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ID PMID Title PublicationDate abstract
25398063 Myocarditis in adult-onset still disease. 2014 Oct This study highlights the clinical features, treatments, and outcomes of the rare myocarditis in adult-onset Still disease (AOSD). Among a case series of 57 patients fulfilling either Yamaguchi or Fautrel AOSD criteria and seen between 1998 and 2010, we identified 4 cases of myocarditis. From a comprehensive literature review, we collected 20 additional cases of myocarditis-complicated AOSD. The characteristics of patients with myocarditis were compared with those of AOSD patients without myocarditis.In these 24 myocarditis-complicated AOSD cases, myocarditis occurred early and was present at AOSD onset in 54% of the cases. Myocarditis was often symptomatic (96% of patients) with nonspecific electrocardiographic abnormalities (79% of patients) and a left ventricle ejection fraction ≤50% (67% of patients). Cardiac magnetic resonance imaging and endomyocardial biopsies showed features consistent with myocarditis in 4 patients and a mononuclear interstitial inflammatory infiltrate in 4 others. Steroids alone were effective in 50% of patients with myocarditis. Intravenous immunoglobulins, methotrexate, and tumor necrosis factor-α-blockers were also prescribed and often found effective. Only 1 patient died from cardiogenic shock. Patients with myocarditis-complicated AOSD were younger and more frequently male than patients with AOSD alone. Pericarditis was more frequent in the myocarditis group; white blood cell count, polymorphonuclear cell count, and serum ferritin levels were also higher.Myocarditis is a potentially life-threatening complication of AOSD but responds positively to steroids and other immunomodulatory drugs. Its prognosis remains good (only 1 death occurred), but the condition requires close monitoring of heart function.
23997769 Detailed Joint Region Analysis of the 7-Joint Ultrasound Score: Evaluation of an Arthritis 2013 Objective. The main objective of this study was to evaluate the 7-joint ultrasound (US7) score by detailed joint region analysis of an arthritis patient cohort. Methods. The US7 score examines the clinically most affected wrist, MCP and PIP II, III, MTP II, and V joints for synovitis, tenosynovitis/paratenonitis, and erosions. Forty-five patients with rheumatoid arthritis (RA) (84.4%) and spondyloarthritis with polyarticular peripheral arthritis (PsA 13.3%; AS 2.2%) with a median disease duration of 6.5 yrs (range 7.5 mths-47.6 yrs) were included and examined at baseline and 3, 6, and 12 months after starting or changing therapy (DMARD/biologic). In this study, detailed US7 score joint region analysis was firstly performed. Results. The joint region analysis performed at baseline disclosed synovitis in 95.6% of affected wrists in the dorsal aspect by greyscale (GS) US where Grade 2 (moderate) was most often (48.9%) detected. Palmar wrist regions presented Grade 1 (minor) capsule elevation in 40% and Grade 2 (moderate synovitis) in 37.8%. Tenosynovitis of the extensor carpi ulnaris (ECU) tendon was found in 40%, with PD activity in 6.6%. Most of the erosions in MCP II were detected in the radial (68.9%), followed by the dorsal (48.9%) and palmar (44.4%) aspects. In MTP V, erosions were seen in 75.6% from lateral. Conclusions. Synovitis in GSUS was more often detected in the wrist in the dorsal than in the palmar aspect. ECU tendon involvement was frequent. Most erosions were found in the lateral scan of MTP V and the medial (radial) scan of MCP II.
24943133 Loss of cerebral white matter in primary Sjögren's syndrome: a controlled volumetric magn 2014 Oct BACKGROUND AND PURPOSE: Although brain involvement is common in primary Sjögren's syndrome (pSS), results from cerebral imaging studies are inconsistent. This study aimed to perform both voxel-wise and global brain volume analyses in a nearly population-based pSS cohort to explore whether the patients displayed any focal or diffuse volume differences compared with healthy subjects. METHODS: Global grey matter (GM) and white matter (WM) volumes were measured and compared in 60 patients with pSS and 60 age- and gender-matched healthy subjects. Regression models were constructed with potential explanatory variables for GM and WM volumes. In the same groups, voxel-wise morphometric analyses were performed. RESULTS: In analyses of global GM and WM, the patients had lower WM volumes than healthy subjects (540 ± 63 cm(3) vs. 564 ± 56 cm(3), P = 0.02), but no differences in GM. Voxel-wise analyses displayed no localized areas of GM or WM volume differences between pSS patients and healthy subjects. CONCLUSION: Individuals with pSS have a diffuse reduction of cerebral WM but no localized loss of WM or GM. This indicates a general deleterious effect on WM due to pSS itself.
