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ID PMID Title PublicationDate abstract
25308575 Effect of non-invasive tear stability assessment on tear meniscus height. 2015 Mar PURPOSE: To investigate the effect of non-invasive tear stability assessment with forced eye opening on the lower tear meniscus. METHODS: Twenty-three eyes of 23 patients with aqueous-deficient dry eye and 23 eyes of 23 normal subjects were enrolled. All subjects underwent imaging with a Keratograph 5M equipped with a modified tear film scanning function. Lower tear meniscus images were captured, and tear meniscus height (TMH) was measured with an integrated ruler before and after non-invasive Keratograph break-up time (NIKBUT) measurements in each subject. Subjects were instructed to keep their eyes open as long as possible during NIKBUT measurements, and the recording was discontinued at the next blink. RESULTS: The TMH values of the normal and dry eye groups were 0.20±0.05 mm and 0.14±0.03 mm, respectively, at baseline. The TMH values of dry eyes were significantly smaller than those of normal eyes (p<0.001). Significant increases in TMH values were observed in both normal (0.10±0.12 mm) and dry eyes (0.04±0.09 mm) with the NIKBUT measurement (p<0.001, p=0.039). A moderate negative correlation was observed between increased TMH and baseline TMH in dry eyes (r=-0.44, p=0.03), whereas no correlation was observed in normal eyes (r=0.04, p=0.85). CONCLUSIONS: Forced eye opening required for the non-invasive tear stability assessment influences the TMH measurement possibly due to reflex tear secretion, even in patients with aqueous-deficient dry eye. TMH should be assessed before tests that require forced eye opening.
24706034 Increased dental visits in patients prior to diagnosis of primary Sjögren's syndrome: a p 2014 Nov The objective of this study was to investigate the utilization of ambulatory dental services in patients with primary Sjögren's syndrome (pSS) prior to their diagnosis using a population-based health claim database in Taiwan. From the Registry of Catastrophic Illness database in the National Health Insurance Research Database in Taiwan, we identified 389 patients who were diagnosed with pSS from 2005 to 2010. We also obtained 1,945 control subjects frequency-matched on sex, 10-year age interval, and enrollment date from the Longitudinal Health Insurance Database. Both groups were retrospectively traced to 1997 to obtain any records of utilization of dental care services that occurred preceding the index date. Patients with pSS had significantly higher utilization of annual ambulatory dental services over an eight-year interval preceding diagnosis. A significantly higher proportion of patients with pSS (95.1 %) had utilized ambulatory dental services over the whole eight-year interval preceding diagnosis compared with the controls (88.8 %) (p < 0.001). Utilization of ambulatory dental services for dental caries, pulpitis, gingivitis, stomatitis, and periodontitis were significantly greater in patients with pSS over a three-year period prior to diagnosis compared with the controls. In conclusion, the utilization of ambulatory dental services was significantly increased in patients with pSS preceding its diagnosis. Health professionals can play an important role in the early recognition of pSS by including it in the differential diagnosis for xerostomia-related complaints.
23182291 [Severe hepatitis in a patient with adult-onset Still's disease treated with anakinra]. 2013 Mar INTRODUCTION: Adult-onset Still's disease is characterized by non-specific polymorphic features. The efficacy of anakinra, an IL-1 receptor antagonist, has been shown in several studies. This medication is well-tolerated, and only one case of severe hepatitis has been previously reported. CASE REPORT: A 22-year-old woman presented with fever, rash, arthritis, and pericarditis, associated with systemic inflammatory response syndrome and elevated ferritin serum level with low glycosylated ferritin. Adult-onset Still's disease was diagnosed, but treatment with steroids did not achieve remission. The patient was then treated with anakinra, which resulted in spectacular improvement, but 3 weeks after the initiation of the treatment she experienced severe hepatitis that resolved after the discontinuation of anakinra. CONCLUSION: Hepatitis is a rare side effect of anakinra and the monitoring of liver tests should be recommended during anakinra therapy.
