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ID PMID Title PublicationDate abstract
17683708 [Adult onset Still's disease: review of 26 cases]. 2007 Jul 14 BACKGROUND AND OBJECTIVE: We intended to describe the clinical characteristics, treatment and evolution of 26 patients with adult onset Still's disease. PATIENTS AND METHOD: This was a retrospective study (1984-2004). The clinical records of patients with adult onset Still's disease were reviewed. RESULTS: Twenty six patients were included. Most frequent clinical characteristics were: fever (100%), arthritis (81%), rash (92%) sore throat (92%) and lymphadenopathy (42%). Aspirin controlled the disease in 27% of patients, prednisone was needed in 70% and methotrexate was added in 50% cases. A monocyclic course was seen in 54% and polycyclic in 46% patients. CONCLUSIONS: The clinical characteristics were similar to previous series. A febrile polyarthritis was the most frequent presentation form. A polycyclic course was found in 58% of cases and it seems to be associated with poor prognosis and need for aggressive treatment.
17670852 Successful treatment of refractory neuroSjogren with Rituximab. 2007 We report a 47-year-old female with Sjogren's syndrome (SS) and severe weakness in her lower extremities refractory to cyclophosphamide therapy, who was treated with the monoclonal anti CD-20 antibody rituximab at a weekly dose of 375 mg/m2 for four consecutive weeks. Patient responded within few days of the first dose and her motor power in the lower extremities started to improve gradually along with progressive resolution of the paresthesias and dysesthesias. The improvement was sustained and progressive and eight months after the last dose, she was able to walk for 60 meters without aid or rest. Rituximab may be considered as an effective and promising novel therapy in SS patients with neurological involvement.
16365690 A multicenter study of patients with adult-onset Still's disease compared with systemic ju 2006 Sep Adult-onset Still's disease (AOSD) has often been regarded as the adult spectrum of systemic juvenile idiopathic arthritis (sJIA). The present study aims to compare the clinical and laboratory features, the disease course and the response to treatment in patients having AOSD with those having sJIA. Retrospective review of all available data that were filled out by adult and paediatric rheumatologists from six centers using a standard data extraction form was performed. A total of 95 patients with AOSD and 25 patients with sJIA were recruited for the study. The frequency of fever, rash, myalgia, weight loss and sore throat was higher in patients with AOSD. The pattern of joint involvement differed slightly. Laboratory findings were similar in both groups, except that liver dysfunction and neutrophilia were more common among adults. A multiphasic pattern dominated the childhood cases, whereas the most frequent course was a chronic one in adults. Corticosteroids and methotrexate were the most commonly employed therapy; however, chloroquine was another popular therapy in the adult group. We showed a difference in the rate of clinical and laboratory features between patients with AOSD and those with sJIA. AOSD and sJIA may still be the same disease, and children may simply be reacting differently as the result of the first encounter of the putative antigens with the immune system.
16941204 Unusual sites of Salmonella osteoarthritis in patients with sickle cell disease: two cases 2007 Aug Salmonella osteoarticular infections involve mainly long bones such as the femur, tibia, and humerus in patients with sickle cell disease (SCD). We report here two unusual cases of Salmonella osteoarthritis affecting sacroiliac and sternoclavicular joints in two patients with SCD, one patient also being followed for rheumatoid arthritis. Because of misleading presentation, diagnosis of septic osteoarthritis in patients with SCD requires a high index of suspicion and an early treatment.
18984419 Issues related to clinical trials of oral and biologic disease-modifying agents for Sjögr 2008 Nov Published studies and trials of oral and biologic disease-modifying antirheumatic drugs for the treatment of Sjögren's syndrome have shown disappointing results. Improvements in trial design, including development of consortia for the conduct of national and international multicenter studies and use of standardized classification and outcome measures coupled with the emergence of newer biologic, immunomodulatory, and small molecule agents, hopefully will result in the addition of disease-modifying agents to the armamentarium.