24365313 Connective tissue disorders: systemic lupus erythematosus, Sjögren's syndrome, and sclero 2014 Connective tissue disorders are systemic, autoimmune, multiorgan diseases in which the central and peripheral nervous systems are frequently involved. The objective of this chapter is to describe the neurological manifestations of three of the most common systemic autoimmune disorders: systemic lupus erythematosus (SLE), scleroderma, and Sjögren's syndrome (SS). In SLE the neuropsychiatric manifestations involve mainly the central nervous system (CNS), including cognitive dysfunction, headache, psychosis and mood changes, seizures, cerebrovascular disease, and myelopathy. Peripheral nervous system (PNS) manifestations are less common and include polyneuropathies as well as mononeuropathies and acute inflammatory demyelinating polyneuropathy. Antiphospholipid syndrome (APLS) is relatively common and should be searched for whenever focal neurological symptoms occur. In scleroderma the PNS is more commonly involved; symptoms include polyneuropathies, entrapment neuropathies, and mononeuropathies (mostly cranial neuropathies or mononeuritis multiplex). Additionally autonomic involvement occurs and myopathies are relatively common. In SS the PNS is similarly involved with several types of polyneuropathies, mononeuropathies, and autonomic dysfunction. Also common are myelopathies and aseptic meningitides. These and other, less common manifestations, as well as the diagnostic procedures and the therapeutic approaches, will be dealt with in this chapter.
23424683 The application of optical coherence tomography in musculoskeletal disease. 2013 Many musculoskeletal disorders (MDs) are associated with irreversible bone and cartilage damage; this is particularly true for osteoarthritis (OA). Therefore, a clinical need exists for modalities which can detect OA and other MDs at early stages. Optical coherence tomography (OCT) is an infrared-based imaging, currently FDA approved in cardiology and ophthalmology, which has a resolution greater than 10 microns and acquisition rate of 120 frames/second. It has shown feasibility for imaging early OA, identifying changes prior to cartilage thinning both in vitro and in vivo in patients and in OA animal models. In addition, OCT has shown an ability to identify early rheumatoid arthritis (RA) and guide tendon repair, but has the potential for an even greater impact. Clinical trials in OA are currently underway, as well as in several other MDs.
23912652 Lymphocytic interstitial pneumonia as a manifestation of SLE and secondary Sjogren's syndr 2013 Aug 2 A 47-year-old woman with systemic lupus erythematosus (SLE) diagnosed at age of 35 years was admitted for dyspnoea, substernal chest pain, dry mucosas and difficulty in swallowing. Physical examination revealed vesicular breath sounds bilaterally. Laboratory work showed antinuclear antibody (ANA) (speckled pattern, 1:40), positive anti-Sjogren's syndrome antigen (SSA) and antisingle side band (SSB) and negative double-strand DNA (dsDNA), with normal C3,C4,C50. A high-resolution chest CT scan demonstrated multiple bronchial cysts and diffuse interstitial infiltrates. Surgical lung biopsy revealed emphysematous changes and mild lymphocytic infiltrate around the bronchioles compatible with lymphocytic interstitial pneumonia diagnosis. This case illustrates a patient with primary SLE overlapped by initial manifestations of secondary Sjogren's syndrome (SS) presenting with associated autoimmune interstitial lung disease. Antibody markers, high-resolution chest CT scan and surgical lung biopsy were essential in evaluating this patient, confirming the interstitial lymphocytic infiltration of the lung. Primary SS (pSS) is the most commonly associated disease to lung interstitial pneumonia (LIP) (25%). High-resolution chest CT scan demonstrates areas of ground-glass attenuation, suggestive of interstitial disease. Surgical lung biopsy shows pathologic increase of mature lymphocyte cells and histiocytes. Most of the cases have a benign presentation and shortly relapse. Superimposed infection, pulmonary fibrosis and lymphoma develop in less than 20% of cases. Corticosteroids are the primary therapy. While pSS is commonly associated with interstitial lung involvement, secondary Sjogren's syndrome (sSS) is only rare. It has been described the initial sSS presentation by Sica symptoms development only, and our case is the first report of LIP presentation as initial manifestation of sSS. Our patient remained stable after corticosteroids and hydoxychloroquine therapy and no progression of disease after 6 months follow-up.