25715674 Synthetic polymer as an adjuvant in collagen-induced arthritis. 2014 Mar 21 Collagen-induced arthritis (CIA), the classical animal model for experimental arthritis, resembles human rheumatoid arthritis in several aspects. However, the most widely used method of inducing CIA utilizes Freund's adjuvants, which can skew the elicited immune responses and also pose toxicity problems. This unit describes a new method of inducing CIA using a well defined stimuli-responsive synthetic polymer, poly-N-isopropylacrylamide-based adjuvant, mixed with the joint cartilage protein collagen type II (CII). PNiPAAm as an adjuvant is biodegradable and biocompatible, and does not skew immune responses. Thus, it is helpful in the development of arthritis models for studying antigen and tissue -specific autoimmune responses in an unbiased manner. This model is valuable for analyzing disease pathways, positional identification of genes regulating arthritis, validation of existing therapies, and exploring new therapeutic targets. Furthermore, this newly developed PNiPAAm adjuvant allows investigation of disease induction using specific autoantigens in several autoimmune diseases independently of toll-like receptors, as well as optimization of vaccine delivery systems for infectious diseases.
24458854 A case of dermatomyositis with secondary Sjögren's Syndrome-diagnosis with follow-up stud 2013 Dec PURPOSE: To report a case of dermatomyositis (DM) with secondary Sjögren's syndrome (SS) and propose the clinical application of technetium-99m pyrophosphate ((99m)Tc-PYP) scan. CASE REPORT: A 50-year-old woman had progressive proximal muscle weakness of bilateral thighs, myalgia, tea-colored urine, and exercise intolerance for 6 months. Physical examination showed malar rash, V-sign, periungual erythema, and mechanic hands. Neurological assessment showed symmetric pelvic-girdle weakness, myopathic face, waddling gait, but preserved deep tendon reflex and sensory functions. DM was diagnosed on the basis of typical rashes and serum creatinine kinase elevation (7397 IU/L). Aside from myopathic symptoms, dry eye and mouth were reported. Thorough autoantibody searches showed positive anti-SSA/Ro antibody (198 U/ml). Both Schirmer's test and sialoscintigraphy were positive, leading secondary SS as diagnosis. Initial (99m)Tc-PYP scan revealed increased radiouptake in the muscles of bilateral thighs, compatible with clinical assessment. Followup scan three months later shows abnormal but attenuated radiouptake at bilateral thighs, in the presence of nearly-complete clinical recovery. CONCLUSION: DM with secondary SS in adult is a unique disease entity, with predominantly myopathic symptoms and satisfactory therapeutic response as its characteristics. Our serial muscle imaging studies suggest that (99m)Tc-PYP scan is at once anatomically-specific and persistently-sensitive to microstructural damages within inflammatory muscles, enabling clinician to monitor disease activity and therapeutic response.
23752063 Analysis of tear cytokines and clinical correlations in Sjögren syndrome dry eye patients 2013 Aug PURPOSE: To compare concentrations of tear cytokines in 3 groups composed of Sjögren syndrome (SS) dry eye, non-Sjögren syndrome (non-SS) dry eye, and normal subjects. Correlations between ocular surface parameters and tear cytokines were also investigated. DESIGN: Prospective cross-sectional study. METHODS: SS dry eye patients (n = 24; 40 eyes) were diagnosed with primary SS according to the criteria set by the American-European Consensus Group. Non-SS dry eye patients (n = 25; 40 eyes) and normal subjects (n = 21; 35 eyes) were also enrolled. Tear concentrations of interleukin (IL)-17, IL-6, IL-10, IL-4, IL-2, interferon γ (IFN-γ), and tumor necrosis factor α (TNF-α) were measured by a multiplex immunobead assay. Ocular Surface Disease Index (OSDI), tear film breakup time (TBUT), Schirmer I test, and fluorescein staining scores were obtained from dry eye patients. RESULTS: All cytokine levels except for IL-2 were highest in the SS group, followed by non-SS dry eye group and control subjects. Concentrations of IL-17, TNF-α, and IL-6 were significantly different among the 3 groups (IL-17: SS > control P < .001, non-SS > control P = .042, SS > non-SS P < .001; TNF-α: SS > control P = .006, non-SS > control P = .034, SS > non-SS P = .029; IL-6: SS > control P = .002, non-SS > control P = .032, SS > non-SS P = .002). IL-17 was significantly correlated with TBUT (R = -0.22, P = .012) and Schirmer I test (R = -0.36, P = .027) scores in the SS group. IL-6 was significantly correlated only with TBUT (R = -0.38, P = .02) in the non-SS group. CONCLUSIONS: Differences in tear cytokine levels and correlation patterns between SS dry eye and non-SS dry eye patients suggest the involvement of different inflammatory processes as causes of dry eye syndrome.
