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ID PMID Title PublicationDate abstract
24327971 Acral gangrene as a presentation of non-uremic calciphylaxis. 2013 Oct We are describing a case of 55-year-old obese female with significant history of uncontrolled rheumatoid arthritis, who recently had decreased her immune-suppression medications. She presented with extensive acral gangrene involving multiple fingers and toes. Clinical picture and laboratory findings were suggestive of vasculitis; however, skin biopsy established diagnosis of calciphylaxis, in settings of normal kidney function. Patient was treated with sodium thiosulfate with gradual improvement in her skin lesions.
24318567 Septic arthritis: patients with or without isolated infectious agents have similar charact 2014 Apr PURPOSE: Septic arthritis can be disabling and life-threatening, requiring prompt diagnosis and treatment. The infectious agent is not always identified in these patients. We revaluate septic arthritis cases discharged from our department, describing the affected population, causative microorganisms and antibiotic therapy used, and characterised differences between patients with and without isolated pathogenic agents. METHODS: Sixty-eight septic arthritis patients were included in this study. Diagnosis was based on clinical findings, and/or the presence of joint purulent material, and/or bacterial pathogen isolation from joint fluid/synovial membrane/blood cultures and response to antibiotics. Data analysis was performed using SPSS 20. RESULTS: Patients had a mean age of 61.1 ± 18.8 years, without sex predominance. 26.5 % had an infection ≤ 15 days before septic arthritis diagnosis. Besides previous infection, 57.4 % had ≥ 1 risk factors for septic arthritis, most commonly pharmacological immunosuppression (20.6 %), diabetes mellitus type 2 (19.1 %) and rheumatoid arthritis (17.6 %). The knee was the most often affected (54.3 %). Only 39.7 % presented fever from clinical onset until hospital admission (mean 13.4 ± 18.9 days). Leucocytosis was present in 45.6 % of patients, elevated erythrocyte sedimentation rate (ESR) in 75 % and elevated C-reactive protein (CRP) in 97.1 %. 5.9 % had articular damage attributable to septic arthritis. An infectious agent was isolated in 41.2 % of patients, with Staphylococcus aureus being the most frequent. 38.7 % of synovial fluid and 23.5 % of synovial membrane cultures were positive. Patients with an identified infectious agent have no significant differences other than more days of hospitalisation (p = 0.003) and in-hospital antibiotic treatment (p = 0.017). CONCLUSION: Synovial fluid and synovial membrane cultures more often identified pathogens compared to blood or urine cultures. Patients with and without an identified infectious agent have similar demographic, clinical, laboratory and radiographic characteristics.
27708906 A successful treatment of juvenile idiopathic arthritis with rituximab: A report of two ca 2014 Dec Juvenile idiopathic arthritis (JIA) is defined as arthritis of unknown cause that starts before 16 years of age and lasts at least 6 weeks. It is the most common chronic inflammatory disease in childhood and often persists through adulthood and can lead to severe disability. Biologics are an important therapeutic option for treating patients with JIA. The efficiency of rituximab has not been proven for this indication. Its use has rarely been reported in the literature. We report two new cases of severe and refractory polyarticular JIA with positive rheumatoid factor affecting two African females aged 17 and 18 years successfully treated with rituximab. According to our experience, the use of rituximab in the treatment of JIA, especially in severe polyarticular forms with positive rheumatoid factor, might be a good alternative. Larger therapeutic trials should be conducted in this direction in order to prove the effectiveness of this biotherapy for this indication.