23436737 European League Against Rheumatism Sjögren's Syndrome Disease Activity Index and European 2013 Aug OBJECTIVE: The European League Against Rheumatism (EULAR) Sjögren's Syndrome (SS) Disease Activity Index (ESSDAI) and the EULAR SS Patient-Reported Index (ESSPRI) were recently developed. We aimed to determine whether patients' symptoms differed between patients with and without systemic involvement and if the disease-specific indices correlated with each other in primary SS. METHODS: Fifteen French centers included 395 primary SS patients in the Assessment of Systemic Signs and Evolution in Sjögren's Syndrome Cohort. At enrollment, physicians completed the ESSDAI, the SS Disease Activity Index (SSDAI), and the Sjögren's Systemic Clinical Activity Index (SCAI), and patients completed the ESSPRI, the Sicca Symptoms Inventory, and the Profile of Fatigue and Discomfort. All scores were compared between patients with and without systemic involvement. Correlations between scores of systemic activity and patients' symptoms were obtained. RESULTS: At enrollment, 120 (30.4%) patients had never experienced systemic complication and 155 (39.2%) patients and 120 (30.4%) patients had, respectively, only past or current systemic manifestations. Past or current systemic patients had higher levels of symptoms, except dryness. The ESSDAI did not correlate with the patient-scored ESSPRI (rho = 0.06, P = 0.30), whereas the SSDAI and the SCAI, which include subjective items, did correlate (rho = 0.28 and 0.25, respectively; P < 0.0001 for both). CONCLUSION: Alterations of common patient-reported outcomes are present in all patients with primary SS, including those with systemic complications. However, patient symptoms and systemic complications are 2 different facets of primary SS. Therefore, the use of both systemic and patients' indices, such as the ESSDAI and ESSPRI, are useful. Since these 2 facets weakly overlap, one should identify which of both components is the main target of the treatment to test, when designing clinical trials in primary SS.
24990382 Anti-cyclic citrullinated peptide antibody as a marker of erosive arthritis in patients wi 2014 Oct OBJECTIVE: Anti-cyclic citrullinated peptide (CCP) antibody is an established marker in the diagnosis and prognostication of rheumatoid arthritis (RA). Infrequently, systemic lupus erythematosus (SLE) patients also develop a deforming erosive arthritis, similar to that of RA. Our objective was to determine whether anti-CCP antibody is a useful marker of erosive disease in SLE patients presenting with arthritis. METHODS: Electronic databases EMBASE, MEDLINE and non-indexed MEDLINE citations were searched through April 11, 2014, using the outlined key terms. Studies meeting predefined inclusion and exclusion criteria were reviewed. Two reviewers independently assessed the quality of included articles using previously described criteria. The DerSimonian-Laird random effects model was used to calculate pooled sensitivity and specificity of anti-CCP antibody for erosive arthritis in SLE. RESULTS: Seven articles met inclusion and exclusion criteria. A total of 609 SLE patients with arthritis were identified, 70 of whom had erosive disease. Pooled sensitivity and specificity of anti-CCP antibody for erosive arthritis was 47.8% (95% CI, 26.2%-70.2%) and 91.8% (95% CI, 78.4%-97.2%), respectively. CONCLUSION: Our findings suggest that anti-CCP antibody is a highly specific marker for erosive arthritis in SLE. Longitudinal prospective studies are needed to determine if anti-CCP antibody can be used as a predictor of erosive disease.
