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ID PMID Title PublicationDate abstract
14966357 Adult-onset Still's disease with disseminated intravascular coagulation and multiple organ 2004 Feb Severe systemic manifestations of adult onset Still's disease (AOSD) are often fatal and occasionally related to hemophagocytic syndrome (HS). We describe the case of a 49-yr-old woman with AOSD presenting with non-remitting high fever, confusion, jaundice, hepatosplenomegaly, serositis, azotemia, pancytopenia, coagulopathy with disseminated intravascular coagulation (DIC), hyperferritinemia, acute acalculous cholecystitis and ileocolitis noted in computed tomographic images. The patient had a history of herpes zoster developed prior to the admission, but there is no history of diarrhea or abdominal pain. Although bone marrow examination was not performed due to hemorrhagic diathesis, we suspected AOSD-associated HS on the basis of clinical course without detectable infectious agents in cultures or serologic studies. Intravenous immunoglobulin, pulse methylprednisolone, oral cyclosporine A (CsA) and ceftriaxone brought about transient improvement of fever and confusion, but the disease progressed. After increasing CsA dose, all previously mentioned abnormalities disappeared rapidly. Accordingly, we believe that DIC and multiple organ dysfunctions might have been the complications of HS but not that of sepsis, and that CsA can be used as a first-line therapy in case of life-threatening situations.
12771972 Copper and zinc intake and serum levels in patients with juvenile rheumatoid arthritis. 2003 May OBJECTIVE: To evaluate the copper and zinc intake and serum levels in patients with juvenile rheumatoid arthritis (JRA), considering the pauci and polyarticular types, the disease activity and duration, the number of inflamed joints and the use of corticosteroids therapy. DESIGN: Cross-sectional study with control group. SETTING: Outpatients of the pediatric rheumatology public health clinic, of the Universidade Federal de São Paulo/Escola Paulista de Medicina, Brazil. SUBJECTS: Forty-one patients with JRA were evaluated and 23 patients' brothers, as a control group. INTERVENTIONS: Copper and zinc intake evaluation by Food Register method. Copper and zinc serum levels by atomic absorption spectrophotometry. RESULTS: The disease activity did not determine difference in copper (P=0.624) and zinc (P=0.705) intake, being predominantly below the Recommended Dietary Allowances. The serum copper in relation to control was statistically greater (P=0.018), showing that the number of inflamed joints is statistically significantly related with its variation (P=0.001). The serum zinc was not different either in relation to control (P=0.940) or to the disease characteristics. CONCLUSIONS: The evaluation of copper intake seems to be of fundamental importance. It may influence the efficiency of the organic serum response. More research is needed to indicate, with security, adequate zinc intake.
12657826 [A case of auto-immune hepatitis associated with primary Sjogren's syndrome]. 2003 Mar Auto-immune hepatitis is a chronic necroinflammatory liver disorder that is characterized by hypergammaglobulinemia, auto-antibodies in serum, and, on histological examination, the presence of periportal hepatitis. Although it can be associated with a number of other auto-immune diseases, Sjogren's syndrome is rarely associated with auto-immune hepatitis. We herein report an unusual case of auto-immune hepatitis associated with primary Sjogren's syndrome. A 39-year-old woman was admitted to our hospital due to jaundice. Laboratory data showed negative viral hepatitis marker, increased serum IgG level, positive anti- nuclear antibody, and an increased rheumatoid factor titer. The patient had no history of taking medications and alcohol. Based on characteristic clinical features, liver biopsy findings, positive Schirmer's test, and salivary scintigraph, she was diagnosed as having auto-immune hepatitis and Sjogren's syndrome. The patient achieved complete remission with steroid monotherapy.
15129233 Genetic association between juvenile rheumatoid arthritis and polymorphism in the SH2D2A g 2004 Jun T-cell-specific adapter protein (TSAd) involved in the negative control of T-cell activation is encoded by the SH2D2A gene. Our recent studies indicate that homozygosity for short (ie GA(13) and GA(16)) alleles of the SH2D2A gene promoter is associated with development of multiple sclerosis. To study whether the same SH2D2A promoter polymorphism also contributes to the genetic susceptibility to develop juvenile rheumatoid arthritis (JRA), we examined 210 JRA patients and 558 healthy unrelated controls from Norway. The frequency of the short allele GA(13) was increased among the JRA patients compared to control (0.098 vs 0.05; P(n=8)=0.042). There was a significant increased frequency of HLA-DRB1(*)08-positive patients carrying two copies of 'short' alleles GA(13) and/or GA(16) compared to healthy controls (16% vs 6%; P(n=4)=0.016). Our data indicate that the 'short' alleles of the SH2D2A promoter could contribute to the genetic susceptibility to JRA.
