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ID PMID Title PublicationDate abstract
10914871 Hepatitis A infection mimicking adult onset Still's disease. 2000 Jul Fever, rash, and arthritis may be components of the prodrome of viral hepatitis. In the absence of jaundice and abnormal liver function tests, this form of polyarthritis is easily confused with primary autoimmune diseases. Whereas the association of systemic illness with musculoskeletal symptoms and numerous viral infections is well known, such an association with hepatitis A has only been rarely reported. We describe a case of hepatitis A infection mimicking adult onset Still's disease, and review the pathogenesis and differential diagnosis of Still's disease and the extraarticular manifestations of hepatitis.
10866104 Acute optic neuropathy and transverse myelopathy in patients with antiphospholipid antibod 2000 We describe two patients with established antiphospholipid syndrome, who during periods of subtherapeutic anticoagulation, developed acute optic neuropathy and transverse myelopathy. Treatment with optimal anticoagulation and high dose glucocorticoids was followed by resolution of the neurologic deficits.
10556760 Diagnostic value of sialography with both the conventional and digital subtraction techniq 1999 Nov OBJECTIVE: The application of anamnestic data on siccative symptoms required for classifying adult Sjögren's syndrome is limited in childhood. Instrumental test procedures are therefore necessary for objectively recording the oral and ophthalmologic manifestations of the disease. The aim of this study was to clarify the sialographic changes that occur in Sjögren's syndrome in children. STUDY DESIGN: A total of 23 sialograms were obtained with both conventional and digital subtraction techniques in 21 children with primary (10 girls and 1 boy) or secondary Sjögren's syndrome (10 girls). The films were assessed by 3 physicians and submitted for a consensus analysis if necessary. RESULTS: The pathologic features observed in the children varied from a slightly narrowed ductal system to multiple peripheral ductal ectasias and completely destroyed parenchyma. Sialographic examinations demonstrate that, with progressing disease, regression of acinar dilatations and rarification of the ductal system occur. CONCLUSION: The results show that the spectrum of sialographically recordable lesions in Sjögren's syndrome in children is greater than is described thus far in the literature.
10556265 The occurrence of renal involvement in primary Sjögren's syndrome: a study of 78 patients 1999 Nov OBJECTIVE: To ascertain the occurrence of renal involvement in patients with primary Sjögren's syndrome (pSS). METHODS: Urinary total protein excretion from 24 h urine collection, as well as urinary excretion rates of albumin, alpha-1 microglobulin (alpha1m) and IgG from overnight 8 h collections, were determined from 78 pSS patients (75 females, three males). Urine acidification capacity after oral ammonium chloride load was tested in 55 of these patients. RESULTS: Mild proteinuria (0.15-0.42 g/24 h) was observed in 34 patients (44%). Increased urinary excretion rates of albumin (>/=20 microgram/min), alpha1m (>/=7.0 microgram/min) or IgG (>/=5.0 microgram/min) were detected in nine (12%), nine (12%) and 11 patients (14%), respectively. Latent or overt distal renal tubular acidosis (dRTA) was observed in 18 out of 55 patients with pSS (33%). These patients had a longer duration of the disease (10+/-4 vs 8+/-4 yr; P
10461535 Pulmonary involvement in adult-onset Still's disease. 1999 Sep Adult-onset Still's disease (AOSD) is a rare splenic disorder with an unknown cause. It is not uncommon for AOSD to involve other organs, such as the liver; the kidney; the bone marrow; and, less often, the lungs. In this review, we discuss the pulmonary complications of AOSD. Pulmonary involvement in AOSD usually consists of pleural effusion or transient pulmonary infiltrates, but it may become life threatening if it progresses to the adult respiratory distress syndrome. Chronic conditions, such as restrictive lung disease, have also been reported in patients with AOSD. The only treatment currently available is high-dose steroids, although other agents, such as intravenous immunoglobulin, cyclophosphamide, and azathioprine, have been tried with some success.
9476266 Sjögren's syndrome with anticentromere antibodies. 1997 Dec Clinical and laboratory findings in 10 women with Sjogren's syndrome and positive anticentromere antibodies were compared to those in 50 patients with Sjögren's syndrome and typical serologic features. The anticentromere antibody-positive patients were more likely to have Raynaud's phenomenon and less likely to have leukopenia polyclonal hypergammaglobulinemia, rheumatoid factor, and anti-SSA/Ro antibody. Four anticentromere antibody-positive patients developed limited cutaneous scleroderma during follow-up, but none had lymphoma; some of the other patients in this group exhibited stable symptoms typical for Sjögren's syndrome. Among our overall population of Sjogren's syndrome patients who met diagnostic criteria for primary Sjogren's syndrome at the first evaluation, 16.6% tested positive for anticentromere antibodies.
