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ID PMID Title PublicationDate abstract
11206347 Juvenile chronic arthritis in adult life: a study of long-term outcome in patients with ju 1999 We compared the prognostic factors and outcome of 30 patients with juvenile chronic arthritis (JCA) extending into adult life with those of 30 patients with adult rheumatoid arthritis (RA) at a university adult rheumatology clinic; pairs were matched for sex and duration of disease (mean 8 years). One-third of JCA patients had seronegative polyarticular disease and another third had oligoarticular disease. In a third of the JCA patients, the clinical presentation changed during the follow-up. Over half of the RA patients had seropositive polyarticular and a one-third had seronegative polyarticular disease. Fewer seropositive patients were recorded in the JCA group than in the RA group both at the beginning (16.7% versus 56.7%; p=0.003) and at the end of the follow-up (14.3% versus 59.3%; p=0.001). JCA patients developed less radiographic changes than RA patients (46.7% versus 76.7%; p=0.034); oligoarthritis in the JCA group had the best prognosis whereas seropositive polyarthritis in the RA group had the worst prognosis. Significantly more patients with JCA than RA (60% versus 23%; p=0.009) were in remission at the end of the follow-up. In conclusion, when studied in adult life, the long-term prognosis is better in patients with JCA than in those with RA.
24383735 Immune thrombocytopenic purpura associated with rheumatoid arthritis - a report of five ca 2001 Sep Abstract Immune thrombocytopenic purpura (ITP) associated with rheumatoid arthritis (RA) is relatively rare. We describe five cases of RA with ITP. In all five patients, platelet counts were low, platelet-associated IgG levels were elevated, and bone marrow aspiration showed megakaryocytosis. Glucocorticoid therapy was effective in three cases, but the other two cases required immunosuppressants or intravenous γ-globulin in addition to glucocorticoid. We review the reported cases of RA with ITP and discuss the pathophysiology and differential diagnosis of thrombocytopenia in RA.
14988764 Rheumatoid arthritis. 1998 Jul Rheumatoid arthritis (RA) is a common systemic, inflammatory, rheumatic disease of unknown etiology manifest by a chronic symmetric polyarthritis, particularly involving the small distal joints. Pathogenesis may involve inappropriate T-cell activation or lack of appropriate inhibition, although newer theories center on the importance of cytokines and membrane proteins that signal T-cells. Diagnosis is predicated on clinical assessment rather than extensive testing or exclusion of other illnesses, although well-known mimics of RA should be considered. The presence of objective synovitis in a "rheumatoid distribution" lasting more than 6 weeks is required for confident diagnosis. Treatment should be directed at maintaining function, reducing pain and preventing irreversible joint damage. Options include nonpharmacologic therapy, such as physical and occupational therapy, pharmacologic options such as analgesics, antiinflammatory therapies and second-line agents (or disease-modifying agents for rheumatic disease, DMARDs) and surgery. Newer approaches, including "biologics" aimed at interrupting cytokine effects and combinations of second-line agents, offer exciting potential for improving outcomes in RA. The prognosis of RA is highly variable and not reliably predictable in the individual patient early in disease.
9065621 Middle ear involvement in children with chronic rheumatoid juvenile arthritis. 1997 In a selected sample of patients affected by juvenile rheumatoid arthritis (JRA) little is known about middle ear involvement, even though many synovial joints are affected. Multifrequency tympanometry was used to measure admittance, conductance, susceptance and phase angle at different probe frequencies and resonant frequencies. In all, 35 children with JRA and a control group (30 children) were studied. Findings showed that mean resonant frequency values in all children with JRA were greater than in the control children. The multifrequency tympanometry parameters measured in acute JRA subjects are not different from parameters of remission JRA subjects except for a change in the phase angle. The changes found are due to involvement of the incudomalleolar and incudostapedial joints.
