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ID PMID Title PublicationDate abstract
18231794 Decreased levels of soluble receptor for advanced glycation end products in patients with 2008 Jun The purpose of this study was to evaluate the levels of sRAGE in primary Sjögren's syndrome (SS), and to assess whether there is an association between sRAGE levels and disease characteristics. Thirteen patients were randomly selected from three subgroups: primary SS, (n = 6), secondary Sjögren's, (n = 4), and ANA(+) but lacking criteria for further disease classification (n = 3). Levels of serum sRAGE were measured in triplicate using an enzyme-linked immunosorbent assay kit. Mean sRAGE levels were significantly lower in the primary Sjögren's group. Logistic regression analysis indicated that plasma sRAGE level was a significant predictor of diagnostic status. Analyses using routine serological tests for diagnosing autoimmune disorders failed to reach statistical significance. This preliminary study supports the hypothesis that the RAGE system might participate in the disease pathway of primary SS, and that sRAGE may be a potential biomarker to aid in the diagnosis of primary SS.
16378222 Accessory or sublingual salivary gland biopsy to assess systemic disease: a comparative re 2006 Mar Minor salivary gland biopsies are commonly performed as part of the diagnosis of Sjögren's syndrome or other systemic diseases. Until now, a lip biopsy taken from inside the inferior lip has been the most often performed method to assess the accessory salivary glands. Because of the risk of damaging the inferior alveolar nerve and of harvesting non-contributive biopsies, for the past several years we have chosen the sublingual biopsy described by Adam. The aim of this study was to describe the indication and diagnosis and to evaluate work incapacity, pain and the complication rate among our salivary gland biopsies. In this retrospective study, we evaluated 79 biopsies (lip, n=24, and sublingual, n=55) taken at the Department of Oral and Maxillofacial Surgery of the University Hospital St-Luc, Brussels, by multiple junior trainees and senior surgeons (n=17). Data were collected from record study and from telephonic patient questionnaires. The three major indications were: sicca syndrome (24%), suspicion of Sjögren's syndrome (32%) or exclusion of other systemic diseases (44%). The histology results of the lip and sublingual biopsies, respectively, were: normal tissue (29%, 24%), chronic inflammation (29%, 41%), compatible, but not characteristic for Sjögren (8%, 7%), Sjögren's syndrome (13%, 24%), salivary gland atrophy (13%, 0%) or non-contributive (8%, 0%). The procedures were both almost painless (time during which painkillers had to be taken: 0.33 vs. 0.69 days, NS) and work incapacity was not encountered in any group. After one lip biopsy we had to deal with a permanent anesthesia of the lower lip (6.6%), and after one sublingual biopsy a swelling of the floor of the mouth had to be incised under local anesthesia (2.7%). Thus, sublingual biopsy is an easy procedure with low morbidity and excellent reliability in comparison to lip biopsy. No salivary gland atrophy and no non-contributive biopsies were encountered; no important bleeding or nerve lesion was recorded after sublingual biopsies.
19134304 [Analysis of clinical features of autoimmune disease-related pancreatitis]. 2008 Dec OBJECTIVE: To improve the understanding of autoimmune disease related pancreatitis by analyzing their clinical features. METHODS: The clinical features were analyzed retrospectively in 28 autoimmune disease related pancreatitis cases from Peking Union Medical College Hospital (PUMCH), according to the associated autoimmune diseases. RESULTS: (1) The average age was (40.0 +/- 16.1) years, and the ratio of male to female patients was 1:6. There were 24 acute and 4 chronic pancreatitis in the 28 cases. (2) The common related autoimmune diseases were systemic lupus erythematosus (20/28) and Sjögren's syndrome (6/28). (3) The characteristics of the autoimmune diseases was multi-system involvement, such as hematologic system, kidney, liver, etc. (4) Clinical features of those acute pancreatitis shown that no distinct trigger exist for acute pancreatitis, and the radiological changing was not prominent. (5) In laboratory examination, an obvious increase of CA199 could be seen, paralleling the severity of pancreatitis. (6) Glucocorticoids or immunosuppressors was effective, and the mortality rate of acute pancreatitis cases was 33.3%. CONCLUSIONS: Autoimmune disease related pancreatitis is dominant with acute pancreatitis and females is common, which may reflect the activity of autoimmune diseases. Autoimmune disease related acute pancreatitis has a high mortality rate. Glucocorticoids and/or immunosuppressors may be useful to relieve the pancreatitis.
