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ID PMID Title PublicationDate abstract
16734612 Preferential recognition of the phosphorylated major linear B-cell epitope of La/SSB 349-3 2006 Jun Sera from patients with primary Sjögren Syndrome (pSS) or Systemic Lupus Erythematosus (SLE) often contain autoantibodies directed against La/SSB. The sequence 349-368 aa represents the major B-cell epitope of La/SSB, also it contains, at position 366, a serine amino acid residue which constitutes the main phosphorylation site of the protein. In this study we investigated the differential recognition of the 349-368 aa epitope and its phosphorylated form by antibodies found in sera from patients with systemic autoimmune diseases. Peptides corresponding to the sequence of the unphosphorylated (pep349-368 aa) and the phosphorylated form (pep349-368 aa Ph) of the La/SSB epitope 349-368 aa, as well as to a truncated form spanning the sequence 349-364 aa and lacking the phosphorylation site (pep349-364 aa), were synthesized. Sera from 53 patients with pSS and SLE with anti-La/SSB specificity, 30 patients with pSS and SLE without anti-La/SSB antibodies, 25 patients with rheumatoid arthritis and 32 healthy individuals were investigated by ELISA experiments. Autoantibodies to pep349-368 aa Ph were detected in sera of anti-La/SSB positive patients with a higher prevalence compared to the pep349-368 aa (66%versus 45%). Pep349-368 aa Ph inhibited the antibody binding almost completely (92%), while pep349-368 aa inhibited the binding only partially (45%). Anti-La/SSB antibodies presented a higher relative avidity for the phosphorylated than the unphosphorylated peptide. Immunoadsorbent experiments using the truncated peptide pep349-364 aa indicated that the flow through showed a selective specificity for pep349-368 aa Ph, while the eluted antibodies reacted with both peptide analogues of the La/SSB epitope. These data suggest that sera from pSS and SLE patients with anti-La/SSB reactivity possess autoantibodies that bind more frequently and with a higher avidity to the phosphorylated major B-cell epitope of the molecule.
18614233 The kappa immunoglobulin light chain repertoire of peripheral blood B cells in patients wi 2008 Aug The frequent appearance of antinuclear antibodies in patients with juvenile rheumatoid arthritis (JRA) indicates a loss of tolerance in B cell differentiation and/or activation. In this analysis, we were interested whether particular changes in the immunoglobulin light chain repertoire might exist in early-onset pauciarticular arthritis (EOPA) patients thereby potentially revealing distinct molecular patterns, which characterize defects in central tolerance mechanisms as well as an autoreactive peripheral B cell repertoire. Using single cell sorting and single cell PCR the distribution of Vkappa Jkappa rearrangements has been analyzed in individual naïve B cells of patients with EOPA-JRA and healthy individuals. The immunoglobulin kappa light chain repertoire of peripheral blood B cells in EOPA patients seems to be skewed to a decreased use of downstream Vkappa gene segments indicating increased events of secondary V(D)J-recombination. Another prominent molecular pattern in JRA B cells seem to be a restricted combination of Vkappa Jkappa rearrangements based on the predominant utilization of the Jkappa 1 and 2 gene segment. The current study indicates disturbances in the peripheral B cell pool in juvenile rheumatoid arthritis. The peripheral blood B cell pool of JRA patients did show molecular changes in the kappa light chain repertoire which, in part, could be a sequel of secondary V(D)J-recombination and of a molecular bias during immunoglobulin rearrangement in the bone marrow. Thus, B cell tolerance might be broken by more than one pathogenic mechanism.