24392743 The forced oscillation technique is a sensitive method for detecting obstructive airway di 2014 OBJECTIVES: To study signs of obstructive airway disease (OAD) in patients with primary Sjögren's syndrome (pSS) using the forced oscillation technique (FOT). METHOD: Thirty-seven female pSS patients (median age 64, range 38-77 years) without previous physician-diagnosed OAD, participating in a longitudinal follow-up study of pulmonary function, and 74 female population-based controls (median age 64, range 47-77 years), also without physician-diagnosed OAD, and matched with regard to age, height, weight, and tobacco consumption, were included in the study. The pSS patients and controls were studied by the FOT, evaluating resistance and reactance of the respiratory system. RESULTS: pSS patients had significantly increased resistances at 5-25 Hz, decreased reactance at 10-35 Hz, and an increased resonant frequency (Fres) in comparison with controls. Resistance was correlated negatively and reactance positively to the vital capacity (VC), the forced expiratory volume in 1 s (FEV1), and the diffusing capacity for carbon monoxide (DLCO). Compared with controls, pSS patients with (n = 14) and without OAD (n = 21), as determined by spirometry, had significantly increased resistances at 5-25 Hz and decreased reactances at 10-35 Hz. In never-smoking subjects, identical FOT signs were found. CONCLUSIONS: pSS patients showed FOT signs of obstruction affecting both peripheral and central airways. pSS patients without spirometric signs of OAD and never-smoking pSS patients also showed clear FOT signs of obstruction. FOT therefore seems to be a sensitive method for detecting obstruction in pSS patients.
23624554 Adult-onset Still's disease and pregnancy: about ten cases and review of the literature. 2014 Jun Little is known about the interactions between adult-onset Still's disease (AOSD) and pregnancy. In an attempt to clarify the link between these 2 conditions, we retrospectively analyzed patients registered as suffering from AOSD seen in our university hospital. A total of 57 patients, among them 30 women, were diagnosed. Ten pregnancies in 8 women were identified. Three cases manifested AOSD in their first trimester, all treated with prednisone. Premature births and flares occurred in 2 patients. One patient developed a monocyclic AOSD during her second pregnancy's postpartum. In the 4 other cases, AOSD was known and quiescent before pregnancy. One patient had 2 pregnancies without any flare or complication. One patient experienced her first pregnancy while under treatment and presented a late flare 8 months after delivery. The third patient developed exacerbation in the first trimester of her second pregnancy which was treated with IgIV alone. The last one presented her first pregnancy 7 years after diagnosis. A prednisone-treated systemic flare occurred during the first trimester without later complication. Based on our own experience and the analysis of only two series of the literature, including, respectively, 4 and 5 patients, we suggest that two settings could be distinguished. First, AOSD can occur during pregnancy and can be responsible for obstetrical complications. Then, in patients with known AOSD, the second trimester and postpartum appear to be periods exposing to disease recurrence. Thus, we recommend a close multidisciplinary monitoring by a rheumatologist and an obstetrician prior to, during and after pregnancies.