18762861 A case report of a patient with refractory adult-onset Still's disease who was successfull 2009 Interleukin-6 overproduction is pathologically involved in adult onset Still's disease (AOSD). We successfully treated a man with refractory AOSD utilizing tocilizumab. Tocilizumab was discontinued after 15 doses due to intestinal bleeding, but the efficacy was sustained over 21 months. Tocilizumab was readministered safely upon recurrence and showed similar efficacy over 6 years. Corticosteroid and NSAIDs could be discontinued and intestinal bleeding was no more observed. Tocilizumab can be a therapeutic option for AOSD.
17586555 Emerging biological therapies in primary Sjogren's syndrome. 2007 Sep Sjögren's syndrome (SS) is a systemic autoimmune disease that mainly affects the exocrine glands and usually presents as persistent dryness of the mouth and eyes. SS primarily affects white perimenopausal women, with an incidence of 4-5 cases per 100 000. Recent studies have analysed new therapeutic approaches, focusing mainly on the use of biological agents. B-cell targeted therapies seem to be the most promising agents in primary SS, especially rituximab, which has been used in more than 50 reported cases. Other promising B-cell targeted therapies include epratuzumab and belimumab, while T-cell targeted agents (efalizumab, abatacept, alefacept) should currently be considered as possible future options. In the near future, biological agents will play key roles in the treatment of severe involvement, broadening the therapeutic options in primary SS and offering a more optimistic point of view of the treatment of this disease, which, at present, is often considered to lack adequate specific therapy. However, the possible risks and benefits of using these agents should be carefully balanced, and a reasonable assessment of the risk of serious adverse events versus the benefits of treatment should be made. The use of biological agents targeting molecules and receptors involved in the aetiopathogenesis of primary SS opens a new era in the therapeutic management of patients with primary SS.
17274588 Amyloid deposition of the breast in primary Sjögren syndrome. 2006 Nov We report amyloid deposition in the breast presenting as suspicious microcalcifications on screening mammography. Stereotactic mammotome biopsy provided the diagnosis. The history of the patient revealed primary Sjögren syndrome. The combination of amyloid deposition in the breast and Sjögren's syndrome has only rarely been reported.
17130315 Infective endocarditis associated with Sjogren's syndrome. 2006 Dec Sjögren's syndrome is one of the major autoimmune diseases, however infective endocarditis associated with Sjögren's syndrome has not previously been reported. A patient with Sjögren's syndrome associated with aortic valve regurgitation due to infective endocarditis, underwent successful aortic valve replacement. Patients with Sjögren's syndrome tend to be at a higher risk of intraoral infections due to diminished secretion of saliva. Particular care must be taken of Sjögren's syndrome patients as they can also be at an increased risk of infective endocarditis.
16883110 [A case of systemic sclerosis and Sjogren's syndrome with cardiac tamponade due to steroid 2006 Jul A 75-year-old woman was admitted to our hospital because of abnormal lung shadow and necrosis of the left feet. She had a history of Raynaud's phenomenon from her twenties. On admission, she was diagnosed as having diffuse systemic sclerosis (SSc) and Sjögren's syndrome (SjS) because of scleroderma, interstitial pneumonia (IP), positive result of anti-Scl-70 and SS-A antibody, sicca, decreased tear excretion, and dysfunction of salivary glands. Seventy days after amputation of her left leg, she presented with edema, hypoxemia, chest discomfort, and fever. Blood test revealed inflammation and cardiac echography revealed pericardial effusion with a collapse sign of right atrium, thereby leading to the diagnosis of cardiac tamponade. After starting the daily dose of 20 mg of prednisolone, the pericardial effusion and cardiac tamponade sign disappeared. Pericarditis is seen in half of patients with SSc and rarely with SjS, and is usually asymptomatic. Pericarditis due to SSc has been reported unresponsive to steroid therapy, but several cases of steroid responsive pericarditis due to SSc or SjS have been reported. Clinically, they shared inflammatory responses and the presence of IP in the cases of SSc, which will be important when considering the pathogenesis and treatment of pericarditis due to SSc or SjS.