23527707 Kinetics and interplay of mediators of inflammation-induced bone damage in the course of a 2013 Jan Rheumatoid arthritis (RA) is an autoimmune disease characterized by chronic inflammation, bone erosion, and cartilage destruction in the joints. It is increasingly being realized that inflammation might play an important role in inducing bone damage in arthritis. However, there is limited validation of this concept in vivo in well-controlled experimental conditions. We addressed this issue using the adjuvant arthritis (AA) model of RA. In AA, the draining lymph nodes are the main sites of activation of pathogenic leukocytes, which then migrate into the joints leading to the induction of arthritis. We tested the temporal kinetics of mediators of bone damage [e.g., receptor activator of nuclear factor kappa-B ligand (RANKL), osteoprotegerin (OPG) and osteopontin (OPN)] and inflammation (pro-inflammatory cytokines and chemokines) in the draining lymph node cells (LNC) at different phases of AA, and then examined their inter-relationships. Our study revealed that, together with cytokines/chemokines, some of the mediators of bone remodeling are also produced in LNC. Various cytokines/chemokines showed distinct kinetics of expression as well as patterns of correlation with mediators of bone remodeling at different phases of the disease. Pro-inflammatory cytokines such as TNF-alpha are known to play an important role in bone damage. Interestingly, there was a positive correlation between TNF-alpha and RANKL, between RANKL and each of the 3 chemokines tested (RANTES, MIP-1alpha, and GRO/KC), and between TNF-alpha and RANTES. Our results in the AA model lend support to the concept of osteo-immune crosstalk during the course of autoimmune arthritis.
24126079 Research gaps in psoriasis: opportunities for future studies. 2014 Jan Over the past 2 decades, considerable progress has been made to further elucidate the complex pathogenesis of psoriasis, facilitating the development of a new armamentarium of more effective, targeted therapies. Despite these important advances, substantial deficits remain in our understanding of psoriasis and its treatment, necessitating further research in many areas. In the sixth section of the American Academy of Dermatology Psoriasis Guidelines of Care, gaps in research and care were identified. We discuss the most important gaps in research that currently exist and make suggestions for studies that should be performed to address these deficits. These encompass both basic science and clinical research studies, including large, prospective epidemiologic studies to determine the true prevalence and natural history of psoriasis; further molecular studies in patients with psoriatic and psoriatic arthritis to understand the function of psoriasis susceptibility genes and to identify novel therapeutic targets; studies to examine the role of environmental factors in the development of psoriasis; further investigation of the relationship between psoriasis and cardiometabolic disease; studies that examine the role of adjunctive therapies such as psychological interventions in appropriate patient groups; and finally, studies to identify biomarkers of disease severity and treatment response to optimize patient therapy.
25362775 [Interstitial lung disease in connective tissue disorders]. 2014 Sep After immunosuppressive-induced infections, interstitial lung disease (ILD) is one of the most serious pulmonary complications associated with connective tissue diseases (CTD). Although it is common for ILD to be diagnosed concurrent with or after CTD, some patients will present with ILD years prior to receiving a diagnosis of CTD. The clinical approach involves an examination of the extrathoracic symptoms (suggestive of CTD) and the evaluation of respiratory disability. Nonspecific interstitial pneumonia is the most common histological finding in patients with CTD. The management of patients with CTD-associated ILD is optimized by multidisciplinary collaboration. ILD-CTD are treated through anti-inflammatory medication, immunosuppressants and biological agents.
25350278 Association between Sjogren's syndrome and respiratory failure: put airway, interstitia, a 2014 OBJECTIVES: Few studies have evaluated the association between Sjogren's syndrome (SS) and respiratory failure (RF). Thus, we conducted a retrospective national cohort study to investigate whether Sjogren's syndrome (SS) increases the risk of respiratory failure (RF). METHODS: The cohort consisted of 4954 newly diagnosed patients with SS but without a previous diagnosis of RF, and 19816 patients as the comparison cohort from the catastrophic illnesses registry, obtained from the 2000-2005 period. All of the study participants were followed from the index date to December 31, 2011. We analyzed the association between the risk of RF and SS by using a Cox proportional hazards regression model, controlling for sex, age, and comorbidities. RESULTS: The overall incidence rate of RF showed a 3.21-fold increase in the SS cohort compared with the comparison cohort. The adjusted HR of RF was 3.04 for the SS cohort compared with the comparison cohort, after we adjusted for sex, age, and comorbidities. The HRs of RF for patients with primary SS and secondary SS compared with the comparison cohort were 2.99 and 3.93, respectively (P for trend <.001). The HRs of RF increased as the severity of SS increased, from 2.34 for those with no inpatient care experience to 5.15 for those with inpatient care experience (P for trend <.001). CONCLUSION: This study indicates that clinical physicians should not only consider secondary SS but also primary SS as a critical factor that increases the risk of RF.