18524744 Consultation with an arthritis specialist for children with suspected juvenile rheumatoid 2008 Jun OBJECTIVES: To describe consultation with an arthritis specialist because of suspected new-onset juvenile rheumatoid arthritis (JRA) and to determine factors associated with prompt consultation. DESIGN: Retrospective cohort study. SETTING: Physician reimbursement administrative data were obtained for all children aged 16 years or younger in the Province of Québec (Canada). PARTICIPANTS: Suspected new-onset cases of JRA in 2000 were defined by a physician visit because of JRA, providing there had been no such claims in the preceding 3 years. MAIN EXPOSURE: First JRA diagnosis made by a non-arthritis specialist. MAIN OUTCOME MEASURES: First consultation with an arthritis specialist subsequent to diagnosis by a non-arthritis specialist and time to first consultation with an arthritis specialist. RESULTS: Of 352 children and adolescents with suspected new-onset JRA identified by non-arthritis specialists, 159 (45.2%) were subsequently seen by an arthritis specialist. Mean (SD) time to consultation for those seen was 115.3 (213.8) days (median, 28 days). Younger children were more likely to obtain care from an arthritis specialist compared with those having JRA first diagnosed by a general practitioner. CONCLUSION: Most patients with suspected new-onset JRA do not obtain prompt care from an arthritis specialist. Further research and action should focus on this issue so that outcomes may be optimized.
16586047 A still image of a transient rash captured by a mobile phone. 2007 Jun The diagnosis of adult onset Still's disease (AOSD) can be difficult as the differential diagnosis is broad, it is based on clinical criteria and the signs, for example rash, can be transient. Clinical photography has an obvious role, and with modern technology, is now in the hands of physicians. We report a case of AOSD where an image of a transient rash taken with a camera phone allowed the diagnosis to be established. Further, we discuss the controversies around hospital bans on mobile phones (due to potential incompatibility with medical devices) and the reality of their widespread use. We conclude that, providing safeguards of consent and data storage are in place, the camera phone is a useful tool in rheumatology practice.
18466563 Classification of rheumatoid arthritis status with candidate gene and genome-wide single-n 2007 Using the North American Rheumatoid Arthritis Consortium (NARAC) candidate gene and genome-wide single-nucleotide polymorphism (SNP) data sets, we applied regression methods and tree-based random forests to identify genetic associations with rheumatoid arthritis (RA) and to predict RA disease status. Several genes were consistently identified as weakly associated with RA without a significant interaction or combinatorial effect with other candidate genes. Using random forests, the tested candidate gene SNPs were not sufficient to predict RA patients and normal subjects with high accuracy. However, using the top 500 SNPs, ranked by the importance score, from the genome-wide linkage panel of 5742 SNPs, we were able to accurately predict RA patients and normal subjects with sensitivity of approximately 90% and specificity of approximately 80%, which was confirmed by five-fold cross-validation. However, in a complete training-testing framework, replication of genetic predictors was less satisfactory; thus, further evaluation of existing methodology and development of new methods are warranted.
18766124 Hypertrophic osteoarthropathy in the hepatopulmonary syndrome. 2008 Aug Hypertrophic osteoarthropathy is an uncommon disease in the pediatric age group characterized by noninflammatory joint effusions, terminal digit clubbing, and radiographic evidence of periosteal new bone formation affecting the hands, feet, and distal limbs. The hepatopulmonary syndrome is also uncommon in childhood and presents as hepatic dysfunction, impaired arterial oxygenation, and intrapulmonary shunting. We report the case of a 17-year-old male with a history of liver transplantation at 4 months for biliary atresia who was initially diagnosed with juvenile rheumatoid arthritis but later developed features of classic hypertrophic osteoarthropathy. In addition, he was found to have the hepatopulmonary syndrome. It is important to consider hypertrophic osteoarthropathy as an imitator of juvenile rheumatoid arthritis, to recognize its known association with chronic liver disease, and to know that hepatopulmonary syndrome can occur in the setting of hypertrophic osteoarthropathy.