10321908 Histomorphometric assessment of bone changes in rats with type II collagen-induced arthrit 1999 May Numerous studies have demonstrated bone loss in rats following immobilization by tenotomy or nerve sectioning and following ovariectomy. However, few experiments have focused on bone change in rats with arthritis. We investigated bone loss in the proximal tibia and lumbar vertebra in rats with type II collagen-induced arthritis, an experimental model of rheumatoid arthritis, using histomorphometry. Bone loss in the early phase after immunization reflected a significant increase in numbers of osteoclasts and temporarily decreased bone formation. In the proximal tibia, near an arthritic joint, osteoclast numbers associated with bone trabeculae were increased four times over control numbers 4 weeks after immunization. In the lumbar vertebra, where arthritis was not shown, recruitment of osteoclasts occurred later than in the proximal tibia. With time, in both the proximal tibia and lumbar vertebra bone resorption normalized, but bone formation rate and double-label surface by tetracycline, a parameter reflecting bone formation, were increased above control values. We conclude that differences between the proximal tibia and lumbar vertebra probably reflected resumption of function as well as distance from areas of inflammation. These findings indicate that collagen-induced arthritis in rats is a useful model not only of autoimmunity, but also of juxta-articular and generalized osteoporosis in rheumatoid arthritis.
24387013 The SCID-HuRAg mouse as a model for rheumatoid arthritis. 2001 Mar Abstract Many animal models have been developed for a study of rheumatoid arthritis (RA). However, RA animal models are not always similar to RA patients in their response to antirheumatic drugs. Recently, humanized monoclonal antibody (mAb) has been developed for the treatment of RA, but at present there is no animal model on which to screen this mAb therapy because of problems with cross-reactivity. We therefore considered the development of a novel animal model for the screening of antirheumatic drugs using the severe combined immunodeficiency (SCID) mouse in order to prevent the rejection of human transplant cells. Following subcutaneous implantation of synovial tissue in the SCID mouse, all target cells within the SCID-HuRAg mouse were of human origin, having migrated from the implanted tissue. Moreover, human interlukin-6 and rheumatoid factor were detected in this mouse serum. We therefore propose that this SCID-HuRAg mouse is a novel, useful animal model for the study and development of new drugs for RA patients. This novel RA animal model is reviewed in this chapter.
10653005 Anti-very late antigen-1 monoclonal antibody modulates the development of secondary lesion 2000 Jan Rats injected in the hind paw with a mixture of Mycobacterium butirricum emulsified in mineral oil (FA) developed a severe polyarthritis that shared some immunological features with human rheumatoid arthritis. After this local administration, rats developed a secondary lesion (edema) in the contralateral paw, which is a hallmark of immune system activation. In vivo intravenous treatment with a monoclonal anti-very late antigen (VLA)-1 antibody (HA31/8) significantly reduced the edema formation in the contralateral paw. T cells isolated from contralateral paw draining lymph nodes of FA rats treated with HA31/8 showed a reduced cell proliferation in vitro, after stimulation with concanavalin A. Furthermore FACS analysis showed that the reduction in proliferation was concomitant to a reduction in the number of T cells positive to surface IL-2 receptor expression. Our data indicate that after in vivo treatment with a monoclonal anti-very late antigen-1 antibody, there is a beneficial effect on the development of the secondary lesion, which correlates to the reduced ability of T cells to proliferate in vitro as well as to a reduced surface expression of IL-2 receptor. The association of this antibody to other drugs interfering at other levels in rheumatoid arthritis may open a new therapeutic window.
9336414 Antibodies against a peptide sequence located in the linker region of the HMG-1/2 box doma 1997 Oct OBJECTIVE: To extend our work on the mapping of B cell epitopes on nucleosomal high mobility group (HMG) proteins in the sera of patients with juvenile rheumatoid arthritis (JRA). METHODS: Seventy-seven pauciarticular-onset JRA serum samples from antinuclear antibody (ANA)-positive patients and 42 polyarticular-onset JRA patient sera found to react with HMG-2 by immunoblotting were used in this study. To identify B cell epitopes on HMG-2, recombinant HMG-2 protein fragments were used in enzyme-linked immunosorbent assay (ELISA) and in competition ELISA experiments with a set of overlapping synthetic peptides. Fine epitope mapping was achieved by oligopeptide synthesis, followed by immunoblotting. RESULTS: Pauciarticular, but not polyarticular, JRA patient sera were found to recognize a lysine-rich major epitope (KKGKKKDP), which is located in the linker region of the HMG box domains of the HMG-2 nonhistone chromosomal protein. No significant immunoreactions were observed in sera from ANA-negative JRA patients and in sera from children with nonrheumatic diseases, indicating that this epitope seems to be specific for pauciarticular-onset JRA. CONCLUSION: In addition to our previous finding that JRA sera will react with a defined epitope on HMG-17, pauciarticular JRA patient sera were also found to recognize a defined epitope on the HMG-2 protein, thus suggesting the importance of this epitope in the etiology of JRA.