18784216 Primary Sjögren Syndrome with tumefactive central nervous system involvement. 2008 Nov Brain MR imaging abnormalities in primary Sjögren syndrome (pSS) are generally discrete white matter lesions. We describe a 50-year-old woman with recurrent neurologic deficits. MR imaging revealed a large brain lesion. A diagnosis of pSS was made on the basis of clinical features, positive anti-Ro and anti-La antibodies, abnormal Schirmer test findings, and salivary gland scintigraphy. The patient was treated with oral prednisone with good response. Large tumefactive brain lesions are a complication of pSS.
17444349 Secondary Sjogren's syndrome and scleroderma presenting as renal tubular acidosis. 2007 Jan We report a case of distal renal tubular acidosis in a twenty year old female patient of scleroderma and secondary Sjogren's syndrome. This patient presented with two episodes of flaccid quadriparesis which were associated with hypokalaemia and was later found to have an underlying scleroderma with secondary Sjogren's syndrome.
17217966 Third cranial nerve palsy? Look for a sicca syndrome. 2007 Feb 15 Sjogren's syndrome (SS) is a systemic autoimmune disorder, and neurological involvement may frequently occur. Here we describe a 79-year-old woman who came to our attention for a sudden right incomplete 3rd cranial nerve palsy. Following extensive investigations, a diagnosis of primary SS was reached, and the patient recovered after treatment with ev Ig and steroids. Therefore, we suggest that SS should be considered in apparently idiopathic 3rd cranial nerve palsies, since, with the appropriate treatment, they might be transient and reversible.
18984412 Mucosa-associated lymphoid tissue lymphoma in Sjögren's syndrome: risks, management, and 2008 Nov Sjögren's syndrome is a chronic inflammatory disease primarily affecting the exocrine glands. Its association with lymphoma is well documented, with salivary extranodal marginal zone lymphomas of the mucosa-associated lymphoid tissue type being the most common and constituting a major disease complication. These neoplasms are antigen-stimulated B-cell lymphomas characterized by localized stage, indolent clinical course, and recurrence in other extranodal sites. This article presents a review of the literature and discusses the clinical, histopathologic, therapeutic, and prognostic aspects of these tumors in Sjögren's syndrome. In addition, it highlights the predictor markers of lymphoma development.
18829369 Diagnostic evaluation and classification criteria in Sjögren's Syndrome. 2009 Jan OBJECTIVES: Our objective is to carry out a clinical study of the performance of the preliminary European classification criteria for Sjögren Syndrome and that of the criteria proposed by the American European Consensus Group. METHODS: Eighty-eight patients who had undergone a biopsy of the salivary gland on suspicion that they were suffering from Sjögren Syndrome were studied by two independent rheumatologists. Two pathologists independently revised the biopsies without knowing the diagnosis. With all of this information, the clinicians established, or did not establish, a diagnosis of primary Sjögren Syndrome or secondary Sjögren Syndrome. Finally, it was analysed whether or not the patients met the American European Consensus Group classification criteria and the preliminary European criteria for primary Sjögren Syndrome and secondary Sjögren Syndrome, and their sensitivity and specificity with respect to the clinical diagnosis were determined. RESULTS: Clinicians estimated that 35 cases (39.8%) had primary Sjögren Syndrome (kappa 0.718) and 17 cases (19.3%) had secondary Sjögren Syndrome (kappa 0.761). The specificity and sensitivity of American European Consensus Group criteria, with regard to the clinical diagnosis, for primary Sjögren Syndrome were 97.2% and 48.6%, respectively. For secondary Sjögren Syndrome, the specificity was 97.2% and the sensitivity 64.7%. The preliminary European criteria for primary Sjögren Syndrome demonstrated a lesser specificity (75%), but a higher sensitivity (65.7%). In secondary Sjögren Syndrome the specificity reached 97.2% with sensitivity at 70.6%. CONCLUSIONS: These results underline the difficulty in applying the Sjögren Syndrome classification criteria from the American European Consensus Group and the preliminary European criteria, in the diagnosis of individual patients.