23858044 HMGB1 levels are increased in patients with juvenile idiopathic arthritis, correlate with 2013 Sep OBJECTIVE: High mobility group box chromosomal protein 1 (HMGB1) has been implicated as a mediator of inflammation in rheumatoid arthritis (RA), while its role in juvenile idiopathic arthritis (JIA) has not been described. To evaluate the role of HMGB1 in the inflammatory process in JIA and its potential as a therapeutic target, we investigated whether extracellular HMGB1 is detectable in JIA and if so, to correlate the levels with established inflammatory markers and clinical measures. METHODS: Matching samples of blood and synovial fluid (SF) were collected from 23 patients with JIA. Levels of HMGB1, soluble receptor for advanced glycation endproducts, S100A12, myeloid-related protein 8/14, and other inflammatory mediators were analyzed. RESULTS: Significantly increased HMGB1 levels were recorded in SF compared to blood samples from patients with JIA. The amount of HMGB1 was highest in patients with early disease onset irrespective of disease duration. In contrast, the proinflammatory S100 protein and interleukin 8 were highest in patients in early phases of disease. Matrix metalloproteinase-3, a marker of cartilage destruction, was higher in patients with late disease onset, indicating similarities with RA in that patient subgroup. CONCLUSION: Levels of extracellular HMGB1 are increased in the inflamed joints of patients with JIA. This warrants further studies of HMGB1 as a mediator of JIA pathogenesis as well as a biomarker for inflammatory activity and as a target for therapy. The variation in levels of HMGB1 and S100 proteins in relation to disease onset indicates a difference in inflammatory phenotype during disease progression.
24289962 HTLV-1-associated arthropathy treated with anti-TNF-alpha agent. 2014 Jul Human T cell leukemia virus type 1 or HTLV-1 infection is a public health problem in endemic regions like Japan, Central America or Africa. Although the majority of HTLV-1 carriers remain asymptomatic throughout their lives, some patients could develop neurological disorder, inflammatory arthropathy also called HTLV-1-associated arthropathy or T-cell malignancy, the adult T-cell leukemia/lymphoma or ATL with a very poor prognosis. Described to be very close to rheumatoid arthritis, HTLV-1-associated arthropathy patients have few or no response to the first line therapy with corticosteroids and disease modifying antirheumatic drugs or DMARDs. The use of anti-TNF-α agents in these patients is an interesting alternative but asks the question of risk of developing an adult T-Cell leukemia/lymphoma. We reported an exceptional case of a smoldering ATL patient with an HTLV-1-associated arthropathy, refractory to corticosteroid, DMARDs and rituximab therapy, treated successfully with etanercept, without progression to aggressive ATL after 5 years.
23469022 IL-23 dependent and independent stages of experimental arthritis: no clinical effect of th 2013 IL-23p19 deficient mice have revealed a critical role of IL-23 in the development of experimental autoimmune diseases, such as collagen-induced arthritis (CIA). Neutralizing IL-23 after onset of CIA in rats has been shown to reduce paw volume, but the effect on synovial inflammation and the immunological autoimmune response is not clear. In this study, we examined the role of IL-23 at different stages of CIA and during T cell memory mediated flare-up arthritis with focus on changes in B cell activity and Th1/Th17 modulation. Anti-IL-23p19 antibody (anti-IL23p19) treatment, starting 15 days after the type II collagen (CII)-immunization but before clinical signs of disease onset, significantly suppressed the severity of CIA. This was accompanied with significantly lower CII-specific IgG1 levels and lower IgG2a levels in the anti-IL-23p19 treated mice compared to the control group. Importantly, neutralizing IL-23 after the first signs of CIA did not ameliorate the disease. This was in contrast to arthritic mice that underwent an arthritis flare-up since a significantly lower disease score was observed in the IL-23p19 treated mice compared to the control group, accompanied by lower synovial IL-17A and IL-22 expression in the knee joints of these mice. These data show IL-23-dependent and IL-23-independent stages during autoimmune CIA. Furthermore, the memory T cell mediated flare-up arthritis is IL-23-mediated. These data suggest that specific neutralization of IL-23p19 after onset of autoimmune arthritis may not be beneficial as a therapeutic therapy for patients with rheumatoid arthritis (RA). However, T cell mediated arthritis relapses in patients with RA might be controlled by anti-IL-23p19 treatment.