14685503 [Chronic arthritis in a child with primary agammaglobulinemia]. 1999 Nov OBJECTIVES: To report a case of a child with Bruton's disease and the unusual association with chronic aseptic arthritis resembling juvenile rheumatoid arthritis. METHODS: A 16 month-old boy was seen at the Pediatric Rheumatology unit of HC-UFG and Hospital da Criança (Goiânia- GO). The authors evaluated relevant clinical and laboratorial features, including follow-up and response to therapy. The data were then compared to previous reports published in the world literature based on a Medline search of the subject. RESULTS: Since the age of 6 months, the patient had recurrent episodes of infection that responded poorly to antibiotics. Forty days before admission, he had onset of arthritis in his left knee. The diagnosis of Bruton's disease was based on the seric levels of immunoglobulins and the response to intravenous gammaglobulin infusions. Besides improvement with this therapy, clinical characteristics and other indirect laboratorial tests highly suggested the diagnosis of chronic, aseptic arthritis. CONCLUSION: We present a case of a rare association between Bruton's disease and chronic, aseptic arthritis, very similar to juvenile rheumatoid arthritis. Early recognition of this rare aseptic articular involvement is important for early and efficient therapy with intravenous gammaglobulin infusions, avoiding unnecessary hospital admissions and inappropriate use of antibiotics.
11346232 Renal involvement in juvenile rheumatoid arthritis: report of two cases. 2001 Renal involvement is a rare occurrence in juvenile rheumatoid arthritis (JRA). We report on two JRA patients with kidney disease. The first was a 14-year-old African-American female with a 12-month history of polyarthritis. On presentation she was found to have an ESR of 127 mm/h and a positive ANA, rheumatoid factor (RF), perinuclear antineutrophil cytoplasmic antibodies (pANCA), haematuria, proteinuria with normal BUN and creatinine. Renal biopsy showed focal segmental glomerulosclerosis. Her renal function deteriorated to end-stage renal failure requiring dialysis within a few months, despite aggressive treatment with steorids and monthly i.v. pulses of cyclophosphamide. The second patient presented with a 6-week history of polyarthritis and intermittent fever, and had a salmon-coloured evanescent rash. On presentation his laboratory evaluation was significant for elevated ESR and negative ANA, RF and ANCA tests. Within 8 months the patient had developed a persistent microscopic haematuria. Renal biopsy showed mild mesangial glomerulonephritis. On low-dose methotrexate therapy his JRA went into remission and his renal function remained normal. The haematuria persisted for 1 year and then resolved spontaneously. This is the first time that focal segmental glomerulosclerosis and mesangial glomerulonephritis have been described in JRA. Although the association may be just coincidental, further studies are needed to define the role of JRA in these renal conditions. In patients with JRA, urinalysis and renal function should be routinely monitored.
9309199 Outcome in pediatric rheumatic disease. 1997 Sep Outcome assessment has been a focus of recent research in rheumatic diseases and in pediatric rheumatology. The establishment of a preliminary core set of outcome variables for juvenile rheumatoid arthritis patients and a preliminary definition of improvement using these variables have been important steps toward standardization of outcome assessment. A population-based epidemiologic study has added to our knowledge of long-term outcome in juvenile arthritis and confirms the chronicity of disease that was found in previous studies. Standardization of outcome assessment in pediatric systemic lupus erythematosus has not been achieved to the same extent as in juvenile rheumatoid arthritis, but renal survival and overall mortality are important outcomes in this disease that are easily quantified. Recent studies of pediatric systemic lupus erythematosus demonstrate similarities with respect to organ involvement and overall survival between adult and pediatric lupus patients.