12582392 Sézary syndrome and seronegative polyarthritis: treatment with extracorporeal photochemot 2003 Feb We describe a patient with therapy-resistant cutaneous T-cell lymphoma, Sézary syndrome variant, in association with concurrent polyarthritis and vitiligo, who was successfully treated with extracorporeal photochemotherapy (ECP). The combination of Sézary syndrome with seronegative rheumatoid arthritis is rare. In our patient the T-cell lymphoma was refractory to standard treatments that included psoralen-UVA, lymph node irradiation, and polychemotherapy. ECP has been shown to be effective in the treatment of selected cases of Sézary syndrome. There is a strong suggestion that ECP as a monotherapy can provide a significant benefit for other T-cell-mediated diseases including rheumatoid arthritis. In spite of a disease duration of 10 years, a very low CD8 cell count (2% of lymphocytes), a very high CD4 cell count (94%), and multiple unsuccessful chemotherapeutic trials before initiation of ECP, our patient achieved a long-lasting complete remission of both diseases with normalization of the CD4+ and CD8+ T-lymphocyte subsets. Concurrent developing vitiligo was unaffected by ECP.
14696006 In-vivo high resolution three-dimensional MRI studies of rat joints at 7 T. 2003 Dec It is important to obtain high resolution images of joints for the study of disease, especially in rodent experimental models. We optimized (1)H magnetic resonance imaging three-dimensional sequences at 7 T, with lipid signal suppression, and T(1) and T(2) measurements for in-vivo experiments on rat joints, in order to assess the effectiveness of high-field MRI. The method was validated by applying it to the early diagnosis of arthritis. We studied the progress of rheumatoid arthritis in an arthritic rat model. We observed the rats' knees for 21 days after inducing arthritis. The images acquired over one hour had a high resolution of 1.75 x 10(-3) mm(3), (105 x 105 x 145 microm(3)) which allowed us to spot the early stages of joint degeneration, such as bone erosion, and to observe an apparent 'MRI' loss of cartilage thickness, attributed to dehydration of the cartilage tissue. The MR images obtained during the early stages of rheumatoid arthritis enabled us to study joint changes accurately before any histological signs of attack were visible.
17656934 Pharmacological interventions for psoriatic arthritis: present and future. 2002 Jul Psoriatic arthritis is a common, chronic inflammatory arthritis that affects 5-7% of patients with psoriasis. Psoriatic arthritis is often treated in the same manner and with the same drugs as rheumatoid arthritis is. Currently much attention is being focused on the role of agents that inhibit the activity of pro-inflammatory cytokines, such as tumor necrosis factor a, which are believed to play a key role in joint destruction. The objective of this article is to review these newer developments besides taking a fresh look at the existing methods of therapy of this potentially disabling disorder.
11824955 Lack of association between juvenile idiopathic arthritis and fas gene polymorphism. 2002 Jan OBJECTIVE: Juvenile idiopathic arthritis (JIA) is a complex genetic disease of autoimmune etiology. Fas is a molecule with a pivotal role in apoptosis and hence in immune regulation. Elevated transcriptional levels of Fas in the synovial fluid of patients with JIA suggest that it might be implicated in disease etiopathogenesis. We investigated whether a polymorphism in the Fas promoter region (-670) confers susceptibility to JIA. METHODS: In this association study, 342 UK patients with JIA and 255 healthy individuals were genotyped for the polymorphism using polymerase chain reaction restriction fragment length polymorphism. Comparisons of the genotypic frequencies were made using chi-square analysis. RESULTS: No statistically significant differences were found when the genotype frequencies of the -670 Fas polymorphism were compared between the JIA cases and the control panel. Similarly, no differences were seen between the JIA subgroups, or when the patients were divided on the basis of rheumatoid factor or antinuclear antibody positivity. CONCLUSION: The -670 polymorphism of Fas does not appear to be associated with susceptibility to JIA.
15801077 Severe carpal tunnel syndrome in a patient with juvenile idiopathic arthritis due to proxi 2004 Dec We report a new case of pediatric carpal tunnel syndrome in a patient with juvenile rheumatoid arthritis. Symptoms were mainly motor weakness and severe atrophy of the thenar.