10935342 Salivary gland MALT lymphoma associated with Helicobacter pylori infection in a patient wi 2000 Jul We report a case of salivary gland MALT lymphoma in Sjögren's syndrome associated with localized H. pylori infection. A 76-year-old woman had a history of bilateral cheek masses for two years. Histologically, the parotid glands were invaded by numerous centrocyte-like cells to form lymphoepithelial lesions. The tumor cells showed immunohistological differentiation into B cells. Southern blotting demonstrated immunoglobulin gene rearrangement. These results indicated that the tumors were MALT lymphoma. H. pylori, as assessed by the urease test (CLO test; BML Ltd., Tokyo, Japan), was positive in the tumor specimen. After wide local excision of the tumors followed by radio therapy and oral administration of antibiotics and proton pump inhibitor, no evidence of recurrence was found during the 24-months of follow up. H. pylori infection in the salivary gland is rare, although the source of infection and transmission of H. pylori organisms has been thought to be the oral cavity. We discussed the association between H. pylori infection and salivary gland MALT lymphoma. The microorganism may play a role as an additional antigenic stimulus for the development of salivary gland MALT lymphoma as well as for the development of gastric MALT lymphoma. This means that H. pylori can play a role in lymphoma progression as booster of B cell lymphoproliferation.
9634933 Alterations of ocular surface gene expression in Sjögren's syndrome. 1998 We have demonstrated that conjunctival epithelium of SS patients displays increased numbers of S-phase cells compared with non-dry eye controls. Moreover, in SS patients, these S-phase cells are distributed throughout all strata of the epithelium. The expression of MUC-1, a cell surface marker indicative of terminally differentiated epithelium, is localized to the conjunctival epithelial surface in SS and control patients. However, MUC-1 surface immunoreactivity appears to be reduced in SS epithelium, suggesting disruption of normal epithelial differentiation. A MUC-1 epitope exposed by pretreatment with neuraminidase is expressed in the basal and suprabasal layers of both patient populations. This antigen likely represents nascent, partially processed MUC-1(6) and may serve as a marker of the preterminally differentiated epithelial phenotype. Messenger RNA encoding several different inflammatory cytokines, including TNF-alpha, IL-1 alpha and beta, IL-6, IL-8, IL-10, and TGF-beta 1, is expressed at elevated levels within the conjunctival epithelium of SS patients compared with non-dry eye controls. Based on these observations, we have formulated a model to explain the ocular surface pathology of Sjögren's syndrome. We hypothesize that mechanical abrasion secondary to aqueous tear deficiency creates an inflammatory environment where conjunctival epithelial cells and lymphocytes are stimulated to produce and secrete various cytokines (i.e., IL-1, TNF-alpha, IL-6, IL-8, etc.) into the tear film. Elevated cytokine levels within the tear film, perhaps combined with reduced concentrations of essential lacrimal gland-derived factors (i.e., EGF, retinol), create an environment in which terminal differentiation of the ocular surface epithelium is impaired. As a consequence, the epithelium becomes hyperplastic, displaying increased mitotic activity, and loses the ability to express mature protective surface molecules including the membrane-bound mucin, MUC-1. This would imply that anti-inflammatory medications (i.e., corticosteroids or cyclosporine) that suppress the inflammatory component of this cascade may ameliorate the ocular surface disease and discomfort experienced by SS patients.
9827363 Increased serum concentrations of secretory leukoprotease inhibitor in patients with prima 1998 Sep BACKGROUND: Secretory leukoprotease inhibitor (SLPI), a 12 kDa serine antiprotease, is produced by serous cells including salivary and lacrimal glands. OBJECTIVE: We sought to assess whether serum levels of SLPI in patients with primary Sjogren's syndrome (SS) are elevated and correlated with conventional and laboratory indices of disease activity. PATIENTS AND METHODS: SLPI levels were determined by ELISA of serum from 21 primary SS, and 26 age-matched normal controls. RESULTS: Increased SLPI concentrations were found in primary SS patients (p < 0.01). Serum SLPI levels were positively correlated with the duration of the disease (r = 0.517, p < 0.025), but not with age, erythrocyte sedimentation rate, CRP, immunoglobulins, number of extraglandular manifestations and saliva production. SLPI levels were also positively correlated with serum levels of beta 2-microglobulin (r = 0.607, p < 0.01). CONCLUSION: Determination of serum SLPI may provide a new simple noninvasive technique for evaluation of primary SS.