11212149 Early degradation of type IX and type II collagen with the onset of experimental inflammat 2001 Jan OBJECTIVE: To determine whether following the onset of intraarticular inflammation, there is early damage to articular cartilage, specifically to types II and IX collagen, and the proteoglycan (PG) aggrecan, and whether measurement of the degradation products of these molecules in synovial fluid (SF) and serum may permit the detection of cartilage damage. METHODS: A rabbit model of rheumatoid arthritis, antigen (ovalbumin)-induced arthritis, was studied. Articular cartilage samples were analyzed by immunoassays for total type II collagen content, its denaturation and cleavage by collagenases, and for type IX collagen content. PG content was determined by colorimetric assay. In serum and SF, total PG content and collagenase-generated peptides of type II collagen were measured. RESULTS: After 6 days, both the PG content and the NC4 domain of type IX collagen were reduced in femoral and tibial cartilage, concomitant with the onset of arthritis. In only the tibial cartilage did this reduction in PG persist up to day 20. However, denatured type II collagen was increased in all cartilage samples, but only on day 20. In SF, the PG content was significantly reduced on day 20, and products of type II collagen cleavage by collagenase were significantly increased on both day 6 and day 20. CONCLUSION: This study, which is the first of its kind examining changes in both types II and IX collagen and PG content, reveals early damage to both types of collagen as well as to PG in articular cartilage samples following induction of joint inflammation. SF analyses reveal this early damage and may be of value in the study and treatment of inflammatory arthritic diseases such as rheumatoid arthritis.
10765938 Salivary gland lymphomas in patients with Sjögren's syndrome may frequently develop from 2000 Apr OBJECTIVE: Patients with Sjögren's syndrome (SS) have an increased risk of developing monoclonal B cell non-Hodgkin's lymphomas (MNHL), which frequently occur in the salivary glands (SG). The transition from the benign lymphocyte infiltrate of the gland that characterizes SS to MNHL is not well understood. Previous sequence analyses of the expressed variable (V) region genes have supported the theory that the surface Ig (sIg) plays an important role in the initial expansion of nonmalignant B cell clones and in lymphomagenesis. However, the antigenic specificities of these B cells were unknown. We describe the specificities of the Ig expressed by 2 cases of MNHL that developed in the SG of 2 patients with SS. METHODS: The expressed V genes were amplified by polymerase chain reaction from biopsy specimens, sequenced, and subcloned into eukaryotic expression vectors. The constructs were transfected into P3X63-Ag8.653 cells to obtain 2 monoclonal cell lines, each secreting 1 of the sIg expressed by the MNHL. These IgM were tested by enzyme-linked immunosorbent assay and immunofluorescence against a panel of antigens potentially implicated in SS. RESULTS: Our main finding was that the Ig products of the neoplastic B cells were rheumatoid factors (RF). Contrary to expectations, they did not react with nuclear or cytoplasmic antigens, double-stranded DNA, self antigens commonly bound by natural autoantibodies, or SG tissue. CONCLUSION: Previous analyses of V gene use have provided indirect evidence that SG MNHL may frequently express RF. We demonstrate that this hypothesis is true in the 2 patients we studied. Large-scale studies will be needed to establish the exact frequency of RF specificity among SS-associated MNHL.
9779314 Lymphocytic interstitial pneumonitis preceding polyarticular juvenile rheumatoid arthritis 1998 Sep We describe the case of a 12-year-old girl who developed lymphocytic interstitial pneumonitis at 2 years of age which preceded polyarticular rheumatoid factor (RF) positive juvenile arthritis. Her disease had a chronic active course, but showed a good response to combination therapy. The pathogenesis of these two immune processes and of the lung involvement in juvenile arthritis is discussed.
11340726 Parotitis as the initial sign of juvenile Sjögren's syndrome. 2001 Mar Parotid swelling may be associated with a variety of glandular disorders in children. This case report describes the characteristic features of juvenile Sjögren's syndrome in an adolescent girl who presented with recurrent and bilateral parotid gland enlargement. Special emphasis is placed on an age-specific differential diagnosis for major salivary gland enlargements.