16507151 Identification of collagen-induced arthritis loci in aged multiparous female mice. 2006 Collagen-induced arthritis in mice is one of the most commonly used autoimmune experimental models, with many similarities to rheumatoid arthritis. Since collagen-induced arthritis is a complex polygenic disease there is a need for identification of several major disease-controlling genes. Because rheumatoid arthritis particularly affects aged women, we have in the present study identified new genetic regions critical for collagen-induced arthritis by studying aged female mice of a cross between NFR/N and B10.Q (H-2q haplotype). The mice in the present study had different reproductive histories, which did not significantly affect the onset, incidence or severity of the disease. A total of 200 female mice were used in a total genome-wide screening with 125 microsatellite markers. We found one new significant quantitative trait locus affecting the arthritis incidence, severity and day of onset on chromosome 11 (denoted Cia40), which colocalizes with a locus controlling pregnancy failure. Furthermore, a quantitative trait locus of suggestive significance associated with the incidence, severity and day of onset was identified on chromosome 1. Finally, a suggestively significant quantitative trait locus associated with collagen type II antibody titers was identified on chromosome 13. This study indicates that several gene loci control arthritis in aged multiparous females, and that at least one of these loci coincides with pregnancy failure.
18333827 Psoriatic arthritis and arthroplasty: a review of the literature. 2008 Psoriatic arthritis is an inflammatory arthropathy as- sociated with the characteristic dermatologic lesions of psoriasis. The diagnosis of psoriatic arthritis is quite difficult, due to the overlap of patients with osteoarthritis (OA) or rheumatoid arthritis (RA) with concomitant non-associated psoriasis. A nonspecific elevation in inflammatory markers (erythrocyte sedimentation rate, ESR; antinuclear antibodies, ANA; or rheumatoid factor, RF) and characteristic radiographic features are often present in these patients. The mainstay of treatment is medical management, using NSAIDs, various immunosuppressants, and anti-TNF agents, for both pain control and possibly as disease modifying agents. Only a minority of patients require surgical intervention, leading to the limited amount of literature concerning total joint arthroplasty and psoriatic arthritis. While past literature has yielded high infection rates post-arthroplasty, newer studies have found more promising results. Alternative surgical options for treating destructive arthritis include open or arthroscopic synovectomy. While early results are promising, recurrence rates and long-term outcomes are not yet available.
18760082 [Our results of the Lapidus procedure in patients with hallux valgus deformity]. 2008 Aug PURPOSE OF THE STUDY: The most frequent deformity of the big toe and forefoot associated with a collapse of the transverse arch of the foot is a valgus deformity. For correction of a hallux valgus, several procedures are described in the literature. A valgus deformity often develops due to a varus deviation of the first metatarsal bone when the intermetatarsal angle between the first and second metatarsals is greater than 10 degrees. When the intermetatarsal angle is larger then 10 degrees or the first ray is hypermobile, a Lapidus procedure is one of the options. The objective of this study was to evaluate the outcomes in patients with hallux valgus deformity treated by the Lapidus procedure. MATERIAL: The group comprised 61 patients, 49 women and 12 men, with an average age of 58.3 years at the time of surgery, who were treated at our department in the period from 2002 to 2006. Fifteen patients had bilateral surgery. The results of 76 operations were evaluated. Indications for surgery were hallux valgus in 22 patients, rheumatoid arthritis in 36 and psoriatic arthritis in three patients. METHODS: Access was gained on the mediodorsal side of the foot through an incision medial to the extensor hallucis longus tendon, over the first tarsometatarsal joint up to the first metatarsophalangeal joint. An arthrodesis was fixed with two Kirschner wires in eight feet and with two screws in 15 feet. Recently, the use of shape memory alloy staples (DePuy Mitek) was adopted and applied in 53 feet with good outcome. In the patients with rheumatoid arthritis the Lapidus procedure together with resection of the heads of the second and fifth metatarsals was used from the plantar approach. RESULTS: The evaluation was focused on the patients' satisfaction and their subjective complaints. No pain was reported on 56 (73%) forefeet, slight pain was experienced on the dorsal side in ten feet (13%) and pain in the transverse arch also in ten feet (13%). Complications included slow healing of the wound in seven feet of the patients with rheumatoid arthritis (9.2%), and infection requiring revision surgery in one patient (1.3%). Five patients (6.5%) reported persisting swelling of the foot dorsum for a period longer than 3 months. Recurrence of hallux valgus was recorded in nine feet. The average American Orthopaedic Foot and Ankle Society score, which was 48.1 points pre-operatively, improved to 89.2 points post-operatively. In one patient, bony union was very slow and was achieved at 5 months after surgery. DISCUSSION: An exact evaluation of the results of a Lapidus procedure is not always possible, particularly in patients with rheumatoid arthritis in whom foot disorders are more complex. A much discussed issue is first metatarsal hypermobility. In our group of 61 patients, this was found in 28. Of these, 18 had rheumatoid arthritis and ten had hallux valgus. The rate of pseudoarthrosis following a Lapidus procedure is reported to range from 3.3% to 9%. In our group only one patient was affected (1.3%). CONCLUSION: A correctly performed: Lapidus procedure enables us, by correcting a varus deviation of the first metatarsal, to repair valgus deformity of the big toe resulting in painless walking.