10410870 Clinical application of a homogeneous colorimetric assay for tear lysozyme. 1999 Mar PURPOSE: Tear lysozyme and tear lactoferrin are enzymes synthesized by the lacrimal gland. Their concentration in human tears reflects tear gland function. Tear gland dysfunction can lead to ocular surface disease. We developed a colorimetric lysozyme assay. The objective of this study was to determine the diagnostic power and the clinical application of this assay that allows rapid and precise quantification of tear lysozyme. METHODS: Tear specimens of 120 eyes (30 Sjögren's patients and 30 controls) were collected using standardized filter paper discs. Tear lysozyme concentration was determined using p-nitrophenyl penta-N-acetyl-beta-chitopentaoside as substrate in the colorimetric assay. The results were compared to clinical findings and to two commonly used tests, the Micrococcus agar diffusion assay for tear lysozyme and the tear lactoferrin immunodiffusion assay. RESULTS: The colorimetric assay showed a good dose-response relationship. The use of the assay as a method of diagnosing aqueous tear deficiency, using the clinical findings and the medical history as gold standard, demonstrated 85% sensitivity and 92% specificity. The results of the colorimetric assay when compared with the Micrococcus agar diffusion assay showed a linear relationship of r=0.77; when compared with the lactoferrin immunoassay r=0.73. CONCLUSIONS: The colorimetric assay is simple to perform and does not require sophisticated laboratory equipment and personnel. Results can be precisely quantified within one hour after tear collection. The diagnostic power of the test is comparable to previously reported assays for lysozyme and lactoferrin and will be useful in the diagnosis of ocular surface disease.
9311000 [A case of Sjögren syndrome associated with multiple mononeuritis and dysautonomia includ 1997 Sep Sjögren syndrome (SjS) is a glandular disease characterized by dry eyes and dry mouth. Extraglandular manifestations in SjS are also common, and peripheral nerve involvement has been reported in 10-20% of cases. We report a case of Sjögren syndrome with bilateral tonic pupils, dysautonomia, and multiple mononeuritis. The fact that sural nerve sections, in addition to marked loss of myelinated and unmyelinated fibers, showed an increased number of infiltrating macrophages without lymphocytes and aberrant expression of HLA-DR (class II) antigen in Schwann cells was an especially interesting finding. No evidence of active vasculitis was detected. The patient was treated with corticosteroids and her condition gradually improved, as confirmed by thermography. Our findings suggested the presence of specific immunological abnormalities simultaneously involving the ciliary ganglia, autonomic ganglia, and dorsal root ganglia in this peculiar form of SjS.
11812334 [Evaluation of saliva ferning test in diagnosis of Sjögren's syndrome]. 2001 May OBJECTIVE: To evaluate saliva ferning test in (SFT) diagnosis of xerostomia in patients with Sjögren's syndrome (SS). METHODS: Dried samples of freshly produced saliva from 78 patients diagnosed as SS according to european community criteria and 80 healthy controls were examined by light microscopy. The crystallization was classified into 4 types according to the ferning phenomenon: uniformity, branching, spreading and integrity (type I normal and type II, III, IV abnormal). Then, the 78 patients underwent labial salivary gland biopsy. According to Tarpley's classification of labial gland biopsy, > or = 2+ was considered positive. RESULTS: (a) The sensitivity and specificity of SFT were 89.74% (70/78) and 83.75% (67/80) respectively. (b) Abnormal SFT was observed in 70/78 (89.74%) of SS and in 13/80 (16.25%) of normal controls. The differences of SFT in SS patients versus normal controls were significant (P < 0.01). (c) The sensitivity of SFT and labial gland biopsy had no significant differences (P > 0.05) as diagnostic tests in SS. CONCLUSIONS: SFT was valuable as a diagnostic test in patients suspective of SS.
