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ID PMID Title PublicationDate abstract
12804996 Porphyria cutanea tarda, dermatomyositis and non-Hodgkin lymphoma in virus C infection. 2003 May Virus C infection has been associated with a broad spectrum of extrahepatic diseases such as essential mixed cryoglobulinemia, membranous glomerulonephritis, vasculitis, rheumatoid arthritis and lupus erythematosus. The etiologic role of virus C has also been observed in some neoplasms such as non-Hodgkin's lymphoma and the monoclonal gammapathies. Many studies also support the link between this virus and porphyria cutanea tarda (PCT). Isolated cases suggest a relationship with dermatomyositis. Herein, we report the coexistence of PCT, non-Hodgkin's lymphoma and dermatomyositis in the same patient affected with virus C infection which has never previously been described.
12379158 Physico-chemical characterisation and biological evaluation of 188-Rhenium colloids for ra 2002 Oct 14 BACKGROUND: Radiosynovectomy is a type of radiotherapy used to relieve pain and inflammation from rheumatoid arthritis. In this study, 188-Rhenium (188Re) colloids were characterized by physical and biological methodologies. This was used to assess which parameters of the kit formulation would be the basis in the development of a more effective radiopharmaceutical for synovectomy. Intraarticular injection in knees of rabbits assessed cavity leakage of activity. METHODS: The physical characteristics of tin (Sn) and sulphur (S) colloids were determined to assess the formulation with suitable properties. Particles were grouped in three ranges for analyzing their distribution according to their number, volume and surface. The ideal particle size range was considered to be from 2 to 10 microns. Membrane filtration and laser diffraction characterization methodologies were used. RESULTS: While membrane filtration could give misleading data, laser diffraction proportions more reliable results. The Sn colloid showed a better distribution of particle volume and surface than S colloid, in the 2 to 10 microns range. The 188Re-Sn colloid was obtained with a radiochemical purity higher than 95% after 30 minutes of autoclaving. While Sn colloid kit stability was verified for 60 days, the 188Re-Sn preparation was stable in the first 24 hrs. No significant intrabatch variability (n = 3) was detected. Biodistribution and scintigraphic studies in rabbits after intraarticular injection showed relevant activity only in knee, being 90% at 48 hours. CONCLUSION: The 188Re-Sn colloid is easy to prepare, is stable for 24 hours and shows minimal cavity leakage after intraarticular injection into rabbit knees, suggesting this radiotherapeutical agent has suitable physical properties for evaluation for joint treatment in humans.
24387109 The role of interleukin-1 receptor antagonist in the prevention and treatment of disease. 2003 Mar Abstract  Interleukin-1 (IL-1) and tumor necrosis factor α (TNF-α) play key proinflammatory roles in a variety of human diseases, including rheumatoid arthritis (RA). IL-1 receptor antagonist (IL-1Ra) is a naturally occurring structural variant of IL-1 that competitively inhibits receptor binding of IL-1. Four forms of IL-1Ra have been described: secretory IL-1Ra (sIL-1Ra) and three intracellular molecules (icIL-1Ra1, 2, and 3). Excess amounts of IL-1Ra are necessary to inhibit the biological effects of IL-1. The endogenous production of IL-1Ra plays an anti-inflammatory role, but the level of production of IL-1Ra in inflamed tissues may not be adequate to block IL-1 effectively. An allelic polymorphism in the IL-1Ra gene is associated with a variety of human diseases, largely of epithelial or endothelial cell origin. The disease associated allele IL1RN(*)2 may lead to a decreased production of icIL-1Ra1 by these cells, predisposing the patient to an imbalance in the IL-1 system. The therapeutic administration of IL-1Ra was found to be safe and efficacious in the treatment of RA. Intraarticular delivery of the IL-1Ra cDNA by ex vivo gene therapy in patients with RA was effective in enhancing local IL-1Ra production. This unique form of therapy is under further evaluation.