23124729 Leflunomide addition in patients with articular manifestations of psoriatic arthritis resi 2013 Nov In contrast to rheumatoid arthritis, in psoriatic arthritis (PsA), the efficacy of disease-modifying antirheumatic drugs (DMARDs) combination has not been documented. We conducted a retrospective study to evaluate the effectiveness of leflunomide (LEF) addition in 11 PsA patients with articular manifestations that failed to respond to methotrexate (MTX) monotherapy [disease activity score in 28 joints (DAS28) > 3.2)]. Eight of them, all with moderate disease activity (DAS28 < 5.1) at baseline, tolerated the combination. A statistically significant improvement of the mean DAS28, based on erythrocyte sedimentation rate (ESR), and its variables, and C-reactive protein (CRP) at 12-16 weeks after LEF addition was observed. Mean change of DAS28 in patients with polyarticular disease did not differ compared with those with oligoarticular. Based on the European League Against Rheumatism (EULAR) response criteria, none of our patients achieved a good response, seven had a moderate response, and one was a non-responder. The two patients with the lower DAS28 at baseline attained low disease activity (LDA, DAS28 ≤ 3.2), while none reached remission (DAS28 ≤ 2.6). Achievement of clinical remission or at least LDA has been recently proposed as the goal of treatment in PsA. Our results imply that LEF addition may serve as an alternative therapeutic modality for patients with moderately active PsA and, as lower as possible, residual disease activity after the initial therapy with MTX alone.
23983144 Determinants of health-related quality of life in children newly diagnosed with juvenile i 2014 Feb OBJECTIVE: To examine the degree to which nonmedical factors explain additional variance in parent proxy report and child self-report of health-related quality of life (HRQOL) among newly diagnosed children with juvenile idiopathic arthritis (JIA) after accounting for medical factors. METHODS: Parents (of children ages 2-16 years; n = 230) and patients (ages >5 years; n = 180) diagnosed within the previous 6 months completed surveys to assess medical (clinical parameters and functional status) and nonmedical (self-efficacy, coping, barriers to adherence, social support, parental distress, and access to care) factors and HRQOL (Pediatric Quality of Life Inventory Generic Core Scales). Physician-rated global assessment of disease activity, active joint count, and select laboratory variables (rheumatoid factor, antinuclear antibodies, and erythrocyte sedimentation rate) were recorded. RESULTS: Nonmedical factors, including self-efficacy, coping with pain, barriers to adherence, social support, and parental distress, explained additional variance in HRQOL total, physical functioning, and psychosocial functioning scales (R(2) increases of 6%, 1%, and 13% for parent proxy report and 16%, 7%, and 30% for self-report, respectively). Parental distress was uniquely associated with parent proxy-report HRQOL, while child self-efficacy and social support were uniquely associated with self-report HRQOL. CONCLUSION: Nonmedical factors are associated with HRQOL in newly diagnosed patients with JIA after accounting for medical variables, particularly for psychosocial functioning.
22955798 EBV reactivation serological profile in primary Sjögren's syndrome: an underlying trigger 2013 May Antibody to Epstein-Barr virus (EBV) early antigen diffuse (anti-EA-D) is associated with viral replication. However, their possible associations with clinical/therapeutic features in primary Sjögren's syndrome (pSS) were not established. We evaluated 100 pSS patients (American-European Criteria) and 89 age/gender/ethnicity-matched healthy controls. Disease activity was measured by EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI). Antibodies to EBV (anti-VCA IgG/IgM, anti-EBNA-1 IgG, anti-EA-D IgG) were determined by ELISA. Patients and controls had comparable frequencies and mean levels of anti-VCA IgG (90 vs. 86.5 %, p = 0.501; 2.6 ± 1.1 vs. 2.5 ± 1.1 AU/mL, p = 0.737) and anti-EBNA-1 IgG (92 vs. 94.4 %, p = 0.576; 141.3 ± 69.8 vs. 135.6 ± 67.5 RU/mL, p = 0.464). Anti-VCA IgM was negative in all cases. Noteworthy, higher frequency and increased mean levels of anti-EA-D were observed in patients than controls (36 vs. 4.5 %, p < 0.0001; 38.6 ± 57.4 vs. 7.9 ± 26.3 RU/mL, p < 0.0001). Further analysis of patients with (n = 36) and without (n = 64) anti-EA-D revealed comparable age/gender/ethnicity (p ≥ 0.551), current prednisone dose (4.8 ± 6.9 vs. 5.1 ± 10.4 mg/day, p = 0.319), and current uses of prednisone (52.8 vs. 37.5 %, p = 0.148) and immunosuppressants (44.4 vs. 31.3 %, p = 0.201). ESSDAI values were comparable (p = 0.102), but joint activity was more frequent (25 vs. 9.4 %, p = 0.045) in anti-EA-D positive patients. Anti-EA-D antibodies were not associated with anti-Ro/SSA (p = 1.000), anti-La/SSB (p = 0.652), rheumatoid factor (p = 1.000), anti-α-fodrin (p = 0.390) or antiphospholipid antibodies (p = 0.573), not suggesting cross-reactivity. The higher anti-EA-D frequency associated with joint activity raises the possibility that a subclinical EBV reactivation may trigger or perpetuate the articular involvement in pSS.
