Browse

Search for: rheumatoid arthritis    methotrexate    autoimmune disease    biomarker    gene expression    GWAS    HLA genes    non-HLA genes   

ID PMID Title PublicationDate abstract
10830178 Diffuse angiodysplasia of the upper gastrointestinal tract in a patient with hypertrophic 2000 May A 64-year-old woman with a known history of hypertrophic obstructive cardiomyopathy presented with severe anemia of unknown origin. She had also suffered from repeated episodes of upper gastrointestinal bleeding for the previous 3 years. Despite bone marrow examination and panendoscopic and angiographic studies, the origin of anemia remained undefined until a small bleeding site was found during a duodenoscopic examination. The lesion proved to be angiodysplasia. This case report is interesting in that angiodysplasia elicited gastrointestinal bleeding and was the cause of anemia. In the international literature, there are very few reported cases of bleeding from gastrointestinal angiodysplasia in association with subvalvular aortic obstruction.
10453537 [Evaluation of parotid sialography and labial salivary gland biopsy to the diagnosis of Sj 1997 Dec OBJECTIVE: To investigate the relationship between the change of parotid sialograms and the results of labial gland biopsies in the patients with Sjögren syndrome (SS). METHODS: Parotid sialography and labial salivary gland biopsy were performed on 100 patients with Sjögren syndrome. RESULTS: The sialectasis of parotid terminal ducts was found in 87 patients, the widening only of main ducts was found in 8 patients, and 5 cases showed normal sialograms. The results of labial salivary gland biopsies showed, that 90 cases had focal lymphocytic sialadenitis (Zhen Linfan's grade score of 2), 59 cases had a score of 4 accoding to Chisholm's grading score; moderate scattered lymphocitic infiltration was shown in 10 patients. CONCLUSION: Our results suggested that the lesions of parotid gland and labial salivary gland in SS were concerted closely. Therefore a new way of combining the changes of parotid gland and labial salivary gland for the diagnose of SS was proposed.
10380841 Reduced 25-hydroxyvitamin D levels in primary Sjögren's syndrome. Correlations to disease 1999 The purpose of this study was to explore the clinical and pathogenic significance of vitamin D metabolites in primary Sjögren's syndrome (primary SS). We measured blood concentrations of 25-hydroxyvitamin D (25 OH D) and calcitriol (1,25(OH)2D)vc in 41 patients and correlated the results with blood levels of various immune activation products, as well as with patients' clinical status. Levels of 25 OH D were slightly decreased as compared to normal controls and the reduced levels of 25 OH D were stable over the observed period of 2 years. Levels of 25 OH D correlated inversely with levels of soluble interleukin-2 receptor, status indices for global disease, total exocrine disease, surface exocrine disease, internal organ exocrine disease, and mediator-induced disease. Levels of 1,25(OH)2D varied considerably and compared to normal control values. Levels of 1,25(OH)2D did not correlate with clinical/immunopathological status. In conclusion the inverse correlations found between levels of 25 OH D and measures of clinical and immunoinflammatory status support the notion that vitamin D metabolism may be involved in the pathogenesis of primary SS.
9858434 Autonomic cardiovascular neuropathy in Sjögren's syndrome. A controlled study. 1998 Dec OBJECTIVE: To test patients with primary Sjögren's syndrome (SS) for evidence of autonomic neuropathy. METHODS: Thirty-two patients with primary SS and 22 age and sex matched healthy individuals were asked specific questions about symptoms suggestive of autonomic neuropathy, and were subjected to a battery of 5 cardiovascular tests: response of blood pressure to sustained hand grip, Valsalva maneuver, heart rate response to deep breathing, and heart rate and blood pressure response to standing up. The chi-squared test with Yates' correction and 95% confidence intervals were used for statistical analysis of the results. RESULTS: Sixteen patients (50%) had symptoms of autonomic neuropathy when specifically asked versus none of the controls (p < 0.0005). The frequency of abnormal responses to the tests was 68.8% in patients and 12.7% in controls (p < 0.0001). Severe autonomic cardiovascular neuropathy was found in 87.5% of the patients but in none of the healthy individuals (p < 0.0001). CONCLUSION: Our results suggest that autonomic neuropathy is a feature of a significant portion of the SS population, and such patients should have appropriate evaluation. Similarly, patients with unexplained autonomic neuropathy should be investigated for evidence of SS.