16758758 [Visual functions' detailed evaluating in patients with Sjögren's syndrome before and aft 2006 May The aim of the study was to determine exact visual functions (log MAR [minimal angle of resolution] and CS [contrast sensitivity]) and to evaluate corneal topographic maps in patients with established (by means of laboratory and biopsy examinations) Sjogren's Syndrome, and to determine the difference in subjective symptoms before and after insertion of the intracanalicular implants as well. PATIENTS AND METHODS: Twelve eyes (1 man, 6 women) with established Sjogren's syndrome were examined before and during two months after the insertion of the plugs. The best-corrected visual acuity (BCVA) was assessed on Landolt C rings optotypes. CS was measured on computer-controlled device (Neuroscientific Corp., U.S.A.) in 6 space-frequencies (0.74-29.55 c/deg). The corneal topographic changes (Keraton Opticon) were established by means of comparing total aberrations values before and after the intracanalicular implants' (Smart Plugs type) insertion. The control group for visual functions assessment consisted of 10 woman (20 eyes) of similar middle age. RESULTS: The BCVA on log MAR optotypes was 0.84 (0.69-0.95) before and 0.88 (0.52-1.23) after the insertion, on both occasions, it was lower than in the control group. The CS was before the insertion in all of the spatial frequencies lower, the largest differences were in the frequencies range 1.97-7.29 c/deg (p < 0.01). After the treatment, the values grow but they don't reach the values of the control group. Subjective complains of the patients decreased markedly; in 100% they had relief, in 3 cases they referred improvement up to 50%; no patient observed tearing. The frequency of drops' application has decreased by 63%. The Schirmer test, in 100% positive before the treatment, was after the insertion in 75% negative; the height of the tear-meniscus was positive in 100% before the procedure, and after that, its measurement improved to 1 mm in 91%; in 9% it was 1.5 mm. We also noticed changes of the ocular surface by means of lissamine green staining; this test was before the procedure positive in 100%, the improvement after that was in 63%. The regularity of the corneal surface is the determining factor of visual functions in "dry eyes". The measurement of the corneal topography is useful in differential diagnosis and helps to distinguish mild and more serious conditions of dry eye. The improvement of BCVA and CS values after insertion corresponds with patients' subjective evaluating. The best value of the treatment is improvement of the patients' comfort; furthermore, after insertion of the permanent plugs they feel pronounced subjective relief and lowering of the frequency of drops application.
18059240 Diagnostic evaluation of serial sections of labial salivary gland biopsies in Sjögren's s 2007 Dec 1 OBJECTIVES: Sjögren's syndrome is a chronic inflammatory disease. The detection of chronic inflammatory infiltrates containing >50 lymphocytes (lymphocytic focus) per 4 mm2 tissue in minor salivary gland biopsies is a diagnostic parameter of the disease. The aim of the study was to examine if an increase in the tissue area of a single minor labial salivary gland biopsy through serial histological sections in patients affected by primary Sjögren's syndrome could facilitate the detection of the diagnostic focus score (grades >1 or >2). METHODS: We observed 24 labial salivary gland biopsies from patients affected by primary Sjögren's syndrome, diagnosed according to the clinical-laboratory criteria proposed by the American-European Consensus Group. The analysis was carried out on sections (n= 72) obtained at three different levels at 200 micrometers from one another. The serial sections regarding the 3 levels were reviewed by the same oral pathologist, who detected both the total number of foci, and their surface, calculating a cumulative focus score. RESULTS: No significant correlation was found between the number of lobules per histological section and the focus score (Pearson correlation 0.363, p= 0.01). No significant variation in focus score distribution was identified in the three serial histological levels at 200 micrometers from one another. From the comparison between the number of lobules observed and the focus score grade, no direct proportionality between the amount of parenchyma analyzed and the focus score was found. CONCLUSIONS: The focus score remained unchanged in the serial sections at different depths.