24892681 Life-threatening pneumonitis complicating low-dose methotrexate treatment for juvenile idi 2014 Jun Methotrexate, a drug commonly used to treat juvenile idiopathic arthritis (JIA), has been reported to cause interstitial pneumonitis as a rare complication in adults with rheumatoid arthritis. Only 1 suspicious case of methotrexate pneumonitis in a child with JIA has been reported in 1998, though with no histopathologic proof. Given its rarity and nonspecific presenting symptoms, diagnosis may be challenging, and a life-threatening illness can occur without a high index of suspicion, as illustrated by this report of a 13-year-old girl with JIA who developed fever, nonproductive cough, and dyspnea as presenting features of interstitial pneumonitis after 1 year of methotrexate therapy. Chest high-resolution computed tomography revealed patchy ground-glass opacities and interlobular septal thickening without pleural effusion. Lung biopsy showed interstitial pneumonitis with diffuse alveolar damage. The symptoms and radiographs improved dramatically after withdrawal of methotrexate and administration of corticosteroids. A restrictive ventilatory defect with decreased diffusion capacity on pulmonary function testing persisted until 20 months after methotrexate withdrawal. There is no single pathognomic feature for methotrexate pneumonitis; rather, diagnosis relies on a constellation of clinical, radiologic, and pathologic findings. This report highlights the necessity for pediatricians to be continuously vigilant for interstitial pneumonitis in children receiving methotrexate who develop new unexplained pulmonary symptoms.
23995354 Conjugate between chondroitin sulfate and prednisolone with a glycine linker: preparation 2013 A conjugate between prednisolone (PD) and chondroitin sulfate (CS) with glycine as a linker was prepared in order to obtain an effective macromolecular prodrug against inflammatory disease, especially rheumatoid arthritis. First, PD was converted to the N-trityl-glycine ester (Tr-GP), and the glycine ester of PD (GP) was obtained by detritylation of Tr-GP. Then, GP and CS were condensed with water-soluble carbodiimide to yield CS-GP. The obtained conjugate had a PD content of 2.24% (w/w). Conversion characteristics were investigated for GP and CS-GP to evaluate their potential as a prodrug. In the stability test of GP, PD was released well in the buffer at pH 6-7.4, but degraded rapidly at pH 8 without sufficient release of PD. As to CS-GP, PD was released more slowly than in GP, and the release rate rose with the increase in the medium pH. PD was released gradually from CS-GP over 24 h at a physiological pH. The conversion profiles of both GP and CS-GP almost followed pseudo-first order kinetics. The calculated conversion rate constants supported the gradual and effective release from CS-GP. The release rate of PD from GP and CS-GP was accelerated by the addition of rat plasma, but the promotion of release from CS-GP was small, suggesting that PD should be released gradually from CS-GP in the systemic circulation. It was demonstrated from the preliminary pharmacological study using rats with adjuvant-induced arthritis that CS-GP had high anti-inflammatory potential against arthritis.