11252941 [Primary Gougerot-Sjogren's syndrome]. 2001 Jan 31 Sjogren's syndrome is characterized by the association of a Sicca syndrome prevailing at the ocular and oral level and of extra-glandular involvement of immuno-inflammatory mechanism: nonerosive polyarthritis, Raynaud's phenomenon, cutaneous and (or) neurological vasculitis, pulmonary involvement and interstitial nephropathy. In the typical forms, the biological signs associate a polyclonal hypergammaglobulinemia, sometimes a cryoblogulinemia, rheumatoid factor and anti SSA and anti SSB ANA. The diagnosis is confirmed by minor salivary gland biopsy showing a lymphoid infiltrate in clusters. These biological anomalies, the presence of major salivary gland enlargements and extra-glandular manifestations, characterize the progressive forms of the disease with a high risk of evolution towards malignant lymphoma. Antimalarial drugs are used in the treatment of polyarthritis, corticosteroids and immunosuppressive agents in serious extra-glandular involvement.
11206352 Pure red cell aplasia in Felty's syndrome: a case report of successful reversal after cycl 1999 We describe the first report of a patient with Felty's syndrome who developed pure red cell aplasia, likely not attributable to medication, that was successfully treated with cyclosporin A.
9412422 [Echocardiographic findings in primary Sjögren syndrome]. 1997 Mar Primary Sjögren's syndrome is an autoimmune disorder characterized by an increased cellular and humoral activity, which determines immune-complex deposition at multisystemic level. The main morphologic and functional alterations associated with this syndrome at cardiovascular level have been described only in isolated cases. In this paper, 23 patients with primary Sjögren's syndrome were studied by transthoracic echocardiography. The aim of this study was to determine the relationship between the duration of the syndrome, sex and age with the type of cardiovascular abnormalities. All patients were women with mean age of 58 years (range from 39 to 76 years). The longest disease duration was 20 years and the shortest, 2 years. The main alterations were localized at valve level and are characterized by two patterns of thickening: 1) the first one involves the whole extension of one or more leaflets, 2) the other one is nodular and involves only the edge of one or more leaflets. The abnormal valves were mitral, aortic and tricuspid, but none of them showed a significant dysfunction. We did not find any association between the type of valve abnormalities and age or disease duration. It was concluded that the wide variety of morphologic abnormalities at valvular level were related with degenerative factors associated with the age in some cases, but in others its development probably depends on immunopathologic features of the primary Sjögren's syndrome. It must be proved in future studies whether the affected tissue can be assessed by immunohistochemical tests.
11764624 Oral and periodontal status in Sjögren's syndrome. 2001 Oct Sjögren's syndrome is a multi-system chronic inflammatory disease of the exocrine glands. Inflammation of the salivary glands leads to reduction in salivary output, which imposes a significant impact on oral health. Dentists and dental hygienists are the primary healthcare providers to identify early signs and symptoms of Sjögren's syndrome. Early diagnosis of Sjögren's syndrome is fundamental for effective management of the disease.
9377853 [Acute hepatitis in a patient with adult onset Still disease]. 1997 Apr Liver abnormalities in the course of Adult Onset Still's Disease (AOSD), both in form of hepatomegaly and elevation of hepatic enzymes, have been reported in up to three-quarts of the affected patients. These abnormalities may reflect disease activity or may be induced by drugs. Only in a few of this patients a liver biopsy was performed. However liver histology has shown, generally, non specific abnormalities or even normal pictures. We have recently observed a 47-year-old woman with a febrile illness started five months before, who after pertinent investigation was diagnosed as AOSD (according to criteria of Yamaguchi et al.). Apart from laboratory findings characteristic of an inflammatory disease, in absence of drug therapies the biochemical data showed raised levels of aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and aminoglutamil transferase. Serological tests for either viral hepatitis viruses (HAV, HBV, HCV) or other viruses were negative. Ultrasonographic examination of gallbladder and bile ducts did not find gallstones or other abnormalities. A liver biopsy was performed, which histopathologic examination showed moderate fatty methamorphosis with focal areas of hepatocellular swelling with minimal necrosis, mild Kuppfer cell hyperplasia, portal and sinusoidal infiltrates of mononuclear cells. This picture consisted with the diagnosis of an acute unspecific reactive hepatitis.