18419828 Mucosal administration of alpha-fodrin inhibits experimental Sjögren's syndrome autoimmun 2008 INTRODUCTION: alpha-Fodrin is an autoantigen in Sjögren's syndrome. We hypothesized that mucosal administration of alpha-fodrin might prevent the disease. METHODS: Four-week-old NOD mice were immunized (intranasal) with a 1 microg or 10 microg dose of alpha-fodrin every other day. PBS 10 microl/dose and Glutathione transferase (GST 10 microg/dose (control mice) were intranasally administrated by the same procedure. The salivary flow was maintained in immunized animals. The animals were analyzed for the presence of anti-Sjögren's syndrome A, anti-Sjögren's syndrome B, rheumatoid factor and antinuclear, anti-alpha-fodrin, and anti-type 3 muscarinic acetylcholine receptor polypeptide (anti-M3RP) by immunofluorescence or ELISA. The cytokines IFNgamma and IL-10 were measured by ELISA. Salivary glands were examined by H&E staining and immunohistochemical analysis. The water-volume intake was calculated for each group. The induction of regulatory T cells was assessed by fluorescence-activated cell sorting analysis for the frequency of Foxp3+ cells among peripheral CD4+CD25+ T cells. RESULTS: The appearance of anti-alpha-fodrin and anti-M3RP antibodies was delayed in mice immunized with alpha-fodrin. The titers of anti-alpha-fodrin and anti-M3RP antibodies were lower in immunized mice (P < 0.05), but there was no significant difference between the low-dose or high-dose immunization groups. Five out of eight mice in the GST group, five of eight mice in the PBS group, two of eight mice in the alpha-fodrin 1 microg/dose group, and three out of eight mice in the alpha-fodrin 10 microg/dose were positive for antinuclear antibodies. The levels of serum IFNgamma in mice immunized with 1 microg/dose or 10 microg/dose alpha-fodrin, with PBS, and with GST were 41.9 +/- 16.2 pg/ml, 37.1 +/- 15.4 pg/ml, 86.8 +/- 17.8 pg/ml and 71.6 +/- 11.1 pg/ml, respectively, while we found no difference in the levels of serum IL-10 among the groups. The number of Foxp3+ CD4+CD25+ regulatory T cells was higher in the alpha-fodrin groups compared with the PBS and GST control groups (P < 0.05). Lymphocytic infiltration and expression of alpha-fodrin in the salivary glands was decreased in alpha-fodrin-treated groups. The fluid intake of mice in the 1 microg/dose alpha-fodrin, 10 microg/dose alpha-fodrin, PBS, and GST groups was 39.2 +/- 2.1 ml, 40.4 +/- 2.5 ml, 49.3 +/- 3.1 ml and 51.6 +/- 2.8 ml, respectively. CONCLUSION: Mucosal administration of alpha-fodrin effectively inhibited the progression of experimental Sjögren's syndrome autoimmunity.