9785986 [Influence on patients with Sjögren's syndrome after the Great Hanshin-Awaji Earthquake]. 1998 Aug We investigated the influence of clinical findings on patients with Sjögren's syndrome after the Great Hanshin-Awaji Earthquake. Fifty eight (90%) of 64 patients were struck severely by the earthquake and 26 patients (42%) were forced to take refuge in emergency shelters. There was no aggravation of clinical findings or complications in eight patients inhabiting the area where the damage was mild. Dry eyes were deteriorated in 14 patients (22%). Dry mouth was aggravated in 6 patients (9%). However, the mean values of Schirmer's test and gum test were not different between pre-earthquake and postearthquake in these patients. Malaise was worsen in 8 patients (13%). The cause was cease of taking prednisolone for autoimmune hepatitis in 2 patients or was impossibility of taking T4 drug for hypothyroidism in 2 patients. Other 4 patients had not taken any medication. Arthralgia was deteriorated in 4 patients (5%). These findings indicated that deterioration of sicca symptoms, arthralgia and malaise was caused predominantly by the worsen living condition including shortage of water and polluted atmosphere in the affected regions.
9715014 Using ultrasonography to diagnose Sjögren's syndrome. 1998 Aug Ultrasonographic imaging of the parotid gland parenchyma represents a relatively new and extremely useful procedure that was applied in this study of patients with secondary Sjögren's syndrome, or SS. A nonhomogeneous parotid gland and hypoechoic areas were apparent in the sonograms of nine consecutive SS patients who were examined using this simple, noninvasive technique.
9084205 Reliability of ultrasonography and sialography in the diagnosis of Sjögren's syndrome. 1997 Mar OBJECTIVE: The purpose of this study was to evaluate quantitatively observer performance with ultrasonography and sialography in the diagnosis of parotid gland involvement of Sjögren's syndrome. STUDY DESIGN: Sixty-four parotid gland sialograms and 65 ultrasonograms were prepared for observer performance experiments. They included both modalities in 24 Sjögren's syndrome and 19 nonspecific parotitis cases, 21 normal parotid gland sialograms, and 22 normal ultrasonograms in healthy volunteers. The images were randomly sequenced and presented to five observers who were asked to describe several findings and finally to determine the imaging diagnosis by ranking the abnormal features and the diagnosis on a five-point-rating scale. Observer performance was evaluated on the basis of the reliability of findings interpreted and the diagnostic accuracy of each modality from observers' rating scores. RESULTS: The diagnostic accuracy of sialography was very high, nearly perfect. The diagnostic accuracy of ultrasonography was lower than that of sialography, resulting from the lower incidence of characteristic findings in the disease groups and lower sensitivity on ultrasonography. In the differentiation of Sjögren's syndrome from the normal, however, the diagnostic accuracy of ultrasonography increased to 80% for all cases, and up to nearly 90% in the advanced sialographic stages. CONCLUSION: Ultrasonography is useful for the diagnosis of Sjögren's syndrome in the advanced stages. Taking the noninvasiveness of this technique into account we recommend first applying ultrasonography to the diagnosis of Sjögren's syndrome and performing sialography when no positive findings are detected on ultrasonography.
11569607 Transferring genes to salivary glands. 2001 Sep This review provides a brief description of gene transfer studies using salivary glands as the target tissue. The aggregate results demonstrate the potential clinical value of this methodological approach for managing several conditions lacking fully satisfactory conventional treatments. Routine clinical applications are still seven to ten years away, primarily because of the need for improved gene transfer vectors. Overall, this body of work provides the dental educator with a substantive example of how biotechnological progress will significantly affect the treatment of oral problems in the near future.