25339641 Identification of Sjögren's syndrome oral fluid biomarker candidates following high-abund 2015 May OBJECTIVE: SS is an autoimmune exocrinopathy affecting ∼1 million patients in the USA that is diagnosed mostly in middle-aged women. Oral fluids (OFs) serving as the mirror of the body were suggested as an ideal non-invasive diagnostic tool. Previously we developed depletion techniques for OF high-abundance proteins to increase visualization of low-abundance proteins. Therefore the aim of this study was to examine the effect of depletion pretreatments on the identification potential of SS OF biomarker candidates. METHODS: Unstimulated OFs were collected from 18 female SS patients and 18 healthy age- and gender-matched controls. High-abundance proteins were depleted using affinity and immunodepletion methodologies followed by semi-quantitative two-dimensional gel electrophoresis and quantitative dimethylation liquid chromatography tandem mass spectrometry (LC-MS/MS). To initially validate the MS results, western blotting was performed. RESULTS: The use of depletion strategy before proteomics analysis increased identification ability by 3-fold. Overall, 79 biomarker candidates were identified. Proteins with the most pronounced fold changes were related to SS serum or tissue factors. In addition, bioinformatics analysis of proteins with a >3-fold increase in SS patients showed calcium-binding proteins, defence-response proteins, proteins involved in apoptotic regulation, stress-response proteins and cell motion-related proteins. Preliminary validation by western blotting of profilin and CA-I indicated similar expression profile trends to those identified by quantitative MS. CONCLUSION: The significance of OF novel depletion methodologies is clearly demonstrated for increased visibility of biomarker candidates as well as for unveiling possible mechanisms involved in this syndrome. This represents a major contribution to our ability to use OF as a future diagnostic fluid.
25325860 A review of corneal melting after Boston Keratoprosthesis. 2014 Sep Use of the Boston Keratoprosthesis (B-KPro) has grown significantly, both in the United States and overseas over the course of the last decade. It is the most frequently employed keratoprosthesis for the management of complex corneal blindness. Improving outcomes and reductions in devastating complications such as corneal melting and infection have motivated this increase in use. We review the epidemiology and pathophysiology of corneal melting following B-KPro as well as the advances in B-KPro design and postoperative care that have halted the occurrence of melting. Eyes with autoimmune diseases such as Stevens-Johnson syndrome, toxic epidermal necrolysis syndrome, and mucous membrane pemphigoid remain particularly vulnerable to corneal melt, leak, and extrusion. The development of new strategies to prevent melting in eyes with autoimmune disease is crucial to improve the outcomes of this group of patients, as they are often those with the most desperate need for visual rehabilitation with a B-KPro.
25318809 Hemophagocytic syndrome and inflammatory myopathy with abundant macrophages in a patient w 2014 We herein describe a 71-year-old woman with adult-onset Still's disease (AOSD) who developed fever, myalgia, and pancytopenia. The bone marrow aspiration and muscle biopsy revealed hemophagocytic syndrome (HPS) and inflammatory myopathy with abundant macrophages (IMAM). Immunostained specimens were positive for expression of retinoic acid-inducible gene-I (RIG-I), which recognizes viral RNA in infiltrated mononuclear cells as well as muscle tissues. These findings suggest that RIG-I may be involved in induction of HPS and IMAM in AOSD.
