, or standardized incidence ratio comparing risk of VTE in patients with SSc versus non-SSc participants. Pooled risk ratio and 95% confidence intervals (CIs) were calculated using a random-effect, generic inverse variance method of DerSimonian and Laird.

Results. Out of 776 potentially relevant articles, five eligible studies were identified and included in the data analysis. The pooled risk ratio of VTE in patients with SSc was 2.51 (95% CI, 1.79–3.54). The statistical heterogeneity of this study was high with an I² of 90%.

Conclusions. Our study demonstrated a statistically significant increased VTE risk among patients with SSc.     

Immunohistochemical and morphometric evaluation of COX-1 and COX-2 in the remodeled lung in idiopathic pulmonary fibrosis and systemic sclerosis ,

OBJECTIVE:

To study the expression of COX-1 and COX-2 in the remodeled lung in systemic sclerosis (SSc) and idiopathic pulmonary fibrosis (IPF) patients, correlating that expression with patient survival.

METHODS:

We examined open lung biopsy specimens from 24 SSc patients and 30 IPF patients, using normal lung tissue as a control. The histological patterns included fibrotic nonspecific interstitial pneumonia (NSIP) in SSc patients and usual interstitial pneumonia (UIP) in IPF patients. We used immunohistochemistry and histomorphometry to evaluate the expression of COX-1 and COX-2 in alveolar septa, vessels, and bronchioles. We then correlated that expression with pulmonary function test results and evaluated its impact on patient survival.

RESULTS:

The expression of COX-1 and COX-2 in alveolar septa was significantly higher in IPF-UIP and SSc-NSIP lung tissue than in the control tissue. No difference was found between IPF-UIP and SSc-NSIP tissue regarding COX-1 and COX-2 expression. Multivariate analysis based on the Cox regression model showed that the factors associated with a low risk of death were younger age, high DLCO/alveolar volume, IPF, and high COX-1 expression in alveolar septa, whereas those associated with a high risk of death were advanced age, low DLCO/alveolar volume, SSc (with NSIP), and low COX-1 expression in alveolar septa.

CONCLUSIONS:

Our findings suggest that strategies aimed at preventing low COX-1 synthesis will have a greater impact on SSc, whereas those aimed at preventing high COX-2 synthesis will have a greater impact on IPF. However, prospective randomized clinical trials are needed in order to confirm that.     

SCLERODERMA WITH MASSIVE REGIONAL LYMPHADENOPATHY

Abstract. Scleroderma in a two-year-old boy with gross enlargement of the right inguinal lymph nodes, as an early sign, is reported. Repeated lymph node biopsies revealed non-characteristic reactive changes and hyperplasia, but eventually histological examinations of skin and muscle from the right leg were diagnostic. The effect of one year of D-penicillamine therapy is briefly mentioned.     

A comparative study of arterial stiffness, flow-mediated vasodilation of the brachial artery, and the thickness of the carotid artery intima–media in patients with systemic autoimmune diseases

Patients with autoimmune diseases may have increased vascular risk leading to higher mortality rates. Novel imaging techniques are necessary for the early assessment and management of these patients. In this study, we compared augmentation index (AIx) and pulse wave velocity (PWV), indicators of arterial stiffness, to brachial arterial flow-mediated vasodilation (FMD) and common carotid artery intima–media thickness (ccIMT), standard indicators of endothelial dysfunction and atherosclerosis, respectively. We wished to assess the vascular status of autoimmune patients by using a novel, cheap, and reproducible technique, the arteriograph. Altogether, 101 patients with systemic autoimmune diseases including primary antiphospholipid syndrome, systemic sclerosis, rheumatoid arthritis, and polymyositis, all having various types of vasculopathies, as well as 36 healthy individuals were investigated. Arterial stiffness was assessed by a TensioClinic arteriograph, a recently validated technique. Brachial arterial FMD and ccIMT were determined using high-resolution ultrasonography. Autoimmune patients exerted impaired FMD (3.7 ± 3.8%), increased ccIMT (0.7 ± 0.2 mm), AIx (1.2 ± 32.2%), and PWV (9.7 ± 2.4 m/s) in comparison to control subjects (FMD = 8.4 ± 4.0%; ccIMT = 0.6 ± 0.1 mm; Aix = −41.1 ± 22.5%; PWV = 8.0 ± 1.5 m/s; p < 0.05). We found a significant negative correlation of FMD with AIx (R = −0.64; p < 0.0001) and PWV (R = −0.37; p = 0.00014). There were significant positive correlations between ccIMT and AIx (R = 0.34; p = 0.0009), ccIMT and PWV (R = 0.44; p < 0.0001), as well as AIx and PWV (R = 0.47; p < 0.0001). AIx, PWV, and ccIMT positively correlated and FMD negatively correlated with the age of the autoimmune patients. Arterial stiffness indicated by increased AIx and PWV may be strongly associated with endothelial dysfunction and overt atherosclerosis in patients with autoimmune diseases. Assessment of arterial stiffness, FMD, and ccIMT are reproducible and reliable noninvasive techniques for the complex assessment of vascular abnormalities in patients at high risk.