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ID PMID Title PublicationDate abstract
16164723 Lupus erythematosus associated with erythema multiforme: report of two cases and review of 2005 Sep Rowell's syndrome (RS) is a rare presentation of lupus erythematosus (LE) with erythema multiforme-like lesions associated with antinuclear, anti-La (SS-B)/anti-Ro (SS-A) antibodies and rheumatoid factor (RF) positivity. This syndrome is suggested to be a different variant of cutaneous lupus erythematosus by some authors in literature. Here we present a 64-year-old woman with LE and a 51-year-old woman with LE and Sjögren syndrome (SS) who had erythema multiforme-like eruptions and discuss the coexistence of lupus erythematosus and erythema multiforme.
16697772 Characterization of peripheral natural killer cells in primary Sjögren's syndrome: impair 2006 May The aim of this study was to compare the number of peripheral blood natural killer (NK) cells, NK cell activity, expression of NK cell activating receptors, and serum cytokine levels in patients with primary Sjögren's syndrome (SS) vs normal controls. The authors found that NK cell number, NK cell killing activity, and the expression of activating receptors CD2 and NKG2D were significantly decreased, and the expression of NKp46, as well as the percentage of apoptotic NK cells, were significantly increased in primary SS patients compared with healthy controls. NK cell killing activity on a per-cell basis was similar in primary SS patients and healthy controls. Moreover, the levels of IL-18 and TNF-alpha, cytokines that have been shown to promote NK cell death, were significantly increased in sera from patients with primary SS compared with controls. These data suggest that reduced NK cell numbers, probably a result of apoptotic death, may contribute to impaired NK cell activity in patients with primary SS.
16512445 [MALT-lymphomas in Sjogren's disease]. 2006 AIM: To develop algorithm of early diagnosis of extranodal lymphoma arising in patients with Sjorgen's disease (SD). MATERIAL AND METHODS: SD diagnosis was made in 457 patients treated in Rheumatology Institute clinic in 1999-2004, 38 (8.3%) females aged 19-82 had lymphoproliferative diseases. MALT-lymphomas were diagnosed in 15 (42.2%) patients. All the patients have undergone morphological, immunomorphological investigations of the salivary glands, postoperative material was analysed in some patients. In addition, the following investigations were made: ultrasonography of the salivary glands, lymph nodes, viscera; scintigraphy; trephine biopsy of the bone marrow; myelograms; CT of the chest, abdomena and brain; tests for monoclonal immunoglobulins in the serum and light chains in urine; biopsy of the parotid gland. Clinical, morphological and immunophenotypical characteristics of MALT-lymphomas were assessed by WHO classification. Lymphoma stages were classified according to Ann Arbor. RESULTS: Parotid glands were affected with MALT-lymphoma most frequently. Predominant were extranodal lymphomas of the parotid submandibular, minor salivary glands of the lip and lacrimal glands of stage I E-II E. Extranodal lymphoma with nodal lesion of stage IV occurred less frequently. Untreated long existing MALT-lymphomas of the parotid glands may transform into B-large cell lymphomas deteriorating SD prognosis. The presence of long-term (> 12 months) massive enlargement of parotid/submandibular salivary and lacrimal glands, massive infiltration, monoclonal immunoglobulins in blood serum and their light chains in the urine predict development of MALT-lymphoma in SD. CONCLUSION: In SD, MALT-lymphomas develop primarily in target organs--salivary and lacrimal glands. SD patients with persistent enlargement of the parotid glands need biopsy for early detection of malignant lymphoproliferation.
15708886 Lamivudine is not effective in primary Sjögren's syndrome. 2005 Sep BACKGROUND: Retroviral infection has been implicated in the pathogenesis of primary Sjögren's syndrome. OBJECTIVE: To examine the efficacy of the reverse transcriptase inhibitor lamivudine in patients with this syndrome. METHODS: 16 patients with primary Sjögren's syndrome were randomised to receive either lamivudine 150 mg twice daily or placebo for three months. Measures of lacrimal and salivary function, including minor salivary gland biopsies, were obtained before and after treatment. RESULTS: Treatment with lamivudine did not result in significant improvement in the primary outcome measure of unstimulated whole salivary flow or other secondary measures, including minor salivary gland biopsy focus scores. CONCLUSION: Lamivudine is not effective in patients with primary Sjögren's syndrome, suggesting either that a retroviral aetiology is not present or that it may be important only in early disease.
