Cases reported "Arthritis, Rheumatoid"

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1/33. Idiopathic hypoparathyroidism associated with adult rheumatoid arthritis.

    A 48-year-old female patient with adult onset idiopathic hypoparathyroidism diagnosed at the age of 28 years developed a typical seropositive rheumatoid arthritis (RA) at 46 years of age after several years of evolution of a palindromic rheumatism. Only one case of an association between idiopathic hypoparathyroidism and RA has been described in the medical literature. autoimmunity seems to play a pivotal role in the aetiopathogenesis of both diseases, and could explain the nature of this association; nevertheless, a chance association could not be excluded.
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2/33. Persistent pneumomediastinum in interstitial fibrosis associated with rheumatoid arthritis: treatment with high-concentration oxygen.

    We present a case of persistent spontaneous pneumomediastinum precipitated by an upper respiratory infection in a patient with interstitial fibrosis associated with rheumatoid arthritis who was receiving chronic corticosteroid treatment. The persistent nature of the mediastinal emphysema over 2 months eventually required treatment with high concentrations of inhaled oxygen that resulted in rapid resolution of the pneumomediastinum without recurrence over 6 months of follow-up. This case, along with others in the medical literature, emphasizes the need for early use of high-concentration inhaled oxygen in the treatment of pneumomediastinum in high-risk patients, such as those with connective tissue disorders.
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3/33. Do B cells influence disease progression in chronic synovitis? Lessons from primary hypogammaglobulinaemia.

    We describe a 62-yr-old male patient with primary hypogammaglobulinaemia (PH) who fulfilled the 1987 American Rheumatism association/American College of rheumatology revised diagnostic criteria for rheumatoid arthritis (RA) but, despite persistent symmetrical synovitis, did not develop erosions. virology studies and blood and synovial fluid (SF) cultures were consistently negative; a search for crystals in the SF was unrevealing. Peripheral blood (PB) B cells were absent, whilst the PB CD3( ) cell count was normal. The ratio of naive (CD45RA( )) to memory (CD45R0( )) cells was also normal (1:1) but the CD4:CD8 ratio was reversed. To our knowledge, this is the first report which combines the immunophenotypic analysis of the PB with that of the SF and synovial membrane (SM). This confirmed the absence of B cells and the reversed CD4:CD8 ratio. However, as in other chronic arthropathies, the SF and SM cellular infiltrate consisted almost exclusively of memory T cells, consistent with the preferential localization of this subset to inflamed tissues. This case indicates that synovitis can proceed persistently in the absence of B cells and that the migratory mechanisms of T cells are not altered. However, the case suggests that the absence of B cells and negativity for rheumatoid factor, combined with an increased presence of CD8( ) (suppresser/cytotoxic) T cells in the joint, might contribute to the non-erosive nature of the synovitis.
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4/33. Elevated vitreous concentration of monoclonal immunoglobulin manifesting as schlieren in juvenile rheumatoid arthritis-associated uveitis.

    We report the clinical findings and analysis of the immunoglobulin (Ig) composition of the vitreous of a 10-year-old girl with juvenile rheumatoid arthritis-associated uveitis. The vitreous had a schlieren appearance at the time of pars plana lensectomy and vitrectomy. Analysis of the vitreous fluid revealed marked elevation of IgG, IgM, IgA, and albumin levels relative to vitreous fluids from control patients without uveitis. The immunoglobulin coefficients were also elevated for the IgG and IgM classes of immunoglobulins. Immunofixation electrophoresis of the vitreous fluid revealed 2 distinct bands of restricted electrophoretic mobility. These studies suggest that there may be local (intraocular) production of immunoglobulins as an immunologic response in ocular inflammatory diseases such as juvenile rheumatoid arthritis-associated uveitis and that this immunologic response may be monoclonal (possibly biclonal or oligoclonal) in nature.
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5/33. Acquired factor viii autoantibody: four cases demonstrating the heterogenous nature of this condition and problems involved in diagnosis and treatment.

    The development of an autoantibody to human factor viii is rare and presents many problems for diagnosis and treatment. We have seen several cases at our institution recently with widely heterogenous clinical and laboratory presentations. A wide range of treatment modalities were used in these cases with no gold standard of treatment or widely accepted guidelines existing. This has prompted us to examine all cases of this condition presenting at Fremantle Hospital over the last decade. We describe four cases which demonstrate the heterogeneity of this condition and its treatment and review the recent literature on the subject.
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6/33. Complete recovery from refractory immune thrombocytopenic purpura in three patients treated with etanercept.

    Management of patients with immune thrombocytopenic purpura (ITP) who have persistent, severe, and symptomatic thrombocytopenia following splenectomy is difficult and empirical. No single agent or regimen provides long-term success for most patients, and for most treatments it is difficult to assess whether benefits outweigh risks. We report three consecutive patients with critical chronic refractory ITP, who responded promptly and completely following treatment with etanercept, an inhibitor of tumor necrosis factor-alpha. These patients had failed 6-11 previous treatments. In the first patient, etanercept was given for its approved indication: a flare of co-existing rheumatoid arthritis. The next two patients were treated with etanercept because of successful outcomes in the previous patients. Although etanercept appeared to be effective treatment for ITP in these 3 patients, the experimental nature of this treatment and the potential risks must be emphasized. On the basis of these case reports, a clinical trial has been initiated to systematically evaluate the efficacy and risks of etanercept in the management of children and adults with chronic ITP.
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7/33. employment and chronic non-cancer pain: insights into work retention and loss.