11352123 Primary biliary cirrhosis: from induction to destruction. 2001 Apr Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that predominantly affects middle-aged women; fatigue and pruritus are the most common symptoms at presentation. Liver function tests are consistent with cholestasis and reveal an elevation of serum alkaline phosphatase and gamma-glutamyl transpeptidase with or without elevation of aminotransferase levels. Histologically, PBC is characterized by the destruction of the intrahepatic small bile ducts and subsequently fibrosis. The serological hallmark of the disease is the presence of antimitochondrial antibodies, which are found in 95% of patients with PBC. The antimitochondrial antibodies are directed against the 2-oxo-acid dehydrogenase complexes located on the inner membrane of the mitochondria. PBC generally slowly progresses, even over decades, and may lead to liver failure. In symptomatic patients, advanced age, elevated serum bilirubin levels, decreased serum albumin levels, and cirrhosis each correlate with shortened survival. Immunosuppressive and anti-inflammatory drugs have been used in the treatment of PBC based on the presumed autoimmune pathogenesis, but satisfactory agents leading to complete reversal or cure of the disease are not available. At present ursodeoxycholic acid appears to be the only effective therapy in preventing or delaying the need for liver transplantation and improving survival. However, a number of patients receiving ursodeoxycholic acid still develop progressive disease and require transplantation; transplantation is the only effective therapy at the end stage of the disease.
11235787 A shock associated with adult-onset Still's disease. 2001 Feb Only two cases of adult-onset Still's disease associated with shock have been previously described. We report a case of shock in a man with adult-onset Still's disease and discuss the relationship between the two processes by assessing tumor necrosis factor-alpha, procalcitonin and interleukin-6 concentrations.
10791624 Different articular outcomes of Still's disease in Chinese children and adults. 2000 The clinical manifestations, treatment and course, and articular outcomes of 24 children with juvenile-onset Still's disease (JOSD) and 21 adults with adult-onset Still's disease (AOSD) were compared retrospectively. There was no significant difference in the initial clinical and laboratory manifestations except that more adults presented with a sore throat (81% vs. 46%, p = 0.03). Although serum ferritin was almost always elevated in both diseases, adults had significantly higher serum ferritin concentrations compared with those of children. Steroid treatment was required in 71% of children and 52% of adults, while disease-modifying antirheumatic drugs were used in 42% of children and 24% of adults during the course. Chronic arthritis (>6 months) occurred in comparable proportions of patients with JOSD and AOSD (46% vs 38%, p = 0.82), irrespective of the disease pattern (monocyclic or polycyclic). However, severe deforming arthritis with marked functional limitation occurred only in JOSD, particularly with polyarthritis at disease onset (more than five affected joints). In contrast, AOSD patients with chronic arthritis had a favourable functional outcome at the end of the follow-up. Our study suggested different articular outcomes of Still's disease in Chinese children and adults.
10411361 Calcinosis cutis and intestinal pseudoobstruction in a patient with adult onset Still's di 1999 Jun Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown origin, characterized by a typical spiking fever, evanescent salmon-colored rash, polyarthralgia, and myalgia. Calcinosis cutis and gastrointestinal involvement have rarely been noted in AOSD. We herein describe a 54-year-old woman who demonstrated repeated disseminated intravascular coagulation (DIC), and adult respiratory distress syndrome (ARDS), associated with AOSD. The patient also revealed a remarkable degree of digital calcinosis cutis and intestinal pseudoobstruction. A connective tissue disease, such as systemic sclerosis, might have been the underlying factor in the latter two symptoms.
10357112 Sjögren's syndrome and hepatitis C virus. 1999 Sjögren's Syndrome (SS) is an autoimmune disease that mainly affects exocrine glands and usually presents as a persistent dryness of the mouth and eyes. The spectrum of the disease extends from an organ-specific autoimmune disease to a systemic process. Viral infection has long been suspected as a potential cause of SS because several viruses have been incriminated in the aetiology of this disease, and a possible relationship between SS and hepatitis C virus (HCV) was postulated in 1992. In this paper, we review the literature concerning SS and HCV infection and summarise the current knowledge regarding their association and their pathogenic, clinical and immunological significances. The main conclusions of this review are that the prevalence of antibodies to HCV in patients with primary SS ranges between 14 and 19% using third-generation ELISA, chronic HCV infection may mimic the main clinical, histological and immunologic features of 'primary' SS and, finally, testing for HCV infection must be performed in patients with SS, especially in those patients with evidence of liver involvement or associated cryoglobulinaemia. HCV seems to be a rare cause of 'primary' SS in the absence of recognised liver disease or cryoglobulinaemia.