21794503 [Strongyloides stercolaris in a Patient With Rheumatoid Arthritis Undergoing Treatment Wit 2008 Mar Biologic therapy for the treatment of autoimmune diseases such as rheumatoid arthritis leads to a series of secondary effects and complications which are ever more frequent and increasingly complicate both the management as well as the associated comorbidity. We present the case of a patient who had one of theses associated complications.
18766426 Anakinra in the treatment of polyarticular-course juvenile rheumatoid arthritis: safety an 2009 Feb This study assessed the safety and preliminary efficacy of the interleukin-1 receptor antagonist anakinra in patients with polyarticular-course juvenile rheumatoid arthritis (JRA). Eighty-six patients entered a 12-week open-label run-in phase (1 mg/kg anakinra daily, < or =100 mg/day). Fifty responders were randomized to anakinra or placebo in a 16-week blinded phase, followed by a 12-month open-label extension (N = 44). Due to low enrollment, the primary endpoint was changed from efficacy to safety. The incidence and nature of adverse events were similar across all study phases, with the exception of injection site reactions, which were mild to moderate and decreased with time. Anakinra produced a nonsignificant (P = 0.11) reduction in disease flares compared with placebo. When normalized to 1 mg/kg dose, anakinra plasma concentrations were similar to values in adult patients with rheumatoid arthritis. These results indicate that anakinra 1 mg/kg once daily (< or =100 mg/day) is safe and well tolerated in patients with JRA.
18378278 Prevalence and clinical relevance of autoimmune neutropenia in patients with primary Sjög 2009 Apr OBJECTIVE: To analyze the prevalence of neutropenia in a large cohort of patients with primary Sjögren's syndrome (SS) and its association with clinical and immunological disease expression and adverse outcomes. METHODS: The study cohort included 300 patients diagnosed with primary SS in our department between 1984 and 2002. The outcomes measured after the first laboratory evidence of neutropenia (<2.5 x 10(9)/L) were first hospital admission caused by infection, development of systemic manifestations, neoplasia, and death. RESULTS: Ninety-nine (33%) patients had neutropenia during the follow-up, which was related to neoplasia or drugs in 9 (3%) patients and was considered idiopathic in the remaining 90 (30%). Patients with neutropenia had a lower mean age at diagnosis of SS (51.9 versus 59.4 years, P < 0.001) and a higher prevalence of anti-Ro/La antibodies (53% versus 22%, P < 0.001), rheumatoid factor (49% versus 32%, P = 0.009), and low C4 levels (17% versus 8%, P = 0.044) than those without neutropenia. Patients with neutropenia had a higher incidence of hospital admission caused by infection (24% versus 9%, P = 0.002), especially those with neutropenia <1 x 10(9)/L (50% versus 9%, P = 0.002), and a higher rate of admission (log rank = 0.0023) in comparison with those without neutropenia. Agranulocytosis was found in 7 (2%) patients, predominantly related to neoplasia (5 cases). One (1%) of the 90 patients with SS-related neutropenia developed large granular lymphocyte T-cell leukemia. CONCLUSION: Neutropenia should be considered a relevant hematologic finding of primary SS, due both to its elevated prevalence and to its clinical significance (close association with anti-Ro/La antibodies, coexistence with other cytopenias, and development of severe infections).
18085728 Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from 2008 Feb OBJECTIVE: To characterize the initial clinical and laboratory features of patients with systemic onset juvenile rheumatoid arthritis (soJRA) through a Web-based registry. METHODS: Patients diagnosed with soJRA in the last 15 years at 3 medical centers in Pennsylvania were identified. Data were collected retrospectively using a Web-based interface in compliance with patient privacy standards. Inferential statistics were used to compare features of patients with and without macrophage activation syndrome. RESULTS: We identified 136 patients; 88% of patients presented with arthritis (8% mono-, 45% oligo-, 47% polyarticular). The most common joints involved were the knee (68% of patients with arthritis), wrist (68%), and ankle (57%). The International League of Associations for Rheumatology criteria for systemic juvenile idiopathic arthritis (SJIA) identified only 30% of patients at presentation. CONCLUSION: We successfully characterized the presenting features of a relatively rare disease, soJRA, through the use of a Web-based registry. Current classification criteria for SJIA may not be particularly sensitive for diagnosis at presentation.