16886810 [A case of primary Sjögren' s syndrome with pathological findings resembling multicentric 2006 Jul A 49-year-old woman with primary Sjögren's syndrome a few years previously was admitted to our hospital complaining of tongue and skin eruptions, swelling of the face and neck and for examination of liver injury and hypereosinophilia. A blood test revealed leukocytosis with eosinophilia, mild liver injury, polyclonal hypergammaglobulinemia, and positive results for anti-nuclear antibody, anti-SS-A antibody and anti-SS-B antibody. Chest CT scan showed multiple nodular opacities with cavities in peripheral regions of both lungs. Biopsy specimens from the right lower lobe obtained by video-assisted thoracoscopy revealed marked infiltration of plasma cells and lymphocytes in alveolar lumina, lymph follicles with germinal centers in other areas of the pulmonary parenchyma, and lymphocytes infiltrate in alveolar wall adjacent bronchi and bronchioles. The histological diagnosis was pulmonary involvement of multicentric Castleman's disease. This was a rare case of Sjögren's syndrome accompanied by pathological findings of multicentric Castleman' s disease showed multiple nodular opacities in chest CT scans.
16831312 Sjogren's syndrome in a celiac patient: searching for environmental triggers. 2006 Apr In the last few years many studies have shown the potential role of different triggers in the pathogenesis of several autoimmune diseases. In particular, in Sjogren's syndrome the presence of a genetic background is considered determining, but environmental factors have recently been described as triggers or precipitators. In this report, we describe the case of a young woman affected by celiac disease in which an Ascaris lumbricoides infestation and estrogen therapy could have played a role in the development of Sjogren's syndrome.
16460489 Rheumatoid-like nodules in the spleen: new extraintestinal manifestation of Crohn's diseas 2006 Jan A 26-year-old, HLA-B27 positive man with steroid dependent Crohn's disease presented with abdominal lymphadenopathy and multiple abscess-like masses in the spleen and liver 2 years after the initial diagnosis. A biopsy of one of the liver lesions showed epitheloid cell granuloma, no microorganisms were detected despite intensive investigations. The liver lesions and the lymphadenopathy repeatedly disappeared completely in response to prednisolone pulse therapy while the lesions in the spleen remained practically unchanged. One year later, on immunosuppressive therapy with azathioprine and low-dose prednisolone, the patient's condition exacerbated with development of fever, disseminated arthritides and enlargement of the splenic lesions. Following ineffective antimicrobial treatment, a splenectomy was carried out. Histology showed that the splenic parenchyma was filled with abacterial, rheumatoid granulomas. After a renewed flare-up of severe extraintestinal symptoms, the patient is now asymptomatic on immunosuppressive therapy with azathioprine and prednisolone. Abdominal lymphadenopathy is no longer present and the liver lesions are regressing. The intermittent course and the positive response to immunosuppressive therapy point to a further extraintestinal manifestation of Crohn's disease.
15877483 Geriatric autoimmune diseases: systemic lupus erythematosus, Sjogren's syndrome, and myosi 2005 May Systemic lupus erythematosus (SLE), Sjogren's syndrome (SS), and dermatomyositis (DM)/polymyositis (PM) may be encountered in geriatric patients due to improved survival rates in patients with younger ages of onset or from elderly-onset (EO) disease. EO disease accounts for up to 20% of patients affected by these disorders, and is typically insidious rather than acute. Whereas SS and DM/PM are considered autoimmune diseases with distinct organ specificity, SLE is a systemic disorder that may affect multiple organ systems. Commonly used clinical and laboratory criteria for defining and diagnosing these diseases were largely developed for patients age <65, and need to be modified in the geriatric patient. Therapeutic strategies include attention to ongoing drug regimens, medical comorbidities, and the roles of fatigue, depression, and arthropathy. Each disease may be responsive to low-dose corticosteroids, with a role for first or second-line immunosuppressives as steroid-sparing agents.