24937841 Cutaneous ulcer in an immunosuppressed patient with adult onset Still's disease: primary c 2014 May Histoplasmosis is caused by the dimorphic fungus Histoplasma capsulatum.Primary infection occurs through inhalation of spores from the air. Immunocompetent individuals are usually asymptomatic, but may develop pulmonary disease. Immunocompromised patients tend to present systemic histoplasmosis with cutaneous lesions occurring by secondary invasion. In this case report, we describe a probable primary cutaneous histoplasmosis (PCH) in a patient with adult onset Still's disease under immunosuppression.
24584928 Systemic autoimmune rheumatic disease prevalence in Canada: updated analyses across 7 prov 2014 Apr OBJECTIVE: To estimate systemic autoimmune rheumatic disease (SARD) prevalence across 7 Canadian provinces using population-based administrative data evaluating both regional variations and the effects of age and sex. METHODS: Using provincial physician billing and hospitalization data, cases of SARD (systemic lupus erythematosus, scleroderma, primary Sjögren syndrome, polymyositis/dermatomyositis) were ascertained. Three case definitions (rheumatology billing, 2-code physician billing, and hospital diagnosis) were combined to derive a SARD prevalence estimate for each province, categorized by age, sex, and rural/urban status. A hierarchical Bayesian latent class regression model was fit to account for the imperfect sensitivity and specificity of each case definition. The model also provided sensitivity estimates of different case definition approaches. RESULTS: Prevalence estimates for overall SARD ranged between 2 and 5 cases per 1000 residents across provinces. Similar demographic trends were evident across provinces, with greater prevalence in women and in persons over 45 years old. SARD prevalence in women over 45 was close to 1%. Overall sensitivity was poor, but estimates for each of the 3 case definitions improved within older populations and were slightly higher for men compared to women. CONCLUSION: Our results are consistent with previous estimates and other North American findings, and provide results from coast to coast, as well as useful information about the degree of regional and demographic variations that can be seen within a single country. Our work demonstrates the usefulness of using multiple data sources, adjusting for the error in each, and providing estimates of the sensitivity of different case definition approaches.
23992263 Assessment of left ventricular functions in patients with Sjögren's syndrome using tissue 2013 Aug AIM: In our study, we aimed to evaluate left ventricular function in patients with Sjögren syndrome (SS) using tissue Doppler echocardiography (TDE) and myocardial performance index (MPI) in addition to conventional echocardiographic methods. METHODS: We evaluated 50 patients with SS and 48 healthy volunteers with similar demographic characteristics. Systolic and diastolic functions of the left ventricle were analyzed with standard two-dimensional (2D) echocardiography, M-mode echocardiography, pulsed-wave (PW) Doppler and tissue Doppler imaging. RESULTS: Septal part of the mitral annulus PW TDE showed that systolic myocardial wave (Sm), early diastolic myocardial wave (Em), late diastolic myocardial wave (Am) and Em/Am ratios are significantly lower, and myocardial isovolumetric relaxation time (IVRTm) and MPI values are significantly higher in patients with SS. Lateral site of the mitral annulus PW TDE showed that Em, Sm and Em/Am ratios are significantly lower, and IVRTm and MPI values are significantly higher in patients with SS compared with healthy controls. CONCLUSION: In this study, it was shown that both left ventricle systolic and diastolic functions of patients with SS were disturbed.
22623015 A case of adult-onset Still's disease complicated by thrombotic thrombocytopenic purpura w 2013 Mar We present a patient who had adult-onset Still's disease (AOSD) complicated by thrombotic thrombocytopenic purpura (TTP) that resulted in retinal microangiopathy and rapidly fatal cerebral edema. The patient was a 37-year-old male who developed fever, eruption, arthritis and hepatic dysfunction, that, based on close examination, was diagnosed as AOSD. Despite treatment with corticosteroids, the patient developed acute visual field defect, neurological deterioration including convulsions and impaired consciousness, as well as acute renal failure that ultimately resulted in death. Pathological examination of autopsy specimens revealed multiple fibrin thrombi disseminated in small vessels of the brain and kidney, which was consistent with TTP, along with marked cerebral edema. Although TTP has rarely been reported in association with AOSD, awareness of the possible coexistence of these two diseases is important for diagnosis and treatment.