16583465 Laboratory and imaging studies used by French rheumatologists to evaluate patients with ea 2006 May OBJECTIVE: To conduct a practice survey of laboratory and imaging studies used by French rheumatologists to identify the cause of recent-onset arthritis. METHODS: We selected a random sample of 210 rheumatologists, who were asked to recruit all patients with recent-onset arthritis (at least one joint involved, for less than one year) during a 2 week period, and to record laboratory and imaging studies performed. Results were analyzed in the overall group, in diagnostic subgroups, and in clinical presentation subgroups. RESULTS: The 119 rheumatologists who participated recruited 104 patients. Investigations done in 50% to 75% of patients were blood cell counts; erythrocyte sedimentation rate; serum assays of C-reactive protein, rheumatoid factors, antinuclear antibodies; and hand radiographs. Investigations in 50% to 74% of patients were serum ASAT/ALAT, creatinine, and uric acid; and foot radiographs. Finally, 25% to 49% of patients were tested for proteinuria; antikeratin antibodies; hepatitis B, hepatitis C, and Lyme serologies; creatine phosphokinase; blood iron; HLA-B27; and radiographs of chest and pelvis. No differences were found between investigations in patients with suspected rheumatoid arthritis and/or undifferentiated arthritis and those in other patients. In contrast, suspected diagnoses and presence of extraarticular manifestations classically associated with specific diseases modified the selection of investigations. CONCLUSION: Although considerable variability occurred, our study suggests that a limited panel of laboratory and imaging studies is performed in at least 25% of patients with recent-onset arthritis, regardless of clues suggesting a specific diagnosis.
18716298 Adalimumab with or without methotrexate in juvenile rheumatoid arthritis. 2008 Aug 21 BACKGROUND: Tumor necrosis factor (TNF) has a pathogenic role in juvenile rheumatoid arthritis. We evaluated the efficacy and safety of adalimumab, a fully human monoclonal anti-TNF antibody, in children with polyarticular-course juvenile rheumatoid arthritis. METHODS: Patients 4 to 17 years of age with active juvenile rheumatoid arthritis who had previously received treatment with nonsteroidal antiinflammatory drugs underwent stratification according to methotrexate use and received 24 mg of adalimumab per square meter of body-surface area (maximum dose, 40 mg) subcutaneously every other week for 16 weeks. We randomly assigned patients with an American College of Rheumatology Pediatric 30% (ACR Pedi 30) response at week 16 to receive adalimumab or placebo in a double-blind fashion every other week for up to 32 weeks. RESULTS: Seventy-four percent of patients not receiving methotrexate (64 of 86) and 94% of those receiving methotrexate (80 of 85) had an ACR Pedi 30 response at week 16 and were eligible for double-blind treatment. Among patients not receiving methotrexate, disease flares (the primary outcome) occurred in 43% of those receiving adalimumab and 71% of those receiving placebo (P=0.03). Among patients receiving methotrexate, flares occurred in 37% of those receiving adalimumab and 65% of those receiving placebo (P=0.02). At 48 weeks, the percentages of patients treated with methotrexate who had ACR Pedi 30, 50, 70, or 90 responses were significantly greater for those receiving adalimumab than for those receiving placebo; the differences between patients not treated with methotrexate who received adalimumab and those who received placebo were not significant. Response rates were sustained after 104 weeks of treatment. Serious adverse events possibly related to adalimumab occurred in 14 patients. CONCLUSIONS: Adalimumab therapy seems to be an efficacious option for the treatment of children with juvenile rheumatoid arthritis. (ClinicalTrials.gov number, NCT00048542.)