17265991 57-year-old Asian-American man with Kikuchi's disease. 2006 Nov Kikuchi's Disease (KD) is a subacute necrotizing lymphadenitis more commonly reported from Asia. The classic presentation includes low-grade fever and cervical lymphadenopathy in a previously healthy woman. The ratio of affected women to men is 4:1 with the average age of onset less than 30 years. We report a case of KD in a 57-year-old Asian-American man whose presentation is associated with Adult Still's Disease.
17062438 The prevalence of Sjögren's syndrome in adult women. 2006 Sep OBJECTIVES: The aim of this study was to determine the prevalence of primary Sjögren's syndrome (pSS) according to European criteria (1993) and to the US-European Consensus Group (US-EU) criteria (2002) in adult women in Bornova, Izmir, Turkey. MATERIALS AND METHOD: The study was designed as a two-phase cross-sectional survey consisting of a baseline questionnaire and collection of blood samples and clinical examination. In the initial phase, positivity for autoantibodies Ro(SS-A), La(SS-B), rheumatoid factor (RF), and anti-nuclear antibodies (ANA) was determined, and in the clinical phase, clinical examination, salivary and ocular tests were performed. Minor salivary gland biopsy was performed for those who had at least three of these five criteria positive. RESULTS: In our sample the prevalence of SS was 1.56% [95% confidence interval (CI) 0.92-2.66] according to the European criteria and 0.72% (95% CI 0.33-1.57) according to the US-EU criteria. CONCLUSION: To prevent the loss in diagnosis of pSS, the addition of ANA, RF, and tear break-up time (BUT) tests to US-EU criteria would be appropriate.
17149056 Response to application of ice may help differentiate between gouty arthritis and other in 2006 Dec AIM: The aim is to determine whether response to topical ice versus heat differentiates between patients with gout versus other arthritides. METHODS: The first 150 patients seen in our clinic with joint pain from February 2004 onward were asked to fill out questionnaires regarding their response to heat and ice. Patients who responded that topical ice eased their pain and who did not have a diagnosis of crystal-induced arthritis were asked to have a joint aspiration if they had active synovitis on presentation to the clinic. RESULTS: Of 150 completed questionnaires, 26 patients never tried heat or cold as adjuvant treatment for their arthritis. The remaining 124 patients were divided into 6 groups: patients with crystal-proven gout (n = 20), rheumatoid arthritis (RA; n = 32), osteoarthritis (OA; n = 32), other forms of inflammatory arthritis (n = 18), and soft tissue conditions (n = 22). None of the patients with gout benefited from topical heating of their affected joints and all preferred topical ice (P < 0.001). Most patients with RA preferred heat (n = 24). Of 4 patients with RA who preferred topical ice, 3 had effusions and arthrocentesis was performed. Intracellular monosodium urate (MSU) crystals were seen in 2 and intracellular calcium pyrophosphate dihydrate (CPPD) crystals were seen in one patient raising questions about coexistence of 2 diseases or previous misdiagnoses. Most patients with OA preferred heat (n = 28). A significantly higher percentage of the patients with gouty arthritis found that topical ice helped relieve their joint pain as compared with patients with RA (P = 8 x 10(-11)) and other inflammatory arthritides (P = 3 x 10(-8)). DISCUSSION: Heat and cold are adjuvant treatments for arthritis. In gouty arthritis, cold applications are a useful adjunct to treatment and may help discriminate patients with gout from other forms of inflammatory arthritis.
16078341 Primary Sjögren's syndrome in dizygotic adolescent twins: one case with lymphocytic inter 2005 Aug Primary Sjögren's syndrome (pSS) is uncommonly recognized in childhood, and familial cases are rare. Pulmonary involvement in pediatric pSS is infrequently reported. In adults, asymptomatic pulmonary involvement is increasingly recognized, manifest by pulmonary function test abnormalities and changes on high resolution computerized tomographic scan. We describe a case of pSS in a 14-year-old Vietnamese-Canadian girl who presented with pulmonary symptoms, radiologic changes, and biopsy confirmation of lymphocytic interstitial pneumonia. Her dizygotic twin sister has primary SS without extraglandular manifestations. To our knowledge this is the first report of pediatric pSS with lymphocytic interstitial pneumonia and multiple pulmonary nodules on chest radiograph. We review the literature on pulmonary involvement and familial cases of pSS in childhood.