17509507 Tear evaporation rates in Sjögren syndrome and non-Sjögren dry eye patients. 2007 Jul PURPOSE: To reinvestigate tear evaporation rates in Sjögren syndrome (SS) and non-Sjögren (non-SS) dry eye patients with a recently reported ventilated chamber evaporimeter system. DESIGN: Prospective case-control study. METHODS: A ventilated chamber evaporimeter system was used to measure tear evaporation rates. A DR-1 camera (Kowa, Nagoya, Japan) was used for tear lipid layer interference image acquisition. The Yokoi severity grading system was used for DR-1 image evaluation. Twenty-four aqueous tear deficiency (ATD) eyes of 21 consecutive patients with SS were studied (SS ATD group). Twenty-one ATD eyes of 12 non-SS patients (non-SS ATD group) were examined as control subjects. RESULTS: Tear evaporation rates of the SS ATD group (5.9 +/- 3.5 [10(-7) g/cm(2) per second]) were significantly higher than those of the non-SS ATD group (2.9 +/- 1.8 [10(-7) g/cm(2) per second]; P = .0009). The severity grading of DR-1 tear interference images of the SS ATD group was significantly higher (P = .03), along with significantly worse meibomian gland expressibility and vital staining scores, compared with those of the non-SS ATD group. CONCLUSIONS: Tear evaporation rates were higher in eyes of the SS ATD group compared with the non-SS ATD group. Tear evaporation assessed in conjunction with tear lipid layer findings and meibomian gland expressibility provides an increased understanding in the differential diagnosis of dry eye states.
16519773 A simple test for salivary gland hypofunction using Oral Schirmer's test. 2006 Apr OBJECTIVE: The objective of this study was to develop a test for detecting salivary gland hypofunction. STUDY DESIGN: Oral Schirmer's test was performed by placing a strip of filter paper on the floor of the mouth and measuring the wetted length after 5 min. The control group consisted of 70 healthy patients, while another group consisted of 61 patients with Sjögren's Syndrome (SS) and a third group of 31 patients who suffered from xerostomia caused by other pathologies. RESULTS: The mean saliva flow was 40.92 +/- 22.28 mm/5 min in the control group, 27.25 +/- 24.11 mm/5 min in patients with SS and 36.847 +/- 23.4 mm/5 min in the third group. The differences between the control group and the other two groups were statistically different (P > 0.001). CONCLUSIONS: The whole saliva test was used to distinguish between healthy adults and subjects with hyposalivation.
18793567 A case of membranous nephropathy associated with Sjögren syndrome, polymyositis and autoi 2008 Sep Sjögren syndrome (SS) is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, especially lacrimal and salivary. The immunologic process which occurs in this syndrome is B cell hyperactivity, which results in production of autoantibodies and immune complexes. SS can exist as a primary disorder or in association with other autoimmune processes. A usually mild, proximal and insidious inflammatory myopathy can occur in patients with SS with a broad clinical and pathological spectrum. Interstitial nephritis with mild proteinuria and tubular dysfunction is the most common renal manifestation of SS, but glomerular involvement due to immune complex deposition may also rarely occur [Goules et al. 2000]. There is an association of SS with hepatic abnormalities, as evidenced by abnormal liver biochemical tests or histological characteristics of primary biliary cirrhosis (PBC), portal tract fibrosis, or autoimmune hepatitis [Abraham et al. 2004]. The pathogenetic mechanism of liver involvement in SS is not clear, but it is possible that hepatic and salivary gland damage share a similar pathology. The combination of Sjögren syndrome with kidney, liver and muscle involvement in one entity is extremely rare and data in the literature are remarkably sparse. We present a case of a 43-year-old female patient suffering from SS accompanied by polymyositis, membranous nephropathy and autoimmune hepatitis.