12097418 Persistent mitochondrial hyperpolarization, increased reactive oxygen intermediate product 2002 Jul 15 Abnormal death signaling in lymphocytes of systemic lupus erythematosus (SLE) patients has been associated with elevation of the mitochondrial transmembrane potential (Delta psi(m)) and increased production of reactive oxygen intermediates (ROI). The resultant ATP depletion sensitizes T cells for necrosis that may significantly contribute to inflammation in patients with SLE. In the present study, the role of mitochondrial signal processing in T cell activation was investigated. CD3/CD28 costimulation of PBL elicited transient mitochondrial hyperpolarization and intracellular pH (pH(i)) elevation, followed by increased ROI production. Baseline Delta psi(m), ROI production, and pH(i) were elevated, while T cell activation-induced changes were blunted in 15 patients with SLE in comparison with 10 healthy donors and 10 rheumatoid arthritis patients. Similar to CD3/CD28 costimulation, treatment of control PBL with IL-3, IL-10, TGF-beta(1), and IFN-gamma led to transient Delta psi(m) elevation. IL-10 had diametrically opposing effects on mitochondrial signaling in lupus and control donors. Unlike healthy or rheumatoid arthritis PBL, cells of lupus patients were resistant to IL-10-induced mitochondrial hyperpolarization. By contrast, IL-10 enhanced ROI production and cell death in lupus PBL without affecting ROI levels and survival of control PBL. Ab-mediated IL-10 blockade or stimulation with antagonistic lymphokine IL-12 normalized baseline and CD3/CD28-induced changes in ROI production and pH(i) with no impact on Delta psi(m) of lupus PBL. The results suggest that mitochondrial hyperpolarization, increased ROI production, and cytoplasmic alkalinization play crucial roles in altered IL-10 responsiveness in SLE.
15590857 Triple arthrodesis in adults with non-paralytic disease. A minimum ten-year follow-up stud 2004 Dec BACKGROUND: The triple arthrodesis was developed to treat sequelae of neurologic disorders affecting the hindfoot. Today, the typical adult patient undergoing this procedure has degenerative disease, usually not related to a neurologic disorder. The purpose of this study was to investigate the long-term outcome of triple arthrodesis in this patient population. METHODS: Twenty-seven adult patients (thirty-one feet) who had undergone triple arthrodesis for the treatment of chronic hindfoot pain and had been followed for a minimum of ten years completed an outcomes questionnaire, and twenty-two patients (twenty-six feet) were available for physical examination, radiographs, and functional testing. The mean age of the patients who were examined was forty-five years at the time of the surgery, and the mean duration of follow-up of those patients was fourteen years (range, eleven to eighteen years). RESULTS: Twenty-five (93%) of the patients were satisfied with the result of the treatment. However, only eleven (41%) reported that they could perform moderate activity with mild or no pain in the foot and ankle. Twenty patients (74%) reported moderate-to-severe difficulty with, or an inability to negotiate, uneven surfaces. The mean Short Form-36 (SF-36) physical component outcomes score was 35.2 points, well below the mean of 50 points for the United States population. The SF-36 score was significantly lower for patients with systemic inflammatory disease (primarily rheumatoid arthritis). There was an average 12 degrees (27%) loss of plantar flexion but no significant loss of dorsiflexion compared with the untreated foot. Severe arthrosis developed in seven of the twenty-six ankles, in seven naviculocuneiform joints, and in six tarsometatarsal joints. Some patients had severe arthrosis at more than one level, and three patients later required an ankle arthrodesis. There were no nonunions or revisions of the triple arthrodeses. The average patient performances on the six-minute walk and the 3-m up-and-go functional tests were well below the age-controlled means. CONCLUSIONS: Triple arthrodesis may provide patients with substantial long-term relief of preoperative symptoms. However, there may also be adverse consequences, particularly degenerative changes in adjacent joints, that may be reasons for orthopaedic surgeons to consider alternatives to triple arthrodesis when feasible. LEVEL OF EVIDENCE: Therapeutic study, Level IV (case series [no, or historical, control group]). See Instructions to Authors for a complete description of levels of evidence.