11159485 Modification of the tear function index and its use in the diagnosis of Sjögren's syndrom 2001 Feb BACKGROUND: The tear function index (TFI) has been shown to be of value in the diagnosis of patients suffering from Sjögren's syndrome. It is dependent, however, on introducing into the conjunctival fornix the correct concentration of fluorescein in at least one and a half times the normal tear volume. The stimulus and effect of this added volume on the tear dynamics is likely to vary between individuals. These factors, together with the method of performing the test, limit its general applicability. AIM: To devise a method of performing the TFI with less variability and more general applicability. To present a theoretical and in vitro assessment of the dynamics of the TFI. METHOD: The study was divided into three parts. The first part was to compare the results obtained using a prepared strip containing 1.3 microl of 0.5% fluorescein with the introduction of the same amount of fluorescein as a drop. The second part was to compare the results obtained with prepared strips with the standard method of performing the TFI, both with and without topical anaesthetic. The third part was an in vitro study of the rate of flow of graded volumes on a filter paper strip. 42 subjects with a diagnosis of Sjögren's syndrome according to the European criteria and 126 without Sjögren's syndrome were included. RESULTS: There was no significant difference between the results obtained with a prepared strip and the introduction of 1.3 microl into the eye before performing the Schirmer's test and TFI (0.1
10828873 GVHD dry eyes treated with autologous serum tears. 2000 May Two cases of GVHD with severe dry eyes are reported where conventional therapy failed to control ocular signs and symptoms. Autologous serum tears, however, resulted in a beneficial clinical effect with marked attenuation of the symptoms. This therapy proved to be safe during 10 months of treatment. Bone Marrow Transplantation (2000).
10784516 Percentage of glycosylated serum ferritin remains low throughout the course of adult onset 2000 May OBJECTIVE: To determine the evolution of levels of total serum ferritin and percentage of the glycosylated form in patients with adult onset Still's disease (AOSD) at the time of diagnosis and during follow up. METHODS: All patients with AOSD were tested at the time of diagnosis and during follow up. Total serum ferritin levels were analysed by immunoassay, and the percentage of glycosylated ferritin was determined by methods using Sepharose-Con A. RESULTS: 14 patients (eight women, six men) with AOSD were enrolled. At the time of diagnosis, mean (SD) age was 36 (16) years. Mean initial total serum ferritin was 6350 (1300) microg/l (normal <250 microg/l). The mean initial percentage of glycosylated ferritin was 14.7 (13)% (normal >50%). Mean follow up time was 37 (35) months. At the time of the last examination all patients were in remission except one, who presented a chronic articular form. Total serum ferritin remained high in this single patient and was normal in the 13 others, with a mean of 98 (73) microg/l. In all patients the percentage of glycosylated ferritin remained low, with a mean of 16 (16)%. CONCLUSION: Total serum ferritin is a marker of the active phase of AOSD. The percentage of glycosylated ferritin is low both in the active phase and in remission. Further studies are needed to confirm these data and to determine their specificity for AOSD before considering any possible use of a low percentage of glycosylated ferritin as a diagnostic tool in suspected AOSD, especially when atypical or previously treated.
10748979 Neonatal lupus erythematosus: our local experience. 2000 Jan INTRODUCTION: Neonatal lupus erythematosus (NLE) presents clinically with either cutaneous lesions or cardiac involvement and is related to the transplacental passage of anti-Ro antibodies from mother to foetus. We report a series of 10 cases seen at the National Skin Centre between 1990 and 1998 including a pair of siblings. CLINICAL PICTURE: The female to male ratio was 2.3:1 and most presented with rash between 3 to 5 weeks of life while 4 had lesions at birth. Four of the 9 mothers had SLE, one was diagnosed to have a lupus-like illness, 2 had Sjogren's syndrome and the remaining two had very high ANA titres but the final diagnosis was uncertain. OUTCOME: The rash resolved spontaneously by 3 to 6 months of age.