23851886 Recurrent submandibular gland swelling as a first manifestation in a child with primary Sj 2013 Jul We reported 1 case of primary Sjögren syndrome in a child. The patient complained recurrent submandibular inflammation for more than 2 years without apparent sicca presentations, and Schirmer test resulted negative. However, ultrasound showed hypoechoic areas in the gland, laboratory tests reported the positivity of Sjögren's syndrome A antigen and Sjögren's syndrome B antigen, and biopsy presented periductal lymphocytic infiltration. Therefore, a diagnosis of primary Sjögren syndrome was conducted. Treatment of it required a multidisciplinary team.
24450381 Dendritic cells, T-cells and epithelial cells: a crucial interplay in immunopathology of p 2014 Apr Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease that is characterized by mononuclear cell infiltration of exocrine glands. T-cells have been shown to play a central role in tissue destruction and regulation of B-cell activity and the production of autoantibodies typifying pSS. Despite the fact that dendritic cells (DCs) are candidate key players in the activation of T- and B-cells in pSS, their contribution has been under evaluated. This manuscript reviews current insights in DC biology and examines literature on the role of DCs in the immunopathology of primary Sjögren's syndrome, focusing on the interplay between dendritic cells, epithelial cells and T-cells.
24184520 White matter water diffusion changes in primary Sjögren syndrome. 2014 Apr BACKGROUND AND PURPOSE: Histopathologic studies have demonstrated WM damage in primary Sjögren syndrome. The purpose of this study was to evaluate WM microstructural changes by use of DTI-derived parameters in patients with primary Sjögren syndrome. MATERIALS AND METHODS: DTI was performed in 19 patients with primary Sjögren syndrome (age, 64.73 ± 9.1 years; disease duration, 11.5 ± 7.56 years) and 16 age-matched control subjects. Exclusion criteria were a history of major metabolic, neurologic, or psychiatric disorder and high risk for cardiovascular disease. Data were analyzed by use of tract-based spatial statistics, for which the WM skeleton was created, and a permutation-based inference with 5000 permutations was used with a threshold of P < .01, corrected for multiple comparisons to enable identification of abnormalities in fractional anisotropy, mean diffusivity, radial diffusivity, and axial diffusivity. RESULTS: Tract-based spatial statistics showed decreased fractional anisotropy in multiple areas in patients with primary Sjögren syndrome compared with control subjects, located mainly in the corticospinal tract, superior longitudinal fasciculus, anterior thalamic radiation, inferior fronto-occipital fasciculus, uncinate fasciculus, and inferior longitudinal fasciculus. Increased mean diffusivity and radial diffusivity and decreased axial diffusivity were observed in most of the fiber tracts of the brain in patients with primary Sjögren syndrome, compared with control subjects. CONCLUSIONS: Patients with primary Sjögren syndrome show loss of WM microstructural integrity, probably related to both Wallerian degeneration and demyelination.