18460271 Sjögren's syndrome in childhood. 2008 Apr This review presents our 10-year experience with children diagnosed with Sjögren's syndrome (SS). Patients between the ages of 9 and 17 years had abnormalities in laboratory values consistent with but not entirely diagnostic of those required to diagnose SS in adults. The spectrum of clinical manifestations suggests that the SS clinical phenotype in children is more variable than that in adults. Here, we review manifestations of SS in children. Our patients were treated with hydroxychloroquine, despite the lack of prospective data about effects on SS progression and/or autoantibody spreading. Patients have been followed for between 3 and 6 years without substantial progression of their disease or change in autoantibody status. Longer term follow-up (10-20 years) is needed to define the natural history of SS in childhood and its treatment outcomes. Prospective validation of SS criteria in childhood could facilitate assessment of the utility of hydroxychloroquine and other therapies.
18422567 Prevalence of sicca symptoms in a South Australian cohort with systemic sclerosis. 2008 Dec BACKGROUND: The presence of sicca symptoms is a frequent finding in patients with systemic sclerosis (SSc). The aim of this study was to examine the prevalence of sicca symptoms in a South Australian cohort of SSc patients and correlate this to a number of parameters, including autoantibody status, use of anticholinergic medication, age and the presence of functional anti-muscarinic-3 receptor (M3R)-blocking antibodies. METHODS: A screening questionnaire was sent out to all patients on the South Australian Scleroderma Register from the years 1998-2006 to determine the prevalence of sicca symptoms. A subset of patients on the register had ocular sicca symptoms tested by use of Schirmer's strips to validate the accuracy of the questionnaire. Eight patients were tested for anti-M3R-blocking antibodies using a functional physiological assay. RESULTS: One hundred and ninety-three SSc patients took part in this study. Sicca symptoms were present in 59% of patients with the limited form of SSc, compared with 49% of patients with the diffuse form and 40% of patients with the overlap syndrome. The use of anticholinergic medication or thyroxine was associated with higher sicca scores in SSc patients. SS-A and SS-B autoantibodies (seen in Sjögren's syndrome) were detected in eight patients in this study. The detection of anti-M3R-blocking antibodies correlated well to presence of sicca. CONCLUSION: This study confirmed that sicca symptoms are found in a high proportion of patients with SSc, especially those with the limited variant. Further testing of larger numbers of SSc patients with sicca for anti-M3R-blocking antibodies will be needed before more definitive conclusions can be drawn. Physicians should be made aware that sicca symptoms are a frequent cause of morbidity for SSc patients*.
17558463 Thyroid disease in Sjögren's syndrome. 2007 Oct From 1960 to 2007, an important number of patients with primary Sjögren's syndrome (pSS) along with thyroid disease diagnosed by laboratory data and clinical presentation were reported. The most common thyroid disorder found was autoimmune thyroiditis and the most common hormonal pattern was subclinical hypothyroidism. The coexistence of SS and thyroiditis is frequent and suggests a common genetic or environmental factor predisposition with similar pathogenic mechanisms. pSS was ten times more frequent in patients with autoimmune thyroid disease and autoimmune thyroiditis was nine times more frequent in pSS. Therefore, SS should be studied in patients with thyroid disease and vice versa. Antigens are shared by both thyroid and salivary glands, which could be responsible for the association between both diseases. Immunogenetic studies had suggested that both diseases have a common genetic predisposition. pSS and thyroid disease patients were mostly women with positive antithyroglobulin, antiparietal cell and antithyroid peroxidase antibodies. Thyroid dysfunction is frequent in pSS patients and those prone to develop thyroid disorders are identified by thyroid-related autoantibodies or by rheumatoid factor and anti-Ro/SSA activity. Patients with pSS have an increased tendency to develop other autoimmune diseases. Hypothyroidism was the most common autoimmune disease developed in pSS patients during follow-up of 10.5 years. Lymphomas are also associated with SS and thyroiditis and a 67-fold increased risk for thyroid mucosa-associated lymphoid tissue (MALT) lymphoma and a 44-fold increased risk for parotid lymphoma is being attributed to autoimmune thyroiditis and pSS. It is suggested that immune mechanism deficiency is a causal factor for B cell lymphoma in pSS and autoimmune thyroid disease. Other studies are necessary to clarify the shared pathogenesis mechanism in SS and autoimmune thyroid disease and to understand this fascinating autoimmune association.