24931957 Female sex and oligoarthritis category are not risk factors for uveitis in Italian childre 2014 Jul OBJECTIVE: To investigate the risk factors for chronic anterior uveitis in patients with juvenile idiopathic arthritis (JIA). METHODS: The clinical charts of patients followed between January 1987 and December 2011 were reviewed to establish whether they had uveitis. Inclusion criteria were a diagnosis of JIA and a disease category of persistent oligoarthritis, extended oligoarthritis, rheumatoid factor-negative polyarthritis, psoriatic arthritis, or undifferentiated arthritis. Risk factors included sex, age at arthritis onset, disease category, and antinuclear antibody (ANA) status. The association of risk factors with occurrence of uveitis was evaluated by survival analysis, with first episode of uveitis as the event of interest, and Cox regression analysis. RESULTS: Of a total of 1189 patients, 278 (23.4%) had uveitis a median of 1.1 years after onset of arthritis. There was no difference in the cumulative probability of developing uveitis between males and females and between patients belonging to different JIA categories, whereas uveitis was strongly associated with age at arthritis onset ≤ 3.5 years and positive ANA. Patients possessing the latter 2 factors in combination had a greater probability of having uveitis than patients who had either of them alone. CONCLUSION: In our patients, the risk of uveitis was related to younger age at onset of arthritis and presence of ANA, but not to female sex and disease category. This finding suggests that the patients who require the most intensive ophthalmologic screening are those who have early-onset JIA and are ANA-positive, regardless of their sex or disease subtype.
24287199 Coping strategies and support networks for Sjögren's syndrome patients. 2014 Feb Sjögren's syndrome is a chronic systemic autoimmune disease that can affect any organ system in the body. The most common symptoms are dryness of the mouth and eyes resulting from chronic inflammation and a progressive loss of secretory function. As with most individuals managing a chronic condition, patients with Sjögren's are on a multipronged path to disease and symptom management. Various coping strategies are presented in this article and the advantages and disadvantages discussed. Additionally, how a support group functions and practical guidance for the initiation of a Sjögren's support group are discussed.
25183244 Adult onset Still's disease (AOSD) in the era of biologic therapies: dichotomous view for 2014 Nov Adult onset Still's disease (AOSD) is a rare inflammatory disorder characterized by hectic spiking fever, evanescent rash and joint involvement. Prognosis is highly variable upon disease course and specific involvements, ranging from benign and limited outcome to chronic destructive polyarthritis and/or life-threatening events in case of visceral complications or reactive hemophagocytic lymphohistiocytosis (RHL). AOSD remains a debatable entity at the frontiers of autoimmune diseases and autoinflammatory disorders. The pivotal role of macrophage cell activation leading to a typical Th1 cytokine storm is now well established in AOSD, and confirmed by the benefits using treatments targeting TNF-α, IL-1β or IL-6 in refractory patients. However, it remains difficult to determine predictive factors of outcome and to draw guidelines for patient management. Herein, reviewing literature and relying on our experience in a series of 8 refractory AOSD patients, we question nosology and postulate that different cytokine patterns could underlie contrasting clinical expressions, as well as responses to targeted therapies. We therefore propose to dichotomize AOSD according to its clinical presentation. On the one hand, 'systemic AOSD' patients, exhibiting the highest inflammation process driven by excessive IL-18, IL-1β and IL-6 production, would be at risk of life-threatening complications (such as multivisceral involvements and RHL), and would preferentially respond to IL-1β and IL-6 antagonists. On the other hand, 'rheumatic AOSD' patients, exhibiting pre-eminence of joint involvement driven by IL-8 and IFN-γ production, would be at risk of articular destructions, and would preferentially respond to TNF-α blockers.