9825931 Salivary gland changes in the NOD mouse model for Sjögren's syndrome: is there a non-immu 1998 Aug Sjögren's syndrome is a systemic autoimmune disease characterized by patient complaints of oral and ocular dryness accompanied by clinical observations of a progressive loss of salivary and lacrimal function, related to the presence of a focal, periductal leukocyte infiltrate. Progress in understanding the mechanisms involved in the development of autoimmune diseases in general, and Sjögren's syndrome specifically, has been generated as a result of renewed interest in animal models, such as the NOD mouse, which mimics autoimmune sialoadenitis. Biochemical analyses have indicated that the salivary glands have reduced beta-adrenergic, muscarinic, and neuropeptide signal transduction responses that correlate to reduced receptor density and the appearance of autoantibodies directed against these and other cell surface proteins. Using the NOD-scid mouse (lacking functional B- and T-lymphocytes) it has been determined that salivary flow rates are normal; however, these animals show abnormal changes in protein biosynthesis with increasing age. Histological evaluation of the submandibular gland from older NOD-scid mice revealed a loss of acinar cells accompanied by a potential increase in the ductal cell component of the tissue. Consistent with this finding, we recently have observed increased levels of cell death-associated cysteine proteases in the submandibular glands of 20 week NOD and NOD-scid mice but not in BALB/c and young NOD controls. Other novel protease activity was detected in the parotid and submandibular glands from NOD mice, which were able to generate the aberrantly processed PSP from purified BALB/c protein. Taken together, these data paint a complex picture of the development of Sjögren's syndrome-like disease in the NOD mouse model. The presence of activated lymphocytes appears to be necessary for the ultimate loss of exocrine gland function, potentially through the loss of tolerance to glandular proteins. However, the findings of high levels of apoptosis and aberrant protein expression in the submandibular gland in the absence of an immune response (NOD-scid) suggests that genetic alterations in glandular homeostasis involving the death program contribute to disease progression or even the initial trigger of autoimmunity.
11561032 Spinal cord magnetic resonance imaging demonstrates sensory neuronal involvement and clini 2001 Oct OBJECTIVES: To determine spinal cord MRI findings in neuronopathy associated with Sjögren's syndrome and their correlation with severity of sensory impairment. METHODS: Clinical and electrophysiological features, pathological findings in the sural nerve, and hyperintensity on T2* weighted MRI in the spinal dorsal columns were evaluated in 14 patients with neuronopathy associated with Sjögren's syndrome. RESULTS: Of 14 patients, 12 showed high intensity by T2* weighted MRI in the posterior columns of the cervical cord. High intensity areas were seen in both the fasciculus cuneatus and gracilis in nine patients, who showed severe and widespread sensory deficits in the limbs and trunk; these patients also had a high frequency of autonomic symptoms. Somatosensory evoked potentials often could not be elicited. Hyperintensity restricted to the fasciculus gracilis was seen in three patients, who showed sensory deficits restricted to lower limbs without trunk involvement, or with only partial limb involvement; no autonomic symptoms were noted. The two patients who did not show high intensity areas in the dorsal columns showed restricted sensory involvement in the limbs. All patients showed axonal loss predominantly affecting large fibres, without axonal sprouting. CONCLUSIONS: High intensity areas on T2* weighted MRI in the spinal dorsal columns reflect the degree of sensory neuronal involvement in neuronopathy associated with Sjögren's syndrome; this finding could also be a helpful marker for estimating severity of this neuronopathy.
11128701 Evaluation, differential diagnosis, and treatment of xerostomia. 2000 Dec The salivary component of Sjögren's syndrome (SS) is defined as xerostomia (dry mouth). However, xerostomia is a common symptom associated with quantitative and qualitative changes in saliva, which are generally referred to as salivary hypofunction. This can be caused by various systemic diseases (including SS), anticholinergic effects of many drugs, psychological conditions, and physiological changes. Chronic salivary hypofunction is clinically significant because it can cause oral dysfunction, dental destruction, and mucosal infection. Evaluating patients complaining of xerostomia requires particular attention to their current medications and physical examination of the major salivary glands, teeth, and oral mucosa. Based on that information and the differential diagnosis of salivary hypofunction, appropriate tests can then be selected to develop a final diagnosis. Effective treatment of patients with chronic salivary hypofunction requires a combination of: (1) ongoing dental decay prevention and treatment supervised by their dentist; (2) salivary flow stimulation; (3) recognition and treatment of chronic oral candidiasis; (4) selective use of saliva substitutes; and (5) prescription drug review.