15368133 [Urinary retention due to acquired closure of labia minor in a female with Sjögren syndro 2004 Sep A case of a 70 year old woman with acute urinary retention and primary Sjögren-Syndrome is presented. On clinical examination a total closure of labia minor was diagnosed. Surgical detachment of labia minor was performed and full recovery was achieved.
12766055 Corneal innervation and morphology in primary Sjögren's syndrome. 2003 Jun PURPOSE: To analyze the in vivo morphology of the different corneal sublayers and corneal nerves in primary Sjögren's syndrome (SS). METHODS: Ten eyes of 10 patients with primary SS and 10 eyes of 10 sex- and age-matched control subjects were investigated. Diagnosis was based on American-European consensus criteria. In vivo confocal microscopy with through-focusing was used to investigate corneal morphology and to measure corneal sublayer thickness. RESULTS: Epithelial punctate staining with fluorescein was observed in 6 of 10 SS and none of 10 control corneas. In addition, Schirmer I test results were significantly lower in SS. Epithelial thickness did not differ between the SS and control groups. Confocal microscopy revealed patchy alterations or irregularities in surface epithelial cells in 6 of 10 SS corneas, whereas the basal epithelium appeared normal in all corneas. Average corneal thickness was lower in the SS group (515.9 +/- 22.0 micro m) than in the control (547.4 +/- 42.0 micro m; P = 0.050, t-test). Accordingly, the mean intraocular pressure was lower in the SS group (13.9 +/- 2.1 mm Hg) than in the control (16.7 +/- 2.9 mm Hg; P = 0.022). The subbasal nerve plexus and stromal nerve fiber bundles were present in all corneas. No difference was noted in nerve density. However, in 4 of 10 SS eyes, the subbasal nerve plexus showed structures resembling nerve sprouting, suggesting ongoing active neural growth. None of the control corneas exhibited such features. Signs of anterior keratocyte activation were observed in 5 of 10 SS corneas. CONCLUSIONS: In SS, the corneal surface epithelium was irregular and patchy. Anterior keratocytes frequently showed morphologic features of activation. The subbasal nerve fiber bundles revealed abnormal morphology, and the central corneal thickness was reduced by stromal thinning. The findings confirm epithelial, stromal, and neural abnormalities in the corneas of patients with SS.
12586180 Efficacy of tear eosinophil cationic protein level measurement using filter paper for diag 2003 Jan PURPOSE: We investigated the efficacy of the tear sampling method using a filter paper to evaluate eosinophil cationic protein (ECP) levels in patients with allergic disorders. METHODS: Subjects were an allergic group comprising patients with allergic conjunctivitis, vernal keratoconjunctivitis, or atopic keratoconjunctivitis, a Sjögren group comprising patients with secondary Sjögren syndrome, and a control group comprising healthy volunteers. Tears were sampled using the Schirmer Method I and the sample was eluted from the filter paper in 50 microL of elution solution containing phosphate buffer solution with 0.5 M NaCl + 0.1% Tween 20. Then the ECP concentration in the elution sample was determined by the enzyme-linked immunosorbent assay method. RESULTS: Tear ECP level in the allergic group was significantly higher than the levels in the other two groups (P <.01 for the Sjögren group and P <.001 for the control). Furthermore, the tear ECP level of each allergic disease subgroup in the allergic group was significantly higher than that in the control group. CONCLUSIONS: This method of determining tear ECP concentration is useful not only to diagnose allergic conjunctival disorders but also to evaluate their clinical stages.
12233074 [Vasculitis and collagenous-vascular involvement in association with sarcoidosis]. 2002 Sep Sarcoidosis is a systemic granulomatous disorder of unknown origin. It unusually complicates systemic vascular involvement. We concisely review clinico-pathological findings of vascular involvement, including granulomatous angitis and microangiopathy. The clinical features of sarcoidosis may mimic those of many rheumatic disorders and sarcoidosis may coexist with autoimmune diseases. We review both rheumatologic manifestations, including bone, joint, and muscle of sarcoidosis and immunological findings of autoimmune diseases complicated with sarcoidosis, including 2-case reports of Sjögren's syndrome and dermatomyositis/polymyositis.