24448118 Prevalence and clinical patterns of psoriatic arthritis in Indian patients with psoriasis. 2014 Jan BACKGROUND: The prevalence and clinical patterns of psoriatic arthritis (PsA) varies in different parts of the world and there is little clinical and epidemiological data from the Indian subcontinent. AIMS: Our study was designed to evaluate the prevalence and clinical patterns of PsA in Indian patients. METHODS: This was a non-interventional, cross-sectional study, in which 1149 consecutive psoriasis patients seen over 1 year were screened for PsA according to classification of psoriatic arthritis (CASPAR) criteria. Demographic and disease parameters were recorded including Psoriasis Area and Severity Index (PASI), Nail Psoriasis Severity Index (NAPSI), and number of swollen and tender joints. RESULTS: Among 1149 patients with psoriasis, 100 (8.7%) patients had PsA, of which 83% were newly diagnosed. The most common pattern was symmetrical polyarthritis (58%), followed by spondyloarthropathy 49%, asymmetric oligoarthritis (21%), isolated spondyloarthropathy (5%), predominant distal interphalangeal arthritis (3%), and arthritis mutilans (1%). Enthesitis and dactylitis were present in 67% and 26% of cases, respectively. The mean number of swollen and tender joints were 3.63±3.59 (range, 0-22) and 7.76±6.03 (range, 1-26), respectively. Nail changes were present in 87% of the cases. The median PASI and NAPSI of the subjects with PsA was 3.6 and 20, respectively. There was no significant correlation of number of swollen/tender joints with PASI or NAPSI. CONCLUSION: There is a relatively low prevalence of PsA among Indian psoriasis patients presenting to dermatologists. No correlation was found between the severity of skin and nail involvement and articular disease.
24707285 Steroids Decrease Prevalence of Positive Tuberculin Skin Test in Rheumatoid Arthritis: Imp 2014 Tuberculin skin test has been used as an indicator of latent tuberculosis in patients with Rheumatoid Arthritis (RA) before administration of biologicals. Effect of Disease modifying antirheumatic drugs (DMARDs) and steroids on the result of tuberculin skin test (TST) may have important implications in interpretation of results of this test. Objectives. To find the prevalence of positive TST in rheumatoid patients and the effect of standard treatment on the results of TST. Method. In this cross-sectional study two hundred and fifty patients of RA above 18 years of age, classified using 1987 ACR criteria for RA, were enrolled from rheumatology outdoor. Demographics, disease activity, disease duration, and therapy were recorded. All patients underwent TST. Results. Fifty-one (20.4%) patients were found to be tuberculin positive. Tuberculin positivity was not affected by MTX intake but it was significantly low in patients with recent steroid intake as compared to patients who had not taken steroids in last 3 months (3% versus 25%, P = 0.002). Conclusion. Prevalence of tuberculin positivity in patients with RA was found to be low. Results were not affected by methotrexate; however tuberculin skin test results in patients with recent use of steroids are likely to be negative.
24371442 Controlled Cohort Study of Serum Gonadal and Adrenocortical Steroid Levels in Males Prior 2013 Serum testosterone levels are generally reported to be lower in male rheumatoid arthritis (RA) patients, but it is not determined if a deficiency may occur before clinical onset of disease (pre-RA). Lower testosterone levels were recently reported in males many years before RA onset but were predictive only of rheumatoid factor (RF)-negative disease. A preceding prospective study did not reveal androgenic-anabolic hormone association with risk of RA in men or women. This cohort study of males analyzed baseline serum levels of gonadal and adrenocortical steroids, luteinizing hormone, and prolactin in 18 pre-RA versus 72 matched non-RA control (CN) subjects. Findings in males were compared to those in female pre-RA and CN subjects in the same cohort, and sex differences were analyzed. Steroidal and hormonal levels, including total testosterone, were similar between male study groups. In females, mean (±SE) serum androstenedione (nmol/L) was slightly (P = 0.048) lower in 36 pre-RA (6.7 ± 0.36) than 144 CN (7.6 ± 0.22). With the exception of 3 partial correlations of hormonal variables observed to differ between pre-RA versus CN subjects, the patterns were similar overall. However, partial correlations of hormonal variables differed frequently by sex, both within and between study groups.