11995256 [Classification criteria and clinical picture of juvenile idiopathic arthritis]. 2002 Feb In recent years a new nomenclature and classification childhood chronic arthritis has been introduced. The terms juvenile chronic arthritis and juvenile rheumatoid arthritis--have been replaced by juvenile idiopathic arthritis (JIA). The term JIA was adapted to indicate a childhood onset (before the 16th. birthday) characterized primarily by arthritis lasting for at least 6 weeks, and the cause haven't been found so far. In this paper the diagnosis and clinical course systemic arthritis, polyarthritis and oligoarthritis were characterized. The current therapeutic approach to JIA consists a complex management as the administration of antiinflammatory, immunosuppressive and immunomodulatory drugs--as well as rehabilitation.
15642140 Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rhe 2005 Macrophage activation syndrome (MAS) has been reported in association with many rheumatic diseases, most commonly in systemic juvenile rheumatoid arthritis (sJRA). Clinically, MAS is similar to hemophagocytic lymphohistiocytosis (HLH), a genetic disorder with absent or depressed natural killer (NK) function. We have previously reported that, as in HLH, patients with MAS have profoundly decreased NK activity, suggesting that this abnormality might be relevant to the pathogenesis of the syndrome. Here we examined the extent of NK dysfunction across the spectrum of diseases that comprise juvenile rheumatoid arthritis (JRA). Peripheral blood mononuclear cells (PBMC) were collected from patients with pauciarticular (n = 4), polyarticular (n = 16), and systemic (n = 20) forms of JRA. NK cytolytic activity was measured after co-incubation of PBMC with the NK-sensitive K562 cell line. NK cells (CD56+/T cell receptor [TCR]-alphabeta-), NK T cells (CD56+/TCR-alphabeta+), and CD8+ T cells were also assessed for perforin and granzyme B expression by flow cytometry. Overall, NK cytolytic activity was significantly lower in patients with sJRA than in other JRA patients and controls. In a subgroup of patients with predominantly sJRA, NK cell activity was profoundly decreased: in 10 of 20 patients with sJRA and in only 1 of 20 patients with other JRA, levels of NK activity were below two standard deviations of pediatric controls (P = 0.002). Some decrease in perforin expression in NK cells and cytotoxic T lymphocytes was seen in patients within each of the JRA groups with no statistically significant differences. There was a profound decrease in the proportion of circulating CD56bright NK cells in three sJRA patients, a pattern similar to that previously observed in MAS and HLH. In conclusion, a subgroup of patients with JRA who have not yet had an episode of MAS showed decreased NK function and an absence of circulating CD56bright population, similar to the abnormalities observed in patients with MAS and HLH. This phenomenon was particularly common in the systemic form of JRA, a clinical entity strongly associated with MAS.
15194585 Autoantibodies against granulocyte-macrophage colony stimulating factor and interleukin-3 2004 Jul OBJECTIVES: Antibodies against granulocyte colony stimulating factor are frequently found in patients with Felty's syndrome (FS). In this study, we examined the prevalence of antibodies against two other granulopoietic cytokines: granulocyte-macrophage colony stimulating factor (GM-CSF) and interleukin-3 (IL3). METHODS: Sera of 32 patients with FS, 20 normocytic patients with rheumatoid arthritis (RA), and 72 healthy individuals were screened for the presence of antibodies against GM-CSF and IL3 by ELISA and bioassays, using the human erythroleukaemia cell line TF-1. RESULTS: In two of the 32 patients with FS, antibodies to GM-CSF and IL3 were detectable by ELISA. Binding anti-GM-CSF antibodies were also detected in one of the 72 healthy controls, while in another healthy subject and in one of the patients with normocytic RA, anti-IL3 antibodies were present. Serum from one of the two patients with FS who tested positive for anti-IL3 and anti-GM-CSF antibodies by ELISA showed strong neutralising capacity to the biological effect of IL3, but not to GM-CSF in vitro. Patients with FS had significantly higher serum levels of GM-CSF (median; 2.82 pg/mL; interquartile range 2.64-3.19 pg/mL) compared with patients with RA (2.52 pg/mL; 2.28-2.72 pg/mL; p = 0.012) and healthy controls (2.23 pg/mL; 2.04-2.52; p<0.001). In addition, serum levels of IL3 in patients were significantly higher in FS (10.05 pg/mL; 8.94-11.98) compared with controls (4.79 pg/mL; 3.72-7.22; p<0.001), but not compared with RA patients (9.52 pg/mL; 8.32-10.42; p = 0.17). CONCLUSIONS: Antibodies to GM-CSF and IL3 are rare in patients with FS and RA and in healthy subjects. In individual patients with FS, the presence of neutralising anti-IL3 antibodies may contribute to the development of cytopenia.