18727822 Disseminated cutaneous Herpes Simplex Virus-1 in a woman with rheumatoid arthritis receivi 2008 Aug 26 INTRODUCTION: We present the case of a 49-year-old woman with a seronegative rheumatoid arthritis who developed pustular psoriasis whilst on etanercept and subsequently developed disseminated herpes simplex on infliximab. CASE PRESENTATION: Our patient presented with an inflammatory arthritis which failed to respond to both methotrexate and leflunomide, and sulphasalazine treatment led to side effects. She was started on etanercept but after 8 months of treatment developed scaly pustular lesions on her palms and soles typical of pustular psoriasis. Following the discontinuation of etanercept, our patient required high doses of oral prednisolone to control her inflammatory arthritis. A second biologic agent, infliximab, was introduced in addition to low-dose methotrexate and 15 mg of oral prednisolone. However, after just 3 infusions of infliximab, she was admitted to hospital with a fever, widespread itchy vesicular rash and worsening inflammatory arthritis. Fluid from skin vesicles examined by polymerase chain reaction showed Herpes Simplex Virus type 1. Blood cultures were negative and her chest X-ray was normal. Her infliximab was discontinued and she was started on acyclovir, 800 mg five times daily for 2 weeks. She made a good recovery with improvement in her skin within 48 hours.She continued for 2 months on a prophylactic dose of 400 mg bd. Her rheumatoid arthritis became increasingly active and a decision was made to introduce adalimumab alongside acyclovir. Acyclovir prophylaxis has been continued but the dose tapered so that she is taking only 200 mg of acyclovir on alternate days. There has been no recurrence of Herpes Simplex Virus lesions despite increasing adalimumab to 40 mg weekly 3 months after starting treatment. CONCLUSION: We believe this to be the first reported case of widespread cutaneous Herpes Simplex Virus type 1 infection following treatment with infliximab. We discuss the clinical manifestations of Herpes Simplex Virus infections with particular emphasis on the immunosuppressed patient and the use of prophylactic acyclovir. Pustular psoriasis is now a well recognised but uncommon side effect of antitumour necrosis factor therapy and can lead to cessation of therapy, as in our patient's case.
17471846 [Adult-onset Still's disease]. 2006 INTRODUCTION: The authors present the course and manifestations of adult-onset Still's disease on the basis of five cases diagnosed at the Department of Rheumatology, Pomeranian Medical University in Szczecin. MATERIAL AND METHODS: The usefulness of two most popular sets of diagnostic criteria of adult-onset Still's disease (Yamaguchi and Cush) was analyzed. At onset of the disease, two out of five patients met both sets of the diagnostic criteria, two others met criteria of Yamaguchi and one of Cush. During follow-up, criteria of Yamaguchi were met in all cases. RESULTS: The authors suggest to use the Cushs criteria of adult-onset Still's disease when the patient does not meet the criteria of Yamaguchi and other causes of fever are excluded.
16646038 Functional characterization of a soluble gp130 isoform and its therapeutic capacity in an 2006 May OBJECTIVE: Soluble gp130 is the naturally occurring antagonist of the interleukin-6 (IL-6)/soluble IL-6 receptor (sIL-6R) complex and selectively inhibits IL-6 trans-signaling. Several isoforms of soluble gp130 have been identified, including an autoantigenic form termed gp130-RAPS (for gp130 of the rheumatoid arthritis antigenic peptide-bearing soluble form) that is present in the serum and synovial fluid of patients with rheumatoid arthritis. The aim of this study was to evaluate the functional properties of gp130-RAPS. METHODS: To define a role for gp130-RAPS in arthritis, a recombinant version was generated using a baculovirus expression system, and its activities were tested in vitro and in vivo. RESULTS: Gp130-RAPS was shown to bind with high affinity to the stable IL-6/sIL-6R complex, hyper-IL-6, and to effectively modulate leukocyte migration in murine acute peritonitis. A single intraarticular injection of gp130-RAPS suppressed chronic antigen-induced arthritis in association with a reduction in local activation of signal transducer and activator of transcription 3. Although gp130-RAPS contains the previously identified autoantigenic sequence Asn-Ile-Ala-Ser-Phe (NIASF), no increase in the prevalence of anti- gp130-RAPS antibodies was observed in serum or synovial fluid obtained from patients with rheumatoid arthritis. CONCLUSION: The use of inhibitory antibodies to block IL-6 responses has shown considerable clinical promise. However, the results presented herein suggest that selective targeting of IL-6 trans-signaling may represent a viable alternative to this strategy. In this respect, our present results suggest that the soluble gp130 isoform gp130-RAPS may be useful in the treatment of chronic inflammatory arthritis.