17628907 [Pulmonary arterial hypertension revealing pseudolymphoma associated with Sjogren's syndro 2006 INTRODUCTION: The authors related a right heart failure related to a primitive pulmonary arterial hypertension without past medical. METHODS AND RESULTS: This diagnosis has been noticed on the cardiac catheterism as well as the cardiac echography (no shunt). Two years later systemic anomalies such as polyarthritis and salivary glands disease were noticed. The Biopsy revelated a primitive Gougerot-Sjorgen pseudolymphomas syndrom. CONCLUSION: Three years under conventional treatment were a failure as the evolution of the disease resulted to death.
17188404 [Painful thoracic neuropathy disclosing Sjögren's syndrome: first report]. 2007 Mar INTRODUCTION: Thoracic neuropathy is rare, and is usually associated with diabetes mellitus. We report a first case of isolated multi-metameric thoracic neuropathy revealing Sjögren's disease. EXEGESIS: A 64-year old man consulted for symptoms suggesting a progressive and extensive (from T7 to T10) bilateral thoracic neuropathy. Diabetes mellitus and other causes of neuropathy were excluded. Spinal MRI, electromyography and CSF analysis were normal. Though the patient had no sicca syndrome symptoms, the diagnosis of Sjögren's syndrome was made on hypergammaglobulinemia, elevated ACAN (1/5000) with anti-SSA specificity and a grade III minor salivary gland biopsy. He improved spectacularly on corticosteroids (prednisone, 1 mg/kg/d). Sicca syndrome became clinically evident in April 2006. CONCLUSION: Pure sensitive thoracic neuropathy can reveal Sjögren's syndrome.
16614610 [Anti-TNF alpha in the treatment of psoriatic arthritis]. 2006 Apr Psoriatic arthritis is an inflammatory and possibly destructive form of arthritis. As in rheumatoid arthritis and ankylosing spondylitis, the use of biological therapy in psoriatic arthritis is a therapeutic revolution: both articular and cutaneous efficacy have been shown, and some improvement is visible on radiography. The benefit-risk ratio will improve when we learn to identify more accurately the patients likely to benefit from these treatments.
17056149 Current status of gene delivery and gene therapy in lacrimal gland using viral vectors. 2006 Nov 15 Gene delivery is one of the biggest challenges in the field of gene therapy. It involves the efficient transfer of transgenes into somatic cells for therapeutic purposes. A few major drawbacks in gene delivery include inefficient gene transfer and lack of sustained transgene expression. However, the classical method of using viral vectors for gene transfer has circumvented some of these issues. Several kinds of viruses, including retrovirus, adenovirus, adeno-associated virus, and herpes simplex virus, have been manipulated for use in gene transfer and gene therapy applications. The transfer of genetic material into lacrimal epithelial cells and tissues, both in vitro and in vivo, has been critical for the study of tear secretory mechanisms and autoimmunity of the lacrimal gland. These studies will help in the development of therapeutic interventions for autoimmune disorders such as Sjögren's syndrome and dry eye syndromes which are associated with lacrimal dysfunction. These studies are also critical for future endeavors which utilize the lacrimal gland as a reservoir for the production of therapeutic factors which can be released in tears, providing treatment for diseases of the cornea and posterior segment. This review will discuss the developments related to gene delivery and gene therapy in the lacrimal gland using several viral vector systems.
16476272 [Nodular primary cutaneous amyloidosis associated with Sjögrens syndrome: presentation of 2005 Sep Nodular primary cutaneous amyloidosis is the least frequent clinical form of the cutaneous amyloidoses. It may be associated with myeloproliferative disorders, as well as with systemic amyloidosis. Its association with other entities, such as Sjögren's syndrome, has recently been described. We present the case of a female patient with Sjögren's syndrome who developed nodular primary cutaneous amyloidosis.