17939337 Pulmonary nodular lymphoid hyperplasia associated with Sjögren's syndrome. 2007 Sep Pulmonary nodular lymphoid hyperplasia (NLH) is a term first suggested by Kradin and Mark to describe one or more pulmonary nodules or localized lung infiltrates consisting of reactive lymphoid proliferation. To date, there have been only a few cases of pulmonary NLH reported associated with autoimmune disorders. There is no case of NLH associated with Sjögren's syndrome from Korea in the medical literature. A 56-year-old woman was referred to our hospital with cough productive of sputum and chest tightness. The Computed tomography scans of the chest revealed multiple and well-defined peribronchiolar nodular opacities. A video assisted thoracoscopic surgery (VATS) biopsy was performed and the nodular opacity in the lung parenchyma was pathologically confirmed as NLH. Through meticulous review of patient's record, we found that she had been suffering from dry eye and dry mouth. The symptoms suggested Sjögren's syndrome, which was confirmed by specific laboratory tests including the Schirmer test, anti-nuclear antibody and anti-Ro/La antibody. The patient is followed regularly and has no further progression of symptoms.
17846210 MR imaging of labial glands. 2007 Sep BACKGROUND AND PURPOSE: The labial salivary gland is a site of occurrence of tumors and cysts, and it serves as the biopsy site for the diagnosis of Sjögren syndrome. However, its imaging features have not been well understood. Here we attempted to depict the labial gland by high-resolution MR imaging. MATERIALS AND METHODS: The labial glands from 89 patients without Sjögren syndrome, 14 patients with Sjögren syndrome, and 3 patients with tumor or cyst of the lips were imaged by using a 1.5T MR imager with a 47- or 110-mm surface coil. RESULTS: The upper and lower labial glands consisted of 1-3 layers of gland clusters, each of which had high signal intensity on T1-weighted and fat-suppressed T2-weighted images and was readily enhanced after gadolinium injection. The posterior parts of the glands were thicker than the anterior parts. The gland areas in the lower lips (186 +/- 64 mm(2) in women and 192 +/- 68 mm(2) in men) were greater than those in the upper lips (140 +/- 46 mm(2) in women and 162 +/- 66 mm(2) in men). We did not find any significant age-related changes or sex differences in the gland area. The labial gland areas were smaller in the patients with Sjögren syndrome than in patients without Sjögren syndrome, though the difference was significant only in the upper lips (104 +/- 53 mm(2)). CONCLUSION: This is the first report describing imaging features of the labial salivary glands. High-resolution MR imaging can readily delineate the labial glands.
16159947 The spectrum of renal tubular acidosis in paediatric Sjögren syndrome. 2006 Jan OBJECTIVES: Renal tubular acidosis (RTA) is a well-recognized extraglandular complication of adult Sjögren syndrome (SS) but has been reported only rarely in paediatric SS. We wished to describe the natural history of RTA in paediatric SS. METHODS: We performed a chart and literature review. Inclusion criteria were primary or secondary SS with onset before 18 yr of age, complicated by RTA before 18 yr of age. RESULTS: Twelve cases were identified: two from chart review and 10 from the literature. RTA was mostly associated with primary SS. RTA was detected at the onset of SS or up to 9 yr later. The clinical spectrum ranged from nearly silent to life-threatening, with plasma pH and serum potassium as low as 7.0 and 1.2 mEq/l, respectively. Hypokalaemia was present in 92%. Half the patients presented with profound weakness or paralysis, most likely from hypokalaemia. Proximal, distal and mixed RTA were detected, reflecting a diffuse 'tubulopathy' from interstitial nephritis, which was the predominant histopathological finding. Diabetes insipidus was the most frequent renal comorbidity. The RTA stabilized in 82% of the cases and resolved in one case. Only one patient had long-term unstable RTA. CONCLUSIONS: RTA is an under-recognized complication of paediatric SS. It can be life-threatening in the acute phase but generally has a good long-term renal outcome. SS should be considered in the older child with otherwise unexplained RTA. Likewise, RTA should be excluded in children and adolescents with SS who develop weakness, fatigue or growth failure. Early recognition would reduce long-term complications such as growth failure.
