[Dermatomyositis combined with IgA vasculitis: A case report].

Dermatomyositis (DM) is an autoimmune disease characterized by muscle involvement of the proximal extremities and specific skin involvement, like Gottron sign and heliotrope rash. HenochSchonlein purpura (IgA vasculitis) nephritis is characterized by hematuria and/or proteinuria clinically, with histologic evidence of IgA nephropathy, and also can be clinically characterized by non-thrombocytopenic purpura, presenting with petechiae and ecchymosis on the skin and mucous membranes, often involving multiple organs and systems, accompanied by abdominal pain, joint swelling and pain, and renal lesions. We reported here a patient with symmetric muscle weakness in her proximal limbs and typical Gottron sign, whose laboratory examination showed elevated creatine kinase (CK) level and myogenic damage electromyographically, which were concomitant with dermatomyositis. We applied prednisone combined with cyclophosphamide, and the patient's muscle strength, interstitial lung disease and all improved gradually. The patient gradually developed severe hepatic damage [significantly increased glutamic-pyruvic transaminase (ALT), glutamic oxalacetic transaminase (AST) and bilirubin], high fever (body temperature fluctuated between 38.0-39.2 °C), thrombocytopenia (limb distal purplish rash, some slightly protruded from the skin surface, some fused into a piece, which did not fade with pressure) and intractable diarrhea (waterlike stool, antidiarrheal drug treatment was not good), with new onset of the skin lesions on multiple areas of her body, as well as abrupt occurrence of massive proteinuria, which resulted in huge challenges in the following diagnosis and treatment. After extensive differential diagnosis from various directions, including pathological biopsies, it finally came out to be dermatomyositis combined with IgA vasculitis, which had been rarely reported. Both cellmediated immunity to muscle antigens and immune-complex disease might participate in the pathogenesis. There was evidence that they were immune complex diseases. Several immune mechanisms played an important role in the pathogenesis of both DM and IgA vasculitis. We conducted a substantial literature review of the above diseases. The purpose of our study is to strengthen the clinical understanding of such complicated diseases, and to highlight the importance of pathological biopsy in the diagnosis (renal biopsy pathology gave us a definite diagnosis). And what is more important is that seizing the opportunity to initiate treatment can control the disease and improve the prognosis.

[Increased serum soluble-endoglin level and its clinical significance in antiphospholipid syndrome].

OBJECTIVE: To detect the serum levels of soluble endothelial glycoprotein endoglin (s-Eng) in patients with antiphospholipid syndrome (APS) and to evaluate the correlation between s-Eng levels and clinical features and laboratory parameters. METHODS: The levels of serum s-Eng were measured by enzyme linked immunosorbent assay (ELISA) in 139 patients with APS, 44 patients with SLE but no APS, 37 patients with primary Sjögren's syndrome (pSS), 23 patients with Bechet's disease (BD), 22 patients with systemic sclerosis (SSc) and 22 persistent anticardiolipin antibody (aCL) positive individuals without SLE or APS (simply aCL positive group) and 87 health controls (HC) without any auto-immune diseases. These APS patients included 64 primary APS patients and 75 APS patients secondary to SLE.The correlation between the clinical data, laboratory parameters, and serum s-Eng levels were analyzed.Independent samples t test, paired t test, Chi-square Test, Mann-Whitney U test, Pearson's χ2 test were used for statistical analyses. RESULTS: (1) The serum levels of s-Eng were significantly higher in the patients with APS whether primary or secondary to SLE than in the health controls and simply aCL positive group and the patients with other autoimmune diseases, including SLE, pSS, BD and SSc (P<0.001). There was no significant difference in the serum s-Eng levels between simply aCL positive group and health controls [(5.17±2.00) mg/L vs. (5.04±1.11) mg/L, P>0.05]. (2) The best cut-off value for the diagnosis of APS was no less than 8.37 mg/L as mean ± 3SD value, with the sensitivity at 0.772 and the specificity at 0.928. The Youden index was 0.700. These results indicated good validity of s-Eng as a diagnostic marker for APS. (3) The proportions of artery thrombosis and pathological pregnancy were higher in the group of s-Eng-positive APS patients than that in s-Eng-negative group (46/81 vs. 19/58, 29/65 vs. 10/44, respectively, all P<0.05). The levels of PLT were lower in the group of s-Eng-positive APS patients (72.00×109/L vs. 119.00×109/L, P<0.001). (4) The proportions of the presence (93.83% vs. 37.93%, P<0.001) and titer (61.70 U/mL vs. 15.45 U/mL, P<0.001) of aCL were both higher in the group of s-Eng-positive APS patients than in s-Eng-negative group. The proportions of the presence (61.73% vs. 43.10%, P<0.05) and titer (33.48 U/mL vs.17.40 U/mL, P<0.05) of anti-ß2-glycoprotein I antibody were both higher in the group of s-Eng-positive APS patients than in s-Eng-negative group too. CONCLUSION: s-Eng serum levels were significantly increased in the patients with APS, and it may play a role as acomplementary serological marker for the diagnosis and risk prediction of APS.

[A study on quality standard of Sambucus chinensis Lindl].

OBJECTIVE: To offer evidences for exploiting the plant resources and drafting the quality standards. METHOD: Studies of pharmacognosy. RESULT: The medicinal properties and the microscopic characteristics of the stem, leaves, rhizome and powder were described, drawn and physicochemically identified. CONCLUSION: A convenient and effective method for identifying the herb has been established.

[Content and activity of the focal adhesion kinase in cultured vascular smooth muscle cells enhanced by urotensin II].

Urotensin II (U II) is the most potent vasoconstrictor identified in vivo, which plays an important role in the smooth muscle cell proliferation in atherosclerosis. All available information suggests that focal adhesion kinase (FAK) is at the crossroads of multiple signaling pathways and is essential for cell proliferation. But the effect of U II on the FAK mediated signal transduction pathway is unclear. In this study, FAK content and tyrosine phosphorylation were assessed by Western blot and immunoprecipitation in cultured rat vascular smooth muscle cells. Increased protein tyrosine phosphorylation was observed within 5 min of U II 10(-7) (mol/L) addition and was maximal by 30 min, while FAK protein content showed no change during the first 30 min but it increased at 2 h reaching a plateau by 4 h, and decreased after 6 h. In addition, the elevated phosphorylation of FAK was detected upon U II stimulation at 10(-8) mol/L, being maximal at 10(-7) mol/L, but decreased at 10(-6) mol/L. Treatment of the cells with cytochalasin B (50 micromol/L), which disrupted the organization of cytoskeleton, had no influence on the increased FAK tyrosine phosphorylation in response to U sti II mulation. In order to study the relationship between FAK and mitogen-activated protein kinase, calmodulin and protein kinase C, selective inhibitors PD98059 (50 micromol/L), W7 (50 micromol/L) and H7 (50 micromol/L) were added following U II treatment. Neither PD98059 nor W7 influenced the increased FAK tyrosine phosphorylation, but H7 further increased it. These findings indicate that FAK activation is independent of the integrity of cytoskeleton and closely related to protein kinase C, but had no relation with mitogen activated protein kinase and calmodulin.