12170307 Chronic infection with hepatitis and herpes viruses in patients with Sjogren's disease. 2002 Jan The prevalence of hepatitis B, C, E, and G viruses, Epstein-Barr virus, and type 6 herpesvirus was studied in Russian and Norwegian patients with Sjogren's disease. The incidence of HBV, HCV, HEV, and HGV markers in Russian patients was higher than in donors. The incidence of serological markers of Epstein-Barr and type 6 herpesvirus was virtually the same in the patients with Sjogren's disease and donors. Epstein-Barr virus DNA was less frequently detected in patients with Sjogren's disease than in donors, as was shown by blood and salivary DNA testing.
11972318 [Transthoracic pacing in a very low birth weight infant with congenital complete atriovent 2002 Mar We report our experience of pacemaker treatment in a premature infant of 832 grams with congenital complete atrioventricular block due to maternal Sjögren's Syndrome. She was delivered by cesarean section at an estimated gestational age of 26 weeks because of fetal bradycardia, decreasing fetal movements and hydrops. Immediate postnatal transesophageal ventricular pacing was not successful, whereas transthoracic pacing with self-adhesive patch electrodes adapted to body size resulted in an effective increase of the infant's heart rate until operative application of temporary epimyocardial pacing wires ensured the external stimulation of the heart.
15556589 Sonographic assessment of the submandibular space. 2004 Dec There is a wide variety of pathological processes which may present with swelling in the submandibular space. Although the submandibular gland is the most important structure in this region, there are a number of extraglandular causes of swelling which frequently mimic submandibular gland enlargement. In this review the use of high-resolution ultrasound in the assessment of the submandibular gland and adjacent structures is discussed and illustrated.
15523370 Severe sialadenitis: a new complication of drug reaction with eosinophilia and systemic sy 2004 Nov BACKGROUND: Drug cutaneous reaction and isolated cases of parotitis induced by terbinafine have been reported. OBSERVATION: We report a case of drug reaction with eosinophilia and systemic symptoms induced by terbinafine associated with a severe sialadenitis and a complete sicca syndrome. Evolution was protracted with a slow recovery of the rash but sicca syndrome persisted with only a very mild improvement at 6 months. CONCLUSION: Liver, kidneys, lungs, and heart are the organs the most frequently involved in drug reaction with eosinophilia and systemic symptoms. Salivary and lacrimal glands can also be severely involved in the course of drug reaction with eosinophilia and systemic symptoms.
15361944 Primary Sjögren's syndrome complicated with cryoglobulinemic glomerulonephritis, myocardi 2004 Sep Glomerulonephritis in primary Sjögren's syndrome is rarely reported. Cryoglobulinemic glomerulonephritis with the presence of cryoglobulin deposition in the glomerular capillary lumen in primary Sjögren's syndrome is extremely rare. A 51-year-old woman with primary Sjögren's syndrome for > 10 years complained of fever, hypertension, and proteinuria. In addition, novel manifestations, including myocarditis with heart failure, pericardial effusion, and polyneuropathy (sensory motor neuropathy) were also noted. Cryoglobulinemia test was positive, and kidney biopsy results were consistent with cryoglobulinemic glomerulonephritis. There were no symptoms associated with systemic lupus erythematosus or other connective tissue disease. Treatment with monthly methylprednisolone and cyclophosphamide pulse therapy for 6 months resulted in resolution of proteinuria, heart failure, and neurologic symptoms.
15285530 Coeliac disease associated with Sjögren's syndrome, renal tubular acidosis, primary bilia 2004 Jul Although coeliac disease may occur in patients affected by another immune-mediated disorder, its coexistence with multiple autoimmune diseases is not frequently described. We report here the case of a 45-year-old woman referred to our centre because of diarrhoea and weight loss, who had already received a diagnosis of primary biliary cirrhosis, Sjögren's syndrome and renal tubular acidosis. Following the development of diarrhoea we established the diagnosis of coeliac disease, based on the presence of anti-endomysium antibodies and a compatible duodenal biopsy. Despite gluten withdrawal she went on to develop an autoimmune hyperthyroidism. The patient tested positive for HLA DRB1*03 and DQB1*02. The association is unlikely to be casual and may be explained by autoimmune mechanisms, genetic susceptibility and favouring environmental factors commonly shared by the diseases of our patient.