17933436 Anti-inflammatory effects of BT-201, an n-butanol extract of Panax notoginseng, observed i 2007 Dec BACKGROUND & AIMS: Although there has been some success with protein-based anti-tumor necrosis factor alpha (TNF-alpha) therapeutics, the problems associated with protein-based drugs demand alternative approaches. We screened various herbal extracts for their ability to inhibit TNF-alpha secretions and found that BT-201, an n-butanol extract of Panax notoginseng (Burk.) F. H. Chen (P. notoginseng) has such an ability. METHODS: The purpose of this study has been to evaluate the anti-inflammatory and anti-rheumatic effects of BT-201. The anti-inflammatory effects were evaluated by measuring the effects of BT-201 on the production of TNF-alpha, interleukin (IL)-1beta, inducible nitric oxide (iNO), and matrix metalloproteinase-13 (MMP-13), in vitro. The anti-rheumatic effects were evaluated by treating mice with collagen-induced arthritis (CIA) using a daily oral administration of BT-201 at 15 mg/kg/day. In addition, the effects on NF-kappaB and mitogen-activated protein kinase (MAPK) pathways were evaluated by Western blotting using phospho-specific antibodies. RESULTS: BT-201 significantly inhibited all the inflammatory parameters evaluated in vitro and delayed the onset and progression of CIA. BT-201 inhibited the activation of NF-kappaB, ERK, p38, and JNK pathways. CONCLUSIONS: Our results demonstrated that BT-201 can modulate various aspects of inflammation in vitro and that it has disease-modifying, anti-rheumatic effects in vivo, suggesting that it can be a potential alternative to the current anti-TNF-alpha therapeutics for rheumatoid arthritis and other inflammatory disease.
17827786 Decreased intestinal CYP3A and P-glycoprotein activities in rats with adjuvant arthritis. 2007 Aug Adjuvant-induced arthritis (AA) rats have been used as an animal model for rheumatoid arthritis. Several studies have shown that the pharmacokinetics of a number of drugs are altered in AA rats. We investigated the effects of AA on the barrier functions of the intestine using a rat model. Intestinal CYP3A activities (midazolam 1'-hydroxylation and 7-benzyloxy-4-(trifluoromethyl)-coumarin 7-hydroxylation) in AA rats were significantly decreased compared with those in normal rats, with marked decrease observed in the upper segment of intestine. Intestinal P-glycoprotein (P-gp) activity at upper segment was also significantly decreased in AA rats to 60% of that in normal rats, and the other segments (middle and lower) of intestine also exhibited tendencies toward decrease in P-gp activity. This decrease was supported by the finding that levels of mdr1a mRNA and P-gp protein were decreased in AA rats. No significant differences were observed in intestinal paracellular and transcellular permeability between AA and normal rats. These results suggest that intestinal CYP3A and P-gp activities are decreased in AA rats, and that the pharmacokinetics and bioavailabilities of drugs whose membrane permeation is limited by intestinal CYP3A and/or P-gp may be altered in rheumatic diseases.
18273538 A case of planned pregnancy with an interruption in infliximab administration in a 27-year 2008 We report a 27-year-old case of juvenile idiopathic arthritis (JIA) having been stopped infliximab during pregnancy. She was safely treated by infliximab therapy with premedications for preventing infusin reactions after her delivery, and then improved in the same manner as when she had been treated with infliximab therapy before pregnancy. As a result, it remains unclear whether or not we can use infliximab to control disease activities during pregnancy. In addition, it is also important to clarify whether or not premedications should be used when resuming infliximab treatment in such patients after pregnancy. These problems still remain controversial. More definitive data are needed in order to allow rheumatologists to better select the optimal TNF-alpha inhibitor therapy when treating pregnant JIA patients.
18984351 Implant arthroplasty for the distal radioulnar joint. 2008 Nov The distal radioulnar joint (DRUJ) is a weight-bearing joint; the ulnar head is frequently excised either totally or partially and in some cases is fused because of degenerative, rheumatoid, or posttraumatic arthritis and treated with a "salvage procedure." The result of these procedures is the inability of those patients to lift even minor weight. Articles about these procedures report the ability to pronate and supinate, but they rarely discuss grip strength or lifting capacity. We present an alternative to the salvage procedures that allows full range of motions as well as the ability to grip and lift weights encountered in daily living, such as a gallon of milk. The Aptis total DRUJ replacement prosthesis (Aptis Medical, Louisville, KY), a bipolar self-stabilizing DRUJ endoprosthesis, restores the forearm function. The technique of implantation is presented here.