25178806 Ocular and systemic morbidity in a longitudinal cohort of Sjögren's syndrome. 2015 Jan PURPOSE: To report vision-threatening ocular manifestations of primary Sjögren's syndrome (SS). DESIGN: Retrospective review. PARTICIPANTS: Consecutive patients evaluated at an SS center between January 2007 and May 2011. METHODS: Data collection was completed in March 2013. The 2002 American-European consensus criteria were used for diagnosis of SS. MAIN OUTCOME MEASURES: Frequency of extraglandular ocular findings and timing of their diagnosis relative to that of SS and dry eye were assessed. RESULTS: One hundred sixty-three patients were included. Almost all patients (98%) had a history of dry eye for an average of 10.4 years (median, 7.9 years) before presentation. One or more extraglandular ocular manifestations were present in 40 patients (25%), and vision-threatening findings were present in 22 patients (13%). Twelve patients (55%) with a vision-threatening ocular finding did not have a diagnosis of SS at presentation. Sixty-eight patients (42%) had extraglandular systemic manifestations of SS. Patients with vision-threatening ocular findings were 3.9 times more likely to have systemic involvement (95% confidence interval, 1.4-11.0; P = 0.010). Peripheral neuropathy, interstitial nephritis, and vasculitis were more common in those with vision-threatening ocular findings compared with patients without (P < 0.05 for all). CONCLUSIONS: These results from a tertiary referral-based cohort demonstrate that primary SS frequently is associated with ocular and systemic complications. Dry eye precedes these findings on average by 1 decade. Therefore, ophthalmologists should consider assessing for SS in patients with clinically significant dry eye.
23904442 CXCL13 is elevated in Sjögren's syndrome in mice and humans and is implicated in disease 2013 Nov SS is an autoimmune disease. pSS affects exocrine glands predominantly, whereas sSS occurs with other autoimmune connective tissue disorders. Currently, care for patients with SS is palliative, as no established therapeutics target the disease directly, and its pathogenetic mechanisms remain uncertain. B-cell abnormalities have been identified in SS. CXCL13 directs B-cell chemotaxis and is elevated in several autoimmune diseases. In this study, we tested the hypothesis that CXCL13 is elevated in SS in mice and humans and that neutralization of the chemokine ameliorates disease in a murine model. We assayed CXCL13 in mouse models and human subjects with SS to determine whether CXCL13 is elevated both locally and systemically during SS progression and whether CXCL13 may play a role in and be a biomarker for the disease. Cxcl13 expression in salivary tissue increases with disease progression, and its blockade resulted in a modest reduction in glandular inflammation in an SS model. We demonstrate that in humans CXCL13 is elevated in serum and saliva, and an elevated salivary CXCL13 level distinguishes patients with xerostomia. These data suggest a role for CXCL13 as a valuable biomarker in SS, as 74% of patients with SS displayed elevated CXCL13 in sera, saliva, or both. Thus, CXCL13 may be pathogenically involved in SS and may serve as a new marker and a potential therapeutic target.
23682999 Diagnostic model of saliva peptide finger print analysis of primary Sjögren's syndrome pa 2013 Jul 16 Saliva diagnostics has become an attractive field utilizing nanotechnology and molecular technologies for pSS (primary Sjögren's syndrome). However, no specific methods have been established. To refine the diagnostic power of the saliva peptide finger print for the early detection of pSS, we screened the expression spectrum of salivary peptides in pSS patients by using mass spectrometry MALDI-TOF-MS (matrix-assisted laser-desorption ionization-time-of-flight MS) combined with magnetic bead. The present study was comprised 12 pSS patients and 13 healthy controls and broken down to two different phases. In the initial 'exploratory phase', we enrolled seven pSS patients with eight age- and sex-matched healthy volunteers. Proteomics analysis of the unstimulated salivary samples was conducted to generate proportional peptide mass fingerprints. A diagnostic model was established. The testing cohort of the second 'validation phase' was represented by five pSS patients and five age- and sex-matched healthy controls. The diagnostic power of this diagnostic panel was then validated. The results showed seven m/z (mass-to-charge) ratio peaks with significant differences. Five peptides were up-regulated and two down-regulated in the pSS patients compared with matched healthy subjects. In the validation phase, four out of five pSS patients were diagnosed as pSS, and four of the five healthy controls were diagnosed as healthy controls, respectively. Potential biomarkers were also primarily predicted. The novel diagnostic proteomic model with m/z peaks 1068.1 Da, 1196.2 Da, 1738.4 Da, 3375.3 Da, 3429.3 Da, 3449.7 Da and 3490.6 Da is of certain value for early diagnosis of pSS.