Disease relevance of Collagen Diseases

• In 62 patients with collagen disease (25 with progressive systemic sclerosis, 19 with systemic lupus erythematosus, 15 with rheumatoid arthritis, three with dermatomyositis) and in 40 healthy subjects an echocardiographic study was performed [1].
• METHODS: One patient with primary pulmonary hypertension and another with secondary pulmonary hypertension caused by collagen disease were given 50 mg oral sildenafil during cardiac catheterization for assessment of the acute hemodynamic effects of the drug [2].
• These patients, whose neurological manifestations were indistinguishable from multiple sclerosis, were thus considered to have systemic lupus erythematosus related collagen disease [3].
• Fish oil supplementation has a beneficial effect on murine models of SLE, while exogenous copper can decrease the formation of lupus erythematosus cells in rats with a hydralazine-induced collagen disease [4].
• Clinically, angiitis and Raynaud's phenomenon associated with collagen disease such as systemic lupus erythematosus were remarkably reduced following a series of ARES treatments [5].

High impact information on Collagen Diseases

• Clinical and serologic assessment excluded other causes of hepatic dysfunction, such as viral hepatitis, administration of other drugs, or worsening of the collagen disease and the patient was improved 2 wk after azathioprine was discontinued [6].
• On the basis of these results and by analogy to certain collagen diseases such as systemic lupus erythematosus, we speculate that BS cells are deficient in the detoxification of active oxygen species [7].
• Folding abnormalities of the triple helix have been demonstrated in collagen diseases such as osteogenesis imperfecta in which the mutation leads to the substitution of a single Gly in the (Gly-X-Y)n sequence pattern by a larger residue [8].
• Plasma levels of alpha 2-plasmin inhibitor (alpha 2PI) and alpha 2-plasmin inhibitor-plasmin complex (alpha 2 PIPC) were measured by sandwich enzyme-linked immunosorbent assay, using a recently developed monoclonal anti-alpha 2PI antibody, in patients with collagen diseases [9].
• In a genome-wide screening of the MRL background genes mediating susceptibility to collagen diseases using N2 progeny mice MRL/lpr x (MRL/lpr x C3H/lpr)F1, we previously found that each collagen disease is controlled by a different set of genes [10].

Chemical compound and disease context of Collagen Diseases

• Patients with hydroxylysine-deficient collagen disease (Ehlers-Danlos type VI) have diminished lysyl hydroxylase activity [11].
• Effects of prostaglandin E1 on collagen diseases with high levels of circulating immune complexes [12].
• The physiological importance of DHEA was not clear until recent research reports showing that DHEA has beneficial effects on preventing diabetes, malignancy, inflammation, osteoporosis, and collagen disease [13].
• Immunological changes in hydroxyurea-induced collagen disease [14].
• Prostaglandin E1 (PGE1) was administered to 4 patients with collagen diseases presenting with high levels of circulating immune complexes (CIC) in sera [12].

Biological context of Collagen Diseases

• We conclude that each of four types of the lesions in MRL/lpr mice is under the control of different set of genes, suggesting the complex pathological manifestations of collagen disease result from polygenic inheritance [15].
• Plasma coagulation and fibrinolysis parameters in patients with collagen diseases, and analysis of the multimeric structure of von Willebrand factor (vWF) [16].
• UVB-induced apoptosis was observed in 4 of 11 patients with SLE (36.3%), 3 of 8 patients with CLE (37.5%) and 2 of 6 patients (33.3%) with other collagen diseases [17].

Anatomical context of Collagen Diseases

• Neutrophil function in systemic lupus erythematosus and other collagen diseases [18].
• The studies suggest the existence in cultured cells of a compensatory mechanism for overproduction of these crucial enzymes in collagen biosynthesis, a mechanism which remains functional in cells derived from patients afflicted with hydroxylysine-deficient collagen disease [19].
• A 53-year-old woman whose history was noncontributory for rheumatoid arthritis and collagen disease had bilateral necrotizing inflammation of the sclera [20].
• In patients with tumours, decreased activity of beta-galactosidase was found in both serum and cerebrospinal fluid, and in patients with multiple sclerosis and collagen diseases, decreased activities of beta-galactosidase and N-acetyl-beta-glucosaminidase were found in cerebrospinal fluid [21].
• Renograms were 100% sensitive for the detection of renal disease in patients with collagen disease, and their specificity was 53% when serum blood urea nitrogen, serum creatinine concentration, and proteinuria were the only measures of renal impairment considered [22].

Gene context of Collagen Diseases

• Two COL1A1 and two COL1A2 RFLPs were more polymorphic than in the English population, making them better markers for the analysis of Italian families affected by osteogenesis imperfecta and some other inherited collagen diseases [23].
• Serum concentrations of vascular endothelial growth factor in collagen diseases [24].
• Genetic studies suggest that TBMD is a heterogeneous disease, but some cases may be related to mutations of COL4A3/COL4A4 genes, thus belonging to the spectrum of type IV collagen diseases [25].
• Serum concentrations of basic fibroblast growth factor in collagen diseases [26].
• Plasma protein S in disseminated intravascular coagulation, liver disease, collagen disease, diabetes mellitus, and under oral anticoagulant therapy [27].

Analytical, diagnostic and therapeutic context of Collagen Diseases

• Tc-99m DTPA renography in patients with collagen disease [22].
• Osteonecrosis frequently occurs in patients being treated with steroids for conditions such as systemic lupus erythematosus and other collagen diseases and in renal transplant recipients [28].
• EBV was frequently detected in RPIP (7/9 by PCR 3/9 by ISH), but was also detected in other collagen diseases of the lungs [29].

References