Serum levels of matrix metalloproteinase‐13 in patients with eosinophilic fasciitis

Matrix metalloproteinase‐13 (MMP‐13), a member of the collagenase family, has been implicated in the pathogenesis of connective tissue diseases characterized by extracellular matrix remodeling. Since serum MMP‐13 levels reflect disease severity of systemic sclerosis and localized scleroderma, we evaluated the clinical significance of serum MMP‐13 levels in eosinophilic fasciitis (EF). All the EF patients had serum MMP‐13 levels lower than the mean – 2SD of healthy controls. Serum MMP‐13 levels were also significantly decreased in EF patients compared with diffuse cutaneous systemic sclerosis, limited cutaneous systemic sclerosis, and generalized morphea patients. Although serum MMP‐13 levels did not reflect any clinical and serological features of EF, these results indicate that MMP‐13 may be involved in the development of this disease.     

硬皮病动物模型建模药物最适浓度探讨

目的  探讨用博来霉素（BLM）制作硬皮病动物模型的最适浓度。方法  将24只BALB/C小鼠随机平均分为3个实验组和1个对照组，各实验组小鼠分别皮下注射200、700和1 000μg/ml浓度的BLM，对照组小鼠皮下注射等量的磷酸盐缓冲液（PBS），注射4周后处死小鼠，取注射部位皮肤行苏木精-伊红染色法（HE）染色、Masson染色、真皮羟脯氨酸含量检测及真皮厚度检测。结果  700和1 000μg/ml浓度的BLM成功建模，但1 000μg/ml的浓度可导致小鼠皮肤浅表溃疡。结论  硬皮病动物模型成功与否与BLM药物浓度高低关系密切，700μg/ml博莱霉素浓度具有较好的建模效果，且不会出现皮肤溃疡的副作用。

     

Discordance of pediatric morphea treatment by pediatric dermatologists

Background/Objectives

Studies describing treatment efficacy in pediatric morphea are lacking. Subspecialists have reached no consensus on how to optimally treat pediatric morphea. The objective of the current study was to describe the most common treatment practices of pediatric dermatologists worldwide who care for children with morphea.

Methods

A survey regarding topical treatment practices of pediatric morphea, with representative case‐based scenarios, was distributed to pediatric dermatologists and results were tallied.

Results

The survey response rate was 13.4%, with 110 respondents in the final analysis. The majority of respondents agreed on red violaceous rim (99%), increased local warmth (75%), raised borders (69%), and dermal thickening (64%) as signs of disease activity. Respondents had less agreement on sclerotic lesions (41%), scaling (43%), dyspigmentation (19%), and atrophy (13%) as signs of disease activity. Ninety‐two percent of respondents used primary therapy or monotherapy with topical medications, including 45% in linear morphea of the limbs and 37% in linear morphea of the head or neck. High‐potency topical corticosteroids were most commonly used (80%), although respondents did not agree on specific regimens. Sixteen different treatment regimens were selected as first‐line therapy for one case scenario of active disease.

Conclusion

The survey found large variation in how pediatric dermatologists treat pediatric morphea. Consensus treatment guidelines developed by pediatric dermatologists and pediatric rheumatologists are urgently needed regarding the efficacy of therapies for pediatric morphea. Prospective studies of treatment efficacy in pediatric morphea are urgently needed as well.     

Systemic sclerosis (scleroderma) in two iron ore mines.

Six males with systemic sclerosis were observed in the work forces of two iron ore mines. The usual spectrum of clinical features encountered in systemic sclerosis patients were present. Histologic examination of pulmonary tissue was performed on three of the cases and showed features of both silicosis and scleroderma but to different degrees and stages of development. Exposure to high levels of silica-containing dusts had occurred in all six cases.     

NAILFOLD CAPILLAROSCOPY: A BLINDED STUDY OF ITS DISCRIMINATORY VALUE IN SCLERODERMA, SYSTEMIC LUPUS ERYTHEMATOSUS, AND RHEUMATOID ARTHRITIS

The appearances of the nailfold capillaries can be used to distinguish between various connective tissue diseases. In a study of 30 patients (10 with scleroderma, nine with systemic lupus erythematosus, and 11 with rheumatoid arthritis), photographs were taken of the eight nailfolds of each patient (thumbs excluded) and then coded. Each of the photographs was later analysed by a rheumatology registrar and an attempt was made to predict the patient's diagnosis using only the appearance of the nailfold. The diagnostic specificity and sensitivity were 89% and 80%, respectively.
The results indicate that nailfold capillaroscopy, performed by a relatively inexperienced observer, can accurately distinguish between patients with scleroderma and those with systemic lupus erythematosus or rheumatoid arthritis. (Aust NZ J Med 1986; 16: 457–460.)     

系统性硬皮病的肾脏损害

目的：研究系统性硬皮病（ＳＳｃ）的肾脏损害、肾危象的发生、临床表现及可能的发病原因。方法：对１０５例ＳＳｃ临床及实验室资料作回顾性分析。对４例ＳＳｃ用浆水激发雷诺现象后观察肾眼质血流情况。结果：１０５例ＳＳｃ中２５例有肾丰，其中３例发生肾危象。冷水激后肾皮质血流减少。结论：ＳＳｃ肾脏损害发生率为２４％。严重者短期引起死亡。其发生可能是由于自身免疫致肾血管为以及遇冷时肾血管收缩致肾损害加重     

Effects of olopatadine in limited scleroderma with peripheral eosinophils

Scleroderma and eosinophilia often occur together, though the pathogenesis is unclear. We investigated the effect of olopatadine hydrochloride in a series of cases of limited scleroderma (LS). Ten patients with LS and positive eosinophil counts (LSE) were enrolled (average age, 85 years; six men and four women). Serum concentrations of the anti‐Scl‐70 antibody were positive. Olopatadine hydrochloride was prescribed at 10 mg/day for 3 weeks. Serum concentrations of the anti‐Scl‐70 antibody significantly decreased, but changes in eosinophil numbers and percentages in peripheral blood were not significant. Factor analysis suggested a correlation between serum concentrations of the anti‐Scl‐70 antibody and complement C⁴. Olopatadine could be effective in reducing anti‐Scl‐70 antibodies in the elderly with LSE.