10399792 Sicca syndrome in patients with sarcoidosis. 1999 Out of 134, 12 sarcoidosis patients with symptoms of mucosal dryness as the first clinical manifestation were identified and compared with 30 consecutive unselected Sjögren's syndrome (SS) patients. Sicca manifestations were similar among the two groups, while parotid gland enlargement (PGE) was more frequently found in sarcoidosis patients (P < 0.05). Patients with sarcoidosis had mainly pulmonary (P < 0.001) and skin involvement (P < 0.05), while SS patients presented more frequently with Raynaud's phenomenon (P < 0.05). Autoantibody profile was more often found in SS patients compared to sarcoidosis (P < 0.0025). The histopathological findings of minor salivary gland biopsy (MSGB) revealed noncaseating granulomas (NCG) in 58% of patients with sarcoidosis, while in SS, MSGB showed focal sialadenitis in the majority of the patients. Transbronchial lung biopsy (TBLB), which was performed in 10 sarcoidosis patients, revealed the presence of NCG in all patients. In patients with sarcoidosis and sicca symptoms as the presenting syndrome, PGE is a useful clinical finding. Searching for pulmonary involvement is a determining factor to differentiate sarcoidosis from SS. The absence of autoantibodies is another useful tool for the diagnosis of sarcoidosis. Finally, MSGB is very helpful to discriminate between sarcoidosis and SS and when MSGB is not specific, then TBLB is valuable to confirm the diagnosis.
9631752 Intravenous immunoglobulin in adult Still's disease refractory to non-steroidal anti-infla 1998 May OBJECTIVE: Adult onset Still's disease (AOSD) is a systemic disorder with an uncertain outcome at the time of diagnosis. The clinical response to nonsteroidal antiinflammatory drugs (NSAIDs) is often unsatisfactory in adult patients. Chronic use of steroids is frequently required, but may result in severe side effects. We report the results of an uncontrolled, unblinded trial of intravenous immunoglobulin (IVIG) in 7 patients with AOSD. METHODS: Seven consecutive patients unresponsive or poorly responsive to NSAIDs were enrolled. None of them had received steroids previously. AOSD was defined by the Yamaguchi criteria (J Rheumatol 1992; 19: 424). IVIG were administered every 4 weeks. NSAIDs were initially maintained. A positive response was defined by the disappearance of fever and arthritis within 2 weeks after the first IVIG infusion. RESULTS: Two patients failed to respond. Five patients initially considered to be good responders were given a total of 4 to 6 IVIG infusions. One of them relapsed at the time of the fourth IVIG infusion. The four others had a favourable clinical and biological course. At the time of evaluation 3 patients were symptom-free and off therapy, while one was still receiving low dose NSAIDs. CONCLUSION: This short, uncontrolled, unblinded study suggests that IVIG might represent a potential alternative to classical steroid therapy in patients with AOSD refractory to NSAIDs. These preliminary results need to be confirmed, however, in a double-blind randomized study.
9039171 Annular erythema associated with lupus erythematosus/Sjögren's syndrome. 1997 Feb BACKGROUND: Annular-polycyclic and papulosquamous lesions are associated with subacute cutaneous lupus erythematosus (SCLE). In some Asian cases, annular erythema has been associated with Sjögren's syndrome (SS). However, the relation between the two is unclear. OBJECTIVE: Our purpose was to clarify the clinical manifestations and immunologic features of patients with annular erythema. METHODS: This study included 15 patients with annular erythema. Systemic, serologic, and genetic findings were analyzed. RESULTS: Histologic examination showed perivascular and periappendageal lymphocytic infiltrates in all patients. However, LE-specific epidermal changes were observed in only three (20%). Although all patients at least partially demonstrated features of LE or SS, eight (53%) fulfilled the American Rheumatism Association criteria for systemic LE (SLE) and five (33%) were diagnosed with SS. Renal (20%) and central nervous system (7%) involvement was observed. Anti-Ro(SS-A) and anti-La(SS-B) antibodies were detected in nine (60%) and seven (47%) patients, respectively. There were no histocompatibility antigen (HLA) haplotype differences. CONCLUSION: Annular erythema in Asian patients is the counterpart of subacute skin lesions of LE in whites, except for the paucity of LE-specific histopathologic findings and HLA-DR3 tissue type.