23238606 Transfer from paediatric rheumatology to the adult rheumatology setting: experiences and e 2013 May Adolescents with juvenile idiopathic arthritis (JIA) are transferred from paediatrics to adult-oriented healthcare when they reach early adulthood. Research on the extent to which patients' expectations about the adult healthcare setting match their actual experience after transfer, may promote successful transfer from paediatrics to adult care. As part of the 'Don't Retard' project ( http://www.kuleuven.be/switch2/rheuma.html ), experiences and expectations of young adults regarding their transfer from paediatric rheumatology to adult rheumatology were explored. A qualitative study was conducted using semi-structured, in-depth interviews of 11 patients with JIA, aged 18 to 30. Data were analysed using procedures inherent to the content analysis approach. For both concepts, experiences and expectations, three main themes emerged: 'preparation', 'parental involvement' and an 'adapted setting for the late-adolescent or early adult'. The need for a gradual process covered the themes 'preparation' and 'parental involvement'. Young people with JIA prefer to have a say in the moment of transfer and in the reduction of parental involvement. The majority of the participants like their parents' presence at the first consultation at the adult rheumatology department. They expect a healthcare setting adapted to their needs and the possibility to meet peers in this setting. Sudden confrontation with older patients with severe rheumatoid arthritis at adult rheumatology was an unsettling experience for some of the young patients and they declared that better preparation is needed. This study enabled us to define three main themes important in transfer. These themes can facilitate healthcare professionals in developing specific interventions to prepare the young people to transfer, to regulate parental involvement and to arrange an adapted setting for them. Since we included patients who were in follow-up at one tertiary care centre, in which both paediatric and adult rheumatology care are located, the results of the study cannot be generalised to the entire population of patients with JIA.
24685418 Anti-rheumatoid and anti-oxidant activity of homeopathic Guaiacum officinale in an animal 2014 Apr BACKGROUND: Homeopathy is a popular form of complementary and alternative medicine. Guaiacum extract is said to be useful for pain and inflammation, but there appears to be no scientific evidence to support this. AIMS: The aim of the present study was to evaluate the anti-rheumatic and anti-oxidant activity of homeopathic preparations of Guaiacum officinale (Gua) on experimental animal model. DESIGN: Rheumatoid arthritis (RA) was induced in male albino rats by Freund's complete adjuvant (FCA) at a dose of (0.25 mg heat killed Mycobacterium tuberculosis/ml of emulsion). Gua mother tincture (MT) (prepared from the latex part of the plant) (MT), Gua 30cc and 200cc were purchased commercially from King Company, Kolkata, India. Male albino Wistar rats (130 ± 10 g) were divided into 6 groups: Sham control; Arthritis control; Standard treatment indomethacin (0.25 mg 100 g(-1) p.o. × 5 alternative days), Gua MT (1 ml kg(-1) p.o. × 5 days) treated; Gua (30c 1 ml kg(-1) p.o. × 5 days) treated; Gua (200c; 1 ml kg(-1) p.o. × 5 days) treated. Anti-rheumatic activity was examined through physical, urinary, serum parameters. All the results were expressed in terms of mean ± SEM (statistical error of mean n = 6) at each dose level. The level of significance was determined through one-way analysis of variance (ANOVA), p < 0.05 was considered significant. RESULTS: It was observed that body weight, ankle and knee diameter, urinary parameters (hydroxyproline (OH-P), glucosamine, calcium (Ca(2)(+)), creatinine (CRE), phosphate (PO4(3)(-))), serum ACP (acid phosphatase)/ALP (alkaline phosphatase)/Ca(2+)/CRE/PO4(3-)/gamma-glutamyl transferase (GGT)/Lipid peroxidation (LPO)/Glutathione (GSH)/Superoxide dismutase (SOD)/Catalase, serum GGT, serum interleukins like IL-1β/CINC-1/PGE2/TNF-α/IL-6, IL-12/IL-4/IL-6 levels were significantly affected. After treatment with Guaiacum in all 3 regimes was associated with normalization of these parameters compared to control group. CONCLUSION: These findings suggest that homeopathic G. officinale possesses anti-rheumatic and anti-oxidant activity in experimental animal and these activities may be more significant in higher potencies.