16900301 Purtscher's-like retinopathy as an initial presentation of adult-onset Still's disease: a 2007 Jul Adult-onset Still's disease is a multisystem inflammatory disorder of unknown etiology and is characterized by high, spiking fever, arthritis, evanescent maculopapular rash, myalgia, serositis, leukocytosis, and involvement of various organs including the eyes. The ocular manifestations have been described including orbital pseudotumor, ptosis, and diplopia with orbital pain but never Purtscher's-like retinopathy. We describe a 21-year-old male patient with adult-onset Still's disease who developed the Purtscher's-like retinopathy. To our knowledge, this is the first reported adult-onset Still's disease patient with Purtscher's-like retinopathy as the initial presentation.
18067414 Proteomic diagnosis of Sjögren's syndrome. 2007 Dec In the last few years, a growing interest has arisen in the application of proteomic analysis to rheumatic disease. Sjögren's syndrome is a systemic disease that affects exocrine glands directly, and is therefore expected to influence the composition of the whole human saliva and lachrymal fluid. Therefore, a rising number of studies have been performed in an attempt to characterize the salivary and lachrymal protein profiles of patients with Sjögren's syndrome by using a proteomic approach. This review summarizes the state of the art and the potential application of proteomics in the systematic search for diagnostic biomarkers in Sjögren's syndrome.
17533107 Noninvasive interference tear meniscometry in dry eye patients with Sjögren syndrome. 2007 Aug PURPOSE: To compare noninvasive tear meniscus height (NI-TMH) using a tear interference device in normal subjects and dry eye patients with Sjögren syndrome (SS), and to investigate the applicability of this new method before and after the punctal occlusion procedure. DESIGN: Prospective case control study. METHODS: Tear meniscus was visualized noninvasively using a tear interference device (Tearscope plus, Keeler, Windsor, United Kingdom). Tear interference image was captured with digital video camera (SP-321, JFC Sales Plan Co, Tokyo, Japan) attached to the slit-lamp. Lower lid margin NI-TMH was measured using image analysis software. NI-TMH of 28 eyes from 17 normal subjects and 46 eyes from 27 aqueous tear deficiency (ATD) dry eye patients with SS were compared. The change of NI-TMH three weeks after the successful punctal occlusion was examined in 11 eyes of eight dry eye subjects. RESULTS: Tear meniscus was well visualized with the tear interference device in all cases. Lower lid margin NI-TMH was 0.22 +/- 0.065 mm in normal subjects, and 0.13 +/- 0.042 mm in SS subjects, respectively (P < .0001). After the punctal occlusion, lower lid margin NI-TMH increased significantly from 0.12 +/- 0.026 mm to 0.42 +/- 0.21 mm (P = .001). CONCLUSIONS: NI-TMH was substantially lower in SS subjects and also significantly improved after punctal occlusion. This method is expected to be helpful in the diagnosis and in the evaluation of the efficacy of punctal occlusion in ATD dry eyes such as SS.