15121246 Grey-scale and power Doppler sonography of unusual cervical lymphadenopathy. 2004 Apr This study was undertaken to document the grey-scale and power Doppler sonographic features of cervical lymphadenopathy in Kikuchi's disease (histiocytic necrotising lymphadenitis), Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy), Sjogren's syndrome and systemic lupus erythematosus (SLE), which have not been reported in the literature. A retrospective review of the grey-scale and power Doppler sonograms of the cervical lymph nodes in nine patients was conducted (Kikuchi's disease, n = 3; Rosai-Dorfman disease, n = 1; Sjogren's syndrome, n = 1; SLE, n = 4). Lymph nodes were proven to be pathologic by fine-needle aspiration cytology (FNAC). On grey-scale ultrasound (US), lymph nodes were assessed by their distribution, size, shape, echogenicity and internal architecture. The vascular pattern of the lymph nodes was assessed with power Doppler sonography. US features of the lymph nodes were compared to those of metastatic and reactive nodes. In Kikuchi's disease, Rosai-Dorfman disease, Sjogren's syndrome and SLE, the distribution of lymph nodes is similar to that of reactive nodes. Most of the lymph nodes are enlarged with a maximum transverse diameter greater than or equal to 10 mm (83.3 to 100%). In Kikuchi's disease, lymph nodes have grey-scale and Doppler appearances similar to reactive nodes. However, lymph nodes in Rosai-Dorfman disease, Sjogren's syndrome and SLE show similar grey-scale and Doppler features to metastatic nodes. There is no specific US feature to characterise lymphadenopathy from these four miscellaneous causes. Definitive diagnosis should still be based on cytology and histology, and US can help in guiding FNAC for a more accurate cytologic examination.
14644860 Oral pilocarpine for the treatment of ocular symptoms in patients with Sjögren's syndrome 2003 Dec OBJECTIVE: To evaluate the efficacy and side effects of oral pilocarpine for the treatment of ocular symptoms in patients with primary Sjögren's syndrome (SS). METHODS: A 12 week, single centre, randomised controlled study was performed. Twenty nine patients were randomly assigned to receive oral pilocarpine (5 mg twice a day), 28 only artificial tears, and 28 inferior puncta occlusion. Patients receiving oral pilocarpine and those with inferior puncta occlusion also received artificial tears. Patients were evaluated at baseline and throughout the study for their subjective global assessment of dry eyes and for their objective assessment of dry eyes (Schirmer's-I test, rose bengal test, and imprint test). RESULTS: Patients taking oral pilocarpine had significant improvement in subjective global assessment of dry eyes, as was evaluated by improvement of >55 mm on a visual analogue scale (VAS) for responses to the eye questionnaire, compared with patients treated with artificial tears (p<0.001) and those with inferior puncta occlusion (p<0.05). Furthermore, patients receiving oral pilocarpine also showed greater objective improvement, as measured by the rose bengal test (p<0.05), while Schirmer's-I test showed no differences between the treated groups. Commonly reported adverse events were headache, increased sweating, nausea, and vomiting in the pilocarpine group, while one patient in the inferior puncta occlusion group had blepharitis and was withdrawn from the study. CONCLUSION: 10 mg of pilocarpine daily given to patients with SS for 12 weeks had a beneficial effect on subjective eye symptoms, as evaluated by improvement >55 mm on a VAS. Additionally, an improvement of rose bengal staining was noted, but an increase in tear production, as measured by the Schirmer-I test, was not substantiated.