24088343 Pulmonary hypertension secondary to hyperviscosity in a patient with rheumatoid arthritis 2013 Oct 2 INTRODUCTION: Acquired von Willebrand disease is initiated by autoantibodies and hyperviscosity syndrome caused by a massive polyclonal hypergammaglobulinemia. Acquired von Willebrand disease associated with autoimmune disease in addition to pulmonary hypertension during emergency room presentation is a rare condition. To the best of our knowledge, this is the second case reported in the literature treated with success; the first one was reported in 1987. CASE PRESENTATION: A 28-year-old mestizo man with a 3-year history of inflammatory arthritis was admitted to our hospital. An overlap of rheumatoid arthritis with systemic lupus erythematosus was suspected; therefore methotrexate was initiated, and later changed to leflunomide because of liver toxicity. Prothrombin time, international normalized ratio and activated partial thromboplastin times were normal (11/10.4 seconds; 1.2; 31.1/26.9 seconds, respectively), von Willebrand factor activity was observed with low ristocetin cofactor at 33.6UI/dL, high von Willebrand factor antigen >200UI/dL, and a low von Willebrand factor: ristocetin cofactor to von Willebrand factor antigen ratio. He was admitted to the emergency room with a 24-hour evolution of progressive dyspnea, cough, thoracic pain, and palpitations, 104 beats/min, 60/40 mmHg, temperature of 38°C, pulse oximetric saturation 88% and 30 breaths/minute. Cold, pale and mottled skin was also observed. He was then transferred to the intensive care unit. The placement of a pulmonary artery catheter was made. The initial patterns showed a precapillary pulmonary hypertension; acute pulmonary embolism was the first choice for diagnosis. Pulmonary angiography was conducted, and when no clot was discovered, pulmonary artery hypertension associated with connective tissue disease was considered. Serum protein electrophoresis confirmed the presence of a massive polyclonal hypergammaglobulinemia, and no paraproteinemia or monoclonal cell population was found from the electrophoretic pattern of the patient's plasma. Hypergammaglobulinemia was the cause of hyperviscosity syndrome associated with autoantibodies. Three sessions of plasma exchange therapy were made, and clinical improvement was observed. He was then discharged from the intensive care unit and hospital, respectively. He is now attended by an external consult and has no respiratory symptomatology. CONCLUSIONS: Hyperviscosity syndrome with pulmonary arterial hypertension presentation in a patient with acquired von Willebrand disease in an autoimmune context is a rare condition that can be treated successfully with plasmapheresis and critical care support.
23183090 Rapid-resolution liquid chromatography TOF-MS for urine metabolomic analysis of collagen-i 2013 Jan 30 AIM OF THE STUDY: Rheumatoid arthritis (RA) is a kind of autoimmune diseases characterized by persistent synovitis, systemic inflammation and autoantibodies. Huang-Lian-Jie-Du-Tang (HLJDT) is a traditional Chinese medicine (TCM) with anti-inflammatory activity. In the present study, a rapid-resolution liquid chromatography tandem time-of-flight mass spectrometry (RRLC-TOF-MS) based metabolomic study was developed to obtain a systematic view of the progression of RA and assess the efficacy of HLJDT and its components in collagen-induced arthritis (CIA) rats. MATERIALS AND METHODS: Forty male Wistar rats were randomly divided into five groups, including model group, normal control group, dexamethasone group, HLJDT group and the mixture of 13 components of HLJDT group after immunized with bovine type II collagen. Urine samples for metabolomic study were collected on 8, 15, 22 day during the animal experiment. RESULTS: The pharmacological changes (swelling paws and arthritis scores) showed that prophylactic treatment with HLJDT and its components significantly suppressed the swelling of rats' paws. By combining with partial least squares discriminant analysis, 24 potential biomarkers were identified and primarily involved in 12 metabolism pathways, such as tricarboxylic acids cycle metabolism, lipid metabolism, tryptophan metabolism and phenylalanine metabolism, which revealed a new insight into the RA network in vivo. These potential metabolites identified in CIA model need to be further investigated to prove their diagnostic and/or prognostic values for RA. Taking potential biomarkers found in the study as screening indexes, it revealed that HLJDT and its components could reverse the pathological process of RA through partly regulating the disturbed metabolic pathways. CONCLUSIONS: This study indicated that the metabolomic strategy based on RRLC-TOF-MS is a useful tool to search potential biomarkers related to RA and to dissect the underlying mechanisms of TCM in treating RA.