11053079 Tumour necrosis factors receptor associated signalling molecules and their role in activat 2000 Nov Tumour necrosis factor (TNF) is a pleiotropic cytokine that mediates apoptosis, cell proliferation, immunomodulation, inflammation, viral replication, allergy, arthritis, septic shock, insulin resistance, autoimmune diseases, and other pathological conditions. TNF transduces these cellular responses through two distinct receptors: type I, which are expressed on all cell types, and type II, which are expressed only on cells of the immune system and endothelial cells. At the cellular level, these receptors activate the pathways leading to the activation of transcription factors NF-kappaB and AP-1, apoptosis and proliferation, and mitogen activated protein kinases. None of these receptors exhibit any enzymatic activity but the signals are transmitted through the recruitment of more than a dozen different signalling proteins, which together form signalling cascades. Inhibitors of TNF signalling have therapeutic value as indicated by the approval of the soluble TNF receptors and anti-TNF antibodies for rheumatoid arthritis and for inflammatory bowl disease.
10841071 [Rationale for using nabumetone and clinical experience]. 2000 Nabumetone's position as one of the most commonly used nonsteroidal anti-inflammatory drugs (NSAIDs) in the world today is based upon over a decade of clinical experience. The popularity of this drug lies in both its unique pharmacokinetic profile and special safety features in pharmacodynamic terms. This nonacidic prodrug with an active 6-methoxy-2-naphthylacetic acid (6-MNA) metabolite has COX-2 preferential features and is also devoid of enterohepatic recirculation. It is felt that these characteristics have provided the basis for its unique long term tolerability, documented in various at-risk osteoarthritis and rheumatoid arthritis populations. The excellent tolerability of nabumetone and its 24-hour half-life, which provides the advantages of a once-daily dosage regimen, make it uniquely suitable for long term anti-inflammatory therapy in arthritis. The tolerability profile of nabumetone has also demonstrated clear cost-effectiveness advantages, as confirmed by comparative and epidemiological studies. Selective COX-2 NSAIDs are likely to prove more expensive because of the increasing costs and demands of clinical research prior to FDA approval. These higher costs may limit and influence patient access, depending on the healthcare delivery system, and many years of experience will be required to document the putative tolerability advantages of these newer COX-2 inhibitor agents. In the meantime, it is comforting that nabumetone has established such an advantageous tolerability profile together with acknowledged efficacy.
10415188 Popliteal cysts in children: prevalence, appearance and associated findings at MR imaging. 1999 Aug OBJECTIVE: The purpose of this study was to determine the prevalence of Baker's cysts on MR images in a paediatric orthopaedic population, to investigate the association of Baker's cyst with joint fluid and joint disorders in children, and to compare the MR appearance of Baker's cysts in children with that previously reported in adults. MATERIALS AND METHODS: Reports from 393 MR studies of the knee performed in children aged from 1 to 17 years were retrospectively reviewed for the presence of a Baker's cyst, joint effusion, meniscal tear, anterior cruciate ligament tear, or any other joint disorder. RESULTS: A Baker's cyst was identified in 6.3 % (25/393) of patients. The MR images and clinical charts of patients with a Baker's cyst were reviewed. None of the 25 patients with a Baker's cyst had an associated anterior cruciate ligament tear or meniscal tear. Two patients had osteochondritis dissecans and two others had synovial disease (infection and juvenile rheumatoid arthritis). Joint fluid was demonstrated in 16 % (4/25) of patients with a Baker's cyst. There was no statistically significant association between presence of a Baker's cyst and presence of joint fluid. CONCLUSIONS: Baker's cyst is less prevalent in a paediatric orthopaedic population than in an adult population. In children, it seems that Baker's cyst is seldom associated with joint fluid, meniscal tear, or anterior cruciate ligament tear. On MR images, a communication between the Baker's cyst and the joint was not demonstrated in any of the patients. In addition, the presence of debris and cyst leakage was not observed.