    OBJECTIVES: To explore the work experiences of three individuals who have chronic non-cancer pain and to identify factors which may affect their ability to remain in employment. methods: Three cases were selected from a data set of six, to illustrate distinct employment history scenarios. All participants had a diagnosis of rheumatoid arthritis. Data was gathered using semi-structured, in-depth interviews that were audiotaped and later transcribed for qualitative, content analysis. Themes identified were those which related to employment experiences and appeared to be important factors influencing the retention of work. FINDINGS: Four main themes were identified: the nature of physical barriers to employment, autonomy at work, social networks, and the importance and motivation to remain in employment. CONCLUSION: These findings support existing research in relation to chronic pain and employment in general and expands upon the nature and importance of social support at work. It is suggested that the qualitative methodology illustrated here, which explores the perspectives of those still in employment (as well as those who have stopped working) may be a useful approach to investigating this important area of research.
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8/33. Anti-Golgi antibody in rheumatoid arthritis patients recognizes a novel antigen of 79 kDa (doublet) by western blot.

    We have detected cytoplasmic anti-Golgi antibody (AGA) during a routine immunofluorescence test for detecting autoantibodies. Two sera from patients with rheumatoid arthritis (RA) reacted to the Golgi complex by an indirect immunofluorescence technique on HEp-2 cells. Localization of AGA in the Golgi complex was confirmed by double-staining with antibodies to beta-COP. The effect of monensin on the integrity and morphology of the Golgi complex was also studied. To confirm the presence of AGA further, we performed immuno-electron microscopy. Both sera reacted with cytoplasmic antigen located in the Golgi complex of various animal tissues. Furthermore, by using the Western blot technique, both sera reacted to a relative molecular weight (MW) of 79 kDa (doublet) Golgi antigen purified from rat liver. To our knowledge, this study may be the first to identify the relative MW of Golgi antigen by the Western blot method. Identification of this antibody could provide better understanding of protein synthesis and secretion. The presence of AGA in RA patients further substantiates the diversified nature of autoantibody production seen in this disease.
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9/33. Rheumatic complications of human immunodeficiency virus infection in the era of highly active antiretroviral therapy: emergence of a new syndrome of immune reconstitution and changing patterns of disease.

    OBJECTIVE: To describe the impact of the introduction of highly active antiretroviral therapy (HAART) on the nature and frequency of rheumatic complications in human immunodeficiency virus (hiv)-infected patients. methods: Case report and systematic review of a newly described syndrome of rheumatic immune reconstitution syndrome and prospective longitudinal cohort study analyzing the frequency and nature of rheumatic complications in the setting of hiv infection from 1989 through 2000. RESULTS: A newly described syndrome of either the de novo appearance or the exacerbation of clinically occult autoimmunity following immune reconstitution from HAART is described. Including the present case report, 32 cases have been individually described with sarcoidosis and autoimmune thyroid disease being most common with arthritis and various forms of connective tissue disease making up the rest. The mean onset to their appearance following HAART was nearly 9 months and most resolved with little or no therapy. In addition, a longitudinal analysis of 395 hiv-infected patients from 1989 to 2000 designed to detect the appearance of rheumatic complications has revealed a dramatic decline in certain problems such as reactive arthritis, psoriatic arthritis, and various forms of connective tissue disease. New rheumatic complications possibly due to the effects of longer survival and metabolic derangements associated with this form of therapy are now being described and may become more formidable problems in this population in the future. CONCLUSIONS: HAART has had a profound beneficial effect on survival in hiv-infected patients but has also contributed to both an altered frequency and a different nature of rheumatic complications now being observed in this population. Rheumatologists need to be aware of these changes to provide optimal diagnosis and treatment for this group.
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10/33. Atypical lymphoplasmacytic and immunoblastic proliferation from rheumatoid arthritis: a case report.

    A case of atypical lymphoplasmacytic and immunoblastic proliferation (ALPIBP) in the lymph nodes associated with well-documented rheumatoid arthritis (RA) is presented. A 68-year-old Japanese female with a 6-year history of RA presented with right neck lymphadenopathy of 3 months duration. A biopsy specimen showed paracortical hyperplasia and numerous lymphoid follicles. On high-power field, the paracortical area was diffusely infiltrated by a polymorphous population consisting of numerous mature plasma cells, plasmacytoid cells, immunoblasts, including Hodgkin-like cells, small- to medium-sized lymphocytes, and histiocytes. Immunohistochemical study demonstrated that immunoblasts usually were CD20 , and a portion of them was CD30 . The histomorphological findings of the present case are similar to those of methotrexate (MTX)-induced atypical lymphoproliferative disorders (LPDs) in some aspects. However, Epstein-Barr virus-encoded small rna-positive cells were not identified by in situ hybridization. The polytypic nature of B lymphocytes also was demonstrated by immunohistochemistry and polymerase chain reaction. Moreover, there was no history of MTX therapy in the present case, indicating that MTX-induced, LPD-like ALPIB may occur even in the RA patients not treated with MTX therapy.
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