19035231 [Lung involvement of primary Sjögren's syndrome]. 2008 Jul OBJECTIVE: To evaluate the incidence, clinical manifestations and immunological features of lung involvement in patients of primary Sjögren's syndrome (pSS). METHODS: Five hundred twenty-two patients with pSS in Peking Union Medical College Hospital between 1985 and 2005 were screened retrospectively for lung involvement by either the abnormalities of chest imaging, lung function or the pulmonary artery systolic pressure estimated by ultrasonic echocardiogram > or = 40 mm Hg (1 mm Hg = 0.133 kPa), excluding infections, chronic obstructive pulmonary disease, asthma, congenital heart disease, rheumatic heart disease and other diseases. The difference was compared between patients with and without lung involvement. All patients fulfilled the 2002 international classification (criteria) for pSS. RESULTS: (1) The incidence of lung involvement in pSS was 42.3% (221/522) and occurred from 0 to 384 months (median, 48 months) after onset, while 25.2% occurred before the diagnosis of pSS. Only 47.1% of the patients showed respiratory symptoms. The average age of onset was older in patients with lung involvement than in those without lung involvement [(43 +/- 13) yr vs (37 +/- 14) yr, t = -5.445, P = 0.000]. Incidences of dry mouth (89.6% vs 81.1%, chi2 = 7.145, P = 0.008), dry eyes (78.7% vs 66.4%, chi2 = 9.472, P = 0.002) and rampant caries (55.2% vs 42.2%, chi2 = 8.647, P = 0.003) were higher in patients with lung involvement than those without. There was no significant difference in sex ratio between the two groups. (2) Interstitial lung disease was the most common lung involvement and occurred in 23.2% of the patients. Pulmonary artery hypertension in 12.5%, multiple pulmonary bullae in 9.2%, pleural effusion in 6.0% and multiple pulmonary nodules in 5.6%. (3) The major histopathological patterns were nonspecific interstitial pneumonia (5/11 cases), lymphocytic interstitial pneumonia (3/11 cases). (4) Incidences of Ranaud' s phenomenon (26.7% vs 13.0%, chi2 = 15.77, P = 0.000 ), low-grade fever (20.4% vs 13.0%, chi2 = 5.175, P = 0.023), arthrosis (29.4% vs 21.6%, chi2 = 4.164, P = 0.041), anti-U1RNP (18.2% vs 11.2%, 2 = 5.069, P = 0.024) and hypergammaglobulinemia (51.6% vs 39.5%, chi2 = 6.597, P = 0.01) were higher in patients with lung involvement than in those without. The incidence of renal tubule acidosis was lower in patients with lung involvement than in those without (5.4% vs 12.6% chi2 = 7.616, P = 0.006). (5) The death incidence in pSS with pulmonary involvement was 5.5 times higher than in those without. The most frequent cause of death was infection (64.3%), especially pulmonary infection. CONCLUSION: Lung involvement in pSS is common. As it is an important factor related to the prognosis of this disease, chest X-ray, HRCT, lung function and ultrasonic echocardiogram after the diagnosis are suggested.