17135704 Preliminary dielectric studies of knee swelling fluid in the case of arthritis, which is h 2006 Dec This study addresses the problems connected with knee joint inflammatory swelling diagnosis, especially cases which have difficulty meeting rheumatic and laboratory criteria. A percentage of patients were considered who met the clinical criteria of the Association of Rheumatoid Arthritis (ARA), proceeded without any deviations within the range of rheumatoid factor (RF) laboratory results, and where only serum non-specific immuno-complexes (CIC) increased. The decision to execute a more effective treatment variant (salazopirin, for example, instead of NSAID), required criterial reliability in practice. The purpose of the study was to attempt to find physiochemical distinguishing features in the exudative liquid taken from the knee joints of two groups of patients: group A meeting the clinical criteria of immunological inflammation and a negative rheumatoid factor, and group B with other inflammation concerned with degenerative changes and with negative rheumatoid factor tests. The main area of interest was the question of whether dielectric scanning enables the distinction between immunology and degenerative inflammatory liquid. Analyses of dielectric joint liquid parameters in the domains of frequency and temperature made it possible to observe recurring changes in the shapes of diagrams in the event of the existence of immunological complexes in the studied environment. We found that (1) dielectric scanning diagrams after joint liquid analyses showed similar negative dielectric parameters in group A (inflammatory), B (degenerative) and C (post-accident) with scanning frequencies of about 10,000,000-3501,300 Hz and similar positive parameters of about 3501,300-1593,700 Hz, but qualitative difference dielectric parameters in group A with a frequency of 1593,700-1225,900 Hz, probably dependent on the inflammatory gamma-globulin concentration; (2) higher dielectric parameters (groups B, C) at a scanning frequency of 94,300-57,600 Hz probably dependent on ion concentration; (3) parametric changes of frequency scanning differentiating the diagrams between groups A and B, C, defining the outline pilot study recommended ranges of frequency and temperature testing for further investigations.
16697230 Matrix metalloproteinase-9 (gelatinase B) deficiency leads to increased severity of Staphy 2006 May Matrix metalloproteinases constitute a family of structurally related endopeptidases that are crucial for the normal turnover of the extracellular matrix. Elevated levels of MMP-9 have been demonstrated in synovial fluids of rheumatoid arthritis patients, and a correlation with the severity of the disease has been described. The aim of this study was to explore the impact of MMP-9 expression on joint inflammation and destruction in a model of bacterially induced septic arthritis. MMP-9 knock-out mice and C57Bl6 congenic controls were inoculated intravenously or intra-articularly with Staphylococcus aureus. Arthritis was evaluated clinically and by means of histology. Zymographic analyses were performed to study ex vivo induction of MMP-9 following exposure to S. aureus. The MMP-9 knock-out mice displayed a significantly higher frequency and severity, but not destructivity, of arthritis than did the wild-type mice. The knock-out mice also proved to harbour an increased number of bacteria locally in joints and systemically in kidneys, possibly by impaired extravasation and recruitment of leukocytes and a deficient early defence against infection. Our findings indicate that deficiency in MMP-9 increases the degree of joint inflammation due to decreased bacterial clearance.
15708937 Psoriatic arthritis assessment tools in clinical trials. 2005 Mar In order to measure disease activity, progression, and change with therapy in psoriatic arthritis (PsA), it is important to use accurate, reliable, and feasible outcome measures that can ideally be employed in longitudinal cohorts, clinical trials, and clinical practice. Until recently, there has been little focus on this methodology in PsA. Clinical trials and long term clinical registries have used disparate outcome measures. With emerging therapies, the focus on the methodology of outcome assessment has increased to ensure that discriminant and responsive instruments are used. The Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA), in conjunction with the society, Outcome Measures in Rheumatology (OMERACT), is focused on refining and developing outcome measures for a variety of disease domains reviewed in this report. Key domains to assess include joints, skin, enthesitis, dactylitis, spine, joint damage as assessed radiologically, quality of life, and function. These domains can be assessed by individual and composite measures. A number of measures have been "borrowed" from the fields of rheumatoid arthritis, ankylosing spondylitis, and psoriasis and adapted to PsA. Others are being developed specifically for PsA. Few are validated but most have been shown to perform well in distinguishing placebo from treatment response. This report reviews the current state of the art of assessment in PsA and points toward future directions of development of this field.