9709843 Ten-year follow-up of a bicondylar unlinked knee endoprosthesis with particular reference 1998 This analysis, submitted retrospectively, evaluates 603 PCA-type or Duracon-type knee endoprostheses implanted in 527 patients over a 10-year period. In addition, the X-rays available in a group of 138 patients (100 women and 38 men) from the entire patient population were analysed. All of these 138 patients were invited to come in for a follow-up examination. The invitation was accepted by 80 patients. As well as radiological and physical examination, these patients were asked about their subjective physical condition in a semi-open questionnaire. The data obtained by the questionnaire is discussed in the Results section. The average age of the 397 women, of whom 61 were operated on in both knees, and 130 men, of whom 15 were operated on in both knees, was 68 years at the time of the operation. The 76 bilateral patients had the knee endoprostheses implanted within a mean interval of 17 months. The most frequent indications for surgery were primary and secondary gonarthrosis, followed by rheumatoid arthritis. The evaluation of the X-rays from the above population of 138 patients revealed no radiological signs of loosening. No correlation between the time after implantation and the formation of radiolucent margins could be established. Those knee endoprostheses which were implanted with a varus deviation of more than 10 degrees did not yield a poorer result than those implanted perpendicular. In comparison with the cemented implants, the cementless implants produced slightly better results.
11624203 Renaissance mummies in Italy. 1999 The paleopathological study of 40 Italian Renaissance mummies has allowed us to perform about 20 diagnoses, of which 5 concern infectious (smallpox, hepatitis, condyloma, syphilis and pneumonia), 4 metabolic (obesity, atherosclerosis, gallstones and uric acid nephrolithiasis), 2 articular (DISH and rheumatoid arthritis) and 2 neoplastic (skin apithelioma and colon adenocarcinoma) diseases. The mummy of an anonymous child, dated back to the 16th century (C14=1569 +/- 60), presented a diffuse vesiculo-pustular exanthema. Macroscopic aspects and regional distribution suggested smallpox, while EM reavealed many egg-shaped, virus-like particles (250 x 50 nm), with a central dense core. Following incubation with anti-smallpox virus antiserum and protein A-gold complex immunostaining, the particles resulted completely covered with protein A-gold. These results clearly show that this Neapolitan child died of a severe form of smallpox some four centuries ago. The mummy of Maria of Aragon, Marquise of Vasto (1503-1568), reavealed on the left arm an oval, cutaneous ulcer (15x10 nm) with linen dressing. Indirect immunofluorescence with anti-treponema pallidum antibody identified a large number of filaments with the morphological characteristics of fluorescent treponemes. EM evidenced typical spirochetes, with axial fibril. These findings clearly demonstrate a treponemal, probably venereal, infection. The mummy of Ferrante I of Aragon, King of Naples (1431-1494), revealed an adenocarcinoma extensively infiltrating the muscles of the small pelvis. A molecular study of the neoplastic tissue evidenced a typical mutation of the K-ras gene codon 12:the normal sequence GGT (glycine) was altered into GAT (aspartic acid). At present this genetic change is the most frequent mutation of the K-ras gene in sporadic colorectal cancer. The alimentary "environment" of the Neapolitan court of the XV century, with its abundance of natural alimentary alkylating agents, well explains this acquired mutation. These and other diseases as, for example, a fatal puerperal complication, a thyroid goiter, a case of Wilson's cirrhosis, some cases of anthracosis and other peculiar traumatic conditions, such as a mortal stab-wound, can elucidate the pathocenosis of the wealthy classes of the Italian Renaissance.