15293104 Pure red cell aplasia and adult-onset Still's disease. 2004 Aug Pure red cell aplasia (PRCA) associated with adult-onset Still's disease (AOSD) is very rare. In this report a 28-year-old woman was admitted with fever, skin rash, jaundice and anemia. She was diagnosed as having AOSD with PRCA by bone marrow examination. Treatment with high-dose prednisolone and intravenous immunoglobulin resulted in remission of the PRCA and a good response of the AOSD.
14749993 Lymphadenopathy in adult-onset Still's disease mimicking peripheral T-cell lymphoma. 2004 Feb Lymphadenopathy (LAP) that is seen in adult onset Still's disease (AOSD) may be confused with lymphoma. Here we present a patient with AOSD and with LAP that histopathologically mimicked T-cell lymphoma.
12849002 Viral etiopathogenesis of Sjögren's syndrome: role of the hepatitis C virus. 2002 Aug Patients with hepatitis C virus (HCV) chronic infection present some extrahepatic manifestations that may mimic the clinical, immunologic and histological manifestations of primary Sjögren's syndrome (SS). Thus, HCV patients with sicca symptomatology and positive autoantibodies could be misdiagnosed as a 'primary' SS. Nevertheless, there are several clinical and immunologic features that could help us differentiate both processes.
15461768 A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adoles 2004 Sep The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. Adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.
12209502 Mortality in early inflammatory polyarthritis: cardiovascular mortality is increased in se 2002 Aug OBJECTIVE: To determine the degree and causes of any excess mortality observed during the early years of inflammatory polyarthritis (IP). METHODS: Between 1990 and 1994, a total of 1,236 patients were registered with the Norfolk Arthritis Register, a primary care-based inception cohort. All patients were tracked on the National Health Service Central Register for notification of death. The vital status of each patient was determined as of December 31, 1999. Causes of death were coded according to the International Classification of Diseases, Ninth Revision. Expected death rates were calculated using annual death rates for the Norfolk population. Standardized mortality ratios (SMRs) were calculated for all IP patients and for the subgroups of patients who did and did not satisfy the American College of Rheumatology (ACR) 1987 criteria for rheumatoid arthritis (RA) at baseline, as well as for the subgroups who were and were not rheumatoid factor (RF) positive at baseline. RESULTS: By December 31, 1999, 160 patients (13%; 79 women and 81 men) had died. The median duration of followup in the entire cohort was 6.9 years. Mortality rates were not significantly increased in the entire group of patients with IP or in the subgroup who met the ACR 1987 criteria for RA at baseline. In contrast, RF-positive patients had an increased rate of death from all causes (SMR in men 1.51, in women 1.41). Cardiovascular disease was the most common cause of death. The majority of the excess mortality in the RF-positive patients could be attributed to cardiovascular causes (SMR in men 1.34, in women 2.02). CONCLUSION: Excess mortality in the early years of IP is confined to patients who are seropositive for RF. While excess cardiovascular mortality has been described in patients with established RA, this is the first report of premature death from heart disease in the early years of IP.
12632432 A rare case of juxtaarticular osteoid osteoma of the calcaneus initially misdiagnosed as j 2003 Mar Juxtaarticular osteoid osteoma is frequently misdiagnosed because the symptoms may mimic arthritis, and radiographs may not be characteristic. A rare case of subtalar pain lasting 5 years in a female teenager is presented here. The initial diagnosis was monarticular juvenile chronic arthritis. Family history was misleading because her mother had rheumatoid arthritis (RA).
15124265 Embolic complications of a mitral valve rheumatoid nodule. 2004 May We describe a patient with a rheumatoid nodule on the mitral valve who developed embolic phenomena from an overlying thrombus. It is important to recognize that thrombus can develop on intracardiac rheumatoid nodules and that these patients may require anticoagulation.
12455820 Knee joint synovitis in Sjögren's syndrome. Sonographic study. 2002 Ultrasound detects effusion and synovial proliferation caused by synovitis. The study was undertaken to evaluate the signs of synovitis in patients with primary Sjögren's Syndrome (SS). Joint effusion was detected and synovial thickness was measured in the suprapatellar synovial bursa. Results have been compared with those obtained by sonographic assessment of knee joint in patients with secondary SS and RA. with secondary SS and connective tissue diseases, with RA, and in healthy subjects. Synovial thickening was demonstrated in all the diseases (higher grades of thickening were found in secondary SS with RA and in RA). Joint effusion was present with significantly higher frequency in secondary SS with RA and in RA. Results demonstrated signs of slight synovitis in primary SS. More severe synovitis was found both in secondary SS with RA and in RA. This is the first sonographic study demonstrating slight synovitis in primary SS.