17458808 Bilateral sequential optic neuropathy as the initial manifestation of Sjögren syndrome. 2007 Apr BACKGROUND: Neuro-ophthalmic findings are uncommon in the setting of Sjögren syndrome. We report the case of a patient with bilateral, sequential optic neuropathy as the initial manifestation of Sjögren syndrome. HISTORY AND SIGNS: A 38-year-old male presented with sudden painless visual loss in his left eye in May 2005. Fundus examination revealed a left swollen optic disk. Magnetic resonance imaging (MRI) revealed a left optic nerve lesion. Elevated titres of autoantibodies (ANA, anti-SSA, anti-SSB) were found, suggestive of Sjögren syndrome. In January 2006, he presented with painful sudden visual loss in the right eye. Fundus examination revealed a right swollen optic disk and left optic nerve atrophy. MRI was normal. Other aetiologies were ruled out. THERAPY AND OUTCOME: Each episode was treated with intravenous methylprednisolone (1 g/day during 3 days), followed by oral prednisone (1 mg/kg/day). Moderate improvement of vision ensued in both eyes. CONCLUSIONS: Atypical presentation of an optic neuropathy must raise the suspicion of an unusual aetiology. Our case illustrates how a bilateral sequential optic neuropathy in an otherwise healthy patient can result from an unusual inflammatory aetiology: primary Sjögren syndrome.
17110311 Immunohistopathology of Sjögren's syndrome. 2006 Nov Sjögren's syndrome (SS) is characterized by keratoconjunctivitis sicca and xerostomia, which occur in an autoimmune lacrimal and salivary gland disease characterized by lymphocyte infiltrates of exocrine glands and/or Sjögren's syndrome autoantibody production. It has been reported that aquaporin-5 distribution is abnormal in SS, perhaps as a result of paracrine effect of TNF-alpha. Also the neurogenic regulation of the salivary gland is impaired in SS. Apart from functional changes, the syndrome is also characterized by structural abnormalities of the secretory acinar apparatus. The acinar basement membrane is abnormal as it lacks laminin alpha1 chain, which may impair its capability to induce the progenitor cells to differentiate to acinar cells. CRISP-3 and TMPRSS-2 can be used as androgen markers and LIV-1 and Cyr61 as estrogen markers to study the sexual dimorphism of the salivary glands. Patients with SS seem to have low concentrations of dehydroepiandrosterone, which may predispose women and the exocrine glands to this syndrome.
16163443 Corticosteroid injections reduce size of rheumatoid nodules. 2006 Feb BACKGROUND: Symptomatic rheumatoid nodules are frequently surgically treated. Injection with steroids might be an alternative treatment. PATIENTS AND METHODS: To determine whether injection with triamcinolon acetonide reduces the size of rheumatoid nodules, we randomized twenty patients with symptomatic nodules to either triamcinolon acetonide 40 mg/ml plus lidocaine 2% or lidocaine 1% (placebo). We measured the nodules before injection and 2, 4, 8, and 12 weeks after injection. Possible side effects were recorded. RESULTS: We found that the volume of the nodules injected with triamcinolon acetonide reduced significantly (p = 0.011), from 130 to 8 mm(3) (median calculated size) at 12 weeks, compared with baseline. Furthermore, at 12 weeks, the difference between the groups was significant (p = 0.03). The median size of the placebo nodules diminished as well, from 358 to 237 mm(3), but this was not significant. Pain at injection was the only side effect, equally distributed in both treatment groups. CONCLUSION: Injection with triamcinolon acetonide seems to be an alternative for surgery of rheumatoid nodules. No adverse events occurred but the limited sample does not allow definitive conclusions.
18592135 Steroid-refractory severe hepatic failure in adult onset Still's disease responding to cyc 2008 Nov We report two Japanese women with severe hepatic dysfunction and adult onset Still's disease. A 51-year-old woman had been diagnosed with adult onset Still's disease 3 years earlier. She relapsed while on maintenance therapy with prednisolone and methotrexate. After induction of remission with methylprednisolone pulse therapy, indomethacin, and methotrexate, severe hepatic failure occurred. This patient lacked the typical symptoms of adult onset Still's disease. The second patient was a 32-year-old woman with typical adult onset Still's disease. Remission was induced by high-dose prednisolone and methylprednisolone pulse therapy plus cyclosporine. After she stopped cyclosporine, severe liver dysfunction occurred. In both patients, liver dysfunction occurred during high-dose steroid therapy, and oral cyclosporine (3 mg/kg per day) dramatically improved their liver function. When steroid-resistant severe hepatic failure occurs in patients with adult onset Still's disease, cyclosporine may be the immunosuppressant of choice.