11811741 The impact of gene therapy on dentistry: a revisiting after six years. 2002 Jan BACKGROUND: Gene therapy is an emerging field of biomedicine that has commanded considerable scientific and popular attention. The procedure involves the transfer of genes to patients for clinical benefit. Transferred genes can b e used for either reparative or pharmacological purposes. OVERVIEW: In 1995, the first author and a colleague described the potential impact of gene therapy on dentistry, on the basis of initial studies of gene transfer applications to salivary glands, keratinocytes and cancer cells. Their conclusion was that gene therapy would have a significant impact on the nature of dental practice within 20 years. In this article, the authors consider research progress since 1995 and reexamine the earlier conclusion. PRACTICE IMPLICATIONS: In the past six years, remarkable progress has been made in the field of gene therapy, including seven areas relevant to dental practice: bone repair, salivary glands, autoimmune disease, pain, DNA vaccinations, keratinocytes and cancer. While considerable problems remain, thus impeding the routine clinical use of gene transfer, gene therapy will have a pervasive and significant impact on areas of dental practice that are based in biological science. By 2015, this will translate into practitioners' having a wide range of novel biological treatment options for their patients.
15368127 Distribution of CXCR3- or CCR4-positive cells in interstitial pneumonia associated with pr 2004 Nov Patients with primary Sjogren's syndrome (SS) occasionally develop interstitial pneumonia (SS-IP), the prognosis of which is less grave compared with that of idiopathic pulmonary fibrosis (IPF). We examined distribution of helper T-cell subsets in open lung biopsy specimens from seven patients with SS-IP and, for comparison, ten patients with IPF. The expression of CXCR3 and CCR4, chemokine receptors associated in vitro with Th1 and Th2 cells, respectively, was analyzed in the mononuclear infiltrate using immunohistochemistry. The expression of CD4, CD8, and CD20 in the infiltrate was similarly examined. The positive cells were semiquantified in fibrosing areas and lymphoid clusters of both SS-IP and IPF. In fibrosing areas, CXCR3-positive cells were dominant over CCR4-positive cells in all cases of SS-IP, whereas the two types of cells showed no such difference in cases of IPF. Each of the CXCR3/CD4 and CXCR3/CCR4 ratios was significantly higher in SS-IP than in IPF ( P<0.05 and P<0.05, respectively). The CCR4/CD4 ratio showed a significantly lower value in SS-IP than in IPF ( P<0.05). In lymphoid clusters, prominent in SS-IP and few and small in IPF, CXCR3-positive cells predominated over CCR4-positive cells in both lung lesions. There was no significant difference of CXCR3/CCR4 ratio in lymphoid clusters between SS-IP and IPF ( P=0.33). These findings in SS-IP are in accordance with those reported in previous studies of the salivary glands of SS patients, where most of the infiltrating lymphocytes expressed CXCR3, and the expression of interferon-gamma was upregulated. In contrast, the Th2 cell dominance was reported in IPF in the previous studies. The present findings suggest that the pathogenesis of interstitial pneumonia is different between SS-IP and IPF in regard to the roles of helper T-cell subsets.
12605324 Antitopoisomerase I antibody in patients with systemic lupus erythematosus/sicca syndrome 2003 Feb We describe two female patients with classic systemic lupus erythematosus (SLE) and secondary sicca syndrome associated with topoisomerase I (topo-I, Scl-70) antibody, a specific marker for scleroderma (SSc), which is rarely found in other collagen diseases. During the course of the disease, the sera of these two patients were repeatedly found to be positive for topo-I antibody following a positive screening by ANA-EIA. Neither patient had clinical evidence of scleroderma. One patient remains well nearly 4 years from the first positive serological test. The progression to sicca syndrome in that patient occurred 2 years after having tested positive for antitopo-I antibody. Her frozen serum also tested positive for anti-Scl-70 by the Western blot technique. The other patient, however, died after developing renal and cardiopulmonary complications of lupus, including Libman Sachs endocarditis and pulmonary hypertension. Contrary to the previous patient, the onset of sicca syndrome in this case had preceded the expression of positive antitopo-I antibody. The present cases and other similar previously reported ones are therefore unique in the sense of being a serological challenge to the high specificity of antitopo-I to scleroderma. In addition, they may also represent a new subset of SLE with or without sicca syndrome, which is characterised by the absence of features of scleroderma despite the presence of antitopo-I antibody.