24840678 Smoking cessation advice by rheumatologists: results of an international survey. 2014 Oct OBJECTIVE: The aim of this study was to understand practices regarding smoking cessation among rheumatologists for patients with inflammatory rheumatic diseases. METHODS: A survey was sent to the rheumatologists participating in the multinational Quantitative Standard Monitoring of Patients with Rheumatoid Arthritis (QUEST-RA) group. The survey inquired about the clinical practice characteristics and practices regarding smoking cessation (proportion of smokers with inflammatory rheumatic diseases given smoking cessation advice, specific protocols and written advice material, availability of dedicated smoking cessation clinic). RESULTS: Rheumatologists from 44 departments in 25 countries (16 European) completed the survey. The survey involved 395 rheumatologists, of whom 25 (6.3%) were smokers, and 199 nurses for patient education, of whom 44 (22.1%) were smokers. Eight departments (18.1 %) had a specific protocol for smoking cessation; 255 (64.5%) rheumatologists reported giving smoking cessation advice to all or almost all smokers with inflammatory diseases. In a regression model, early arthritis clinics (P = 0.01) and high gross domestic product countries (P = 0.001) were both independently associated with advice by the rheumatologist. Nurse gives advice to most patients in 11 of the 36 (30.5%) departments with nurses for patient education. CONCLUSION: Advice for smoking cessation within rheumatology departments is not homogeneous. In half of the departments, most doctors give advice to quit smoking to all or almost all patients with inflammatory diseases. However, only one in five departments have a specific protocol for smoking cessation. Our data highlight the need to improve awareness of the importance of and better practice implementation of smoking cessation advice for inflammatory rheumatic disease patients.
24252506 Polymorphisms of Toll-like receptor-4 and CD14 in systemic lupus erythematosus and rheumat 2013 May 16 BACKGROUND: Toll-like receptor 4 (TLR4) and its co-receptor CD14 play a major role in innate immunity by recognizing PAMPs and signal the activation of adaptive responses. These receptors can recognize endogenous ligands mainly auto-antigens. In addition, TLR4 (Asp299Gly) and CD14 (C/T -159) polymorphisms (SNPs) may modify qualitatively and/or quantitatively their expression. Therefore, they could be implied in autoimmune diseases and can influence both susceptibility and severity of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). PATIENTS AND METHODS: TLR4 (Asp299Gly) and CD14 (C/T -159) SNPs were genotyped using polymerase chain reaction (PCR)-RFLP in 127 SLE patients, 100 RA patients, and 114 healthy controls matched in age and gender. RESULTS: CD14*T allele was significantly more frequent in SLE patients (0.456) comparatively to controls (0.355), p = 0.02 OR (95% CI) = 1.53 [1.04-2.24]. In RA patients, the higher frequency of CD14*T allele (0.405) failed to reach significance, p = 0.28. Investigation of the TLR4 (Asp299Gly) SNP showed no significant association neither with SLE nor with RA.Analysis of these SNPs according to clinical and biological features showed a significant higher frequency of arthritis in SLE patients carrying CD14*T/T genotype (92%) comparatively to those with C/C and C/T genotypes (72.5%), p = 0.04. Moreover, SLE patients carrying CD14*T/T/TLR4*A/A haplotype had significantly more arthritis (91.3%) than the rest of SLE group (73%), p = 0,044 and confirmed by multivariable analysis after adjustment according to age and gender, p = 0.01. CONCLUSION: The CD14 (-159)*T allele seems to be associated with susceptibility to SLE and arthritis occurrence.
25125695 FHL2 regulates the resolution of tissue damage in chronic inflammatory arthritis. 2015 Dec OBJECTIVE: We analysed the role of the adaptor molecule four-and-a-half Lin11, Isl-1 & Mec-3 (LIM) domain protein 2 (FHL2) in the activation of fibroblast-like synoviocytes in human rheumatoid arthritis (RA) and tumour necrosis factor α (TNFα)-dependent animal models of the disease. METHODS: Synovial tissues of patients with RA and osteoarthritis (OA) as well as hind paw sections from arthritic human TNFα transgenic (hTNFtg) mice and synovial fibroblasts from these were analysed. The effects of cytokines on the expression of FHL2 and disease-relevant matrixmetalloproteases (MMPs) were determined. Analyses of human tissue specimens from patients treated with anti-TNFα as well as anti-TNFα treatment of hTNFtg mice were performed to substantiate the TNFα effects on FHL2 levels. FHL2(-/-) mice and hTNFtg mice (with constitutive or inducible transgene expression) were crossbred to generate TNFα overexpressing FHL2-deficient animals. Signalling pathways were analysed in cells from these mice and in human cells after knock down of FHL2 by western blot. RESULTS: FHL2 levels were higher in RA than in OA and in hTNFtg than in wild-type mice. Surprisingly, while transforming growth factor (TGF)β-induced FHL2 expression, TNFα suppressed FHL2. In vivo, anti-TNFα treatment led to higher FHL2 levels both in RA patients and hTNFtg mice. The loss of FHL2 increased joint destruction in hTNFtg mice, which was accompanied by elevated MMP-13. In vitro, TNFα-mediated MMP-13 was significantly higher in FHL2(-/-) cells and after knock down of FHL2, which was caused by prolonged p38 MAPK activation. CONCLUSIONS: These data suggest that FHL2 serves as a protective factor and that, rather than promoting the pathology, the upregulation of FHL2 in RA occurs in frame of a regenerative attempt.
25052974 Clinical and biochemical characteristics of children with juvenile idiopathic arthritis. 2014 Jul OBJECTIVE: To determine the clinical and biochemical characteristics of children with Juvenile Idiopathic Arthritis (JIA) at a tertiary care centre in Karachi, Pakistan. STUDY DESIGN: A descriptive study. PLACE AND DURATION OF STUDY: Paediatric Rheumatology Clinic of The Aga Khan University Hospital (AKUH), Karachi, from January 2008 to December 2011. METHODOLOGY: Clinical and laboratory profile and outcome of children less than 15 years of age attending the Paediatric Rheumatology Clinic of the Aga Khan University, Karachi with the diagnosis of Juvenile Idiopathic Arthritis according to International League against Rheumatism were studied. These children were classified into different types of JIA; their clinical and laboratory characteristics, response to therapy and outcome was evaluated. RESULTS: Sixty eight patients satisfying the criteria of International League against Rheumatism (ILAR) for Juvenile Idiopathic Arthritis were enrolled during the study period of four consecutive years, their age ranged from 9 months to 15 years. Mean age at onset was 6.45 ± 4.03 years while mean age at diagnosis was 7.60 ± 3.93 years. Polyarticular was the most predominant subtype with 37 (54%) patients, out of these, 9 (24%) were rheumatoid factor positive. An almost equal gender predisposition was observed. Fever and arthritis were the most common presenting symptoms, with only 2 patients presenting with uveitis. CONCLUSION: The clinico-biochemical characteristics of JIA at the study centre showed a pattern distinct with early onset of disease, high frequency of polyarticular type and a higher rheumatoid factor (QRA) and ANA positivity in girls.
23664546 AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis. 2013 Oct We report a 12-year-old boy with nephrotic syndrome due to renal AA amyloidosis. The AA amyloidosis was associated with a 3-year history of systemic-onset juvenile idiopathic arthritis. The presence of serum amyloid A protein was confirmed by laser microdissection of Congo Red-positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry; this analysis excluded hereditary and familial amyloidosis. Aggressive management of the systemic-onset juvenile idiopathic arthritis resulted in improvement in clinical and laboratory parameters. The case represents an unusual cause of nephrotic syndrome in children. Early diagnosis of renal amyloidosis and management of systemic-onset juvenile idiopathic arthritis is paramount to preventing progression of kidney disease.
25205184 Biological agents in psoriatic arthritis. 2015 Jan Anti-tumor necrosis factors (TNFs) are effective drugs for the treatment of psoriatic arthritis (PsA) regarding reduction of pain and inflammation, enthesitis, dactylitis, as well as psoriatic skin and nail disease. Moreover, radiographic progression in PsA is decelerated. The efficacy of anti-TNFs seems to be independent of synthetic disease-modifying anti-rheumatic drugs, suggesting only a minor role of combination therapy in PsA. Anti-TNFs are generally well tolerated in patients with PsA. Adverse events are similar to those reported in patients with rheumatoid arthritis. Ustekinumab, a recently approved IL-12/IL-23-antibody is another promising biological agent in the treatment of PsA.