Increased prevalence of DRw3 in the CREST syndrome.
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Biomedical subjects
Publications and source records attributed to B F Germain.
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A patient with relapsing polychondritis who presented with rapidly progressive glomerulonephritis is described. This is the fourth case in which this association is reported. Crescentic glomerulonephritis was found on renal biopsy. Evidence for immunologic participation in the pathogenesis of this condition is suggested by the demonstration of circulating immune complexes, immunoglobulin and complement deposition by immunofluorescence, and electron dense deposits by electron microscopy of the kidney. Favorable response followed therapy with prednisone and dapsone.
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Arthritis developed in two patients during the course of parasite infestation with Strongyloides stercoralis and Taenia saginata, respectively. The joint involvement was polyarticular and symmetrical but seronegative and nonerosive radiologically. We found evidence of abnormal humoral immunity to the parasites, immune complexes in serum and synovial fluid, and immunoglobulin deposits in the synovia. Nonsteroidal anti-inflammatory agents proved ineffective but specific antiparasitic treatment resulted in resolution of the symptoms and immunologic abnormalities. Our findings provide further documentation of the interaction between the body's immune system and parasites and suggest that arthritis induced by parasitic infestation may be mediated by immune complex formation in susceptible hosts.
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Pancreatic pseudocyst formation leading to fatal outcome was observed during the active phase of polyarteritis nodosa in a 48-year-old patient. Persistent hepatitis B antigenemia and high levels of circulating immune complexes were present during his illness. Prednisone and azathioprine therapy was not able to control the manifestations of the basic disorder.
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Gas gangrene is a rare infectious disease syndrome complicating medico-surgical procedures. We describe a case of gas gangrene secondary to intra-articular steroid injection. Clostridia species and Escherichia coli were the etiologic organisms in this case. The presence of underlying diseases such as diabetes mellitus, hepatic insufficiency, and metabolic acidosis could have contributed to the fatal outcome of this patient. A high index of suspicion, early diagnosis, and appropriate treatment may improve the prognosis in gas gangrene. Although uncommon, infection is a significant complication of intra-articular steroid administration. Thus, meticulous aseptic technique should always be observed in the performance of this procedure.
Circulating immune complexes (CIC) were detected in a large population of patients with polymyalgia rheumatica (PMR) alone, PMR-giant cell arteritis (GCA), and GCA alone. CIC were found in the majority of patients studied. A direct correlation was observed between levels of CIC material and the erythrocyte sedimentation rate. The presence of immune complexes in the sera of patients with these disorders do not have diagnostic significance but may play an important role in their pathogenesis.
The etiology and pathogenesis of acne arthritis and the arthritis of hidradenitis suppurativa remain unknown. Some patients have an asymmetrical pauciarticular arthritis compatible with the 'reactive' peripheral and central forms of Reiter's syndrome, whereas others have a symmetrical polyarthritis/polyarthralgias syndrome. The cutaneous disease is clinically manifest as acne conglobata or hidradenitis of axilla or groin. We determined human leukocyte antigens (HLA), levels of immunoglobulins, C3, C4 and circulating immune complexes from six patients with acne arthritis. Four of six patients had HLA antigens of B7 Creg group (B7, BW22, B27, BW40, BW42) and all six possessed DRW4. Isolated elevations of immunoglobulins, C3 and C4 were observed. Immune complexes were elevated uniformly. These observations suggest that immunogenetic mechanisms may play a role in the systemic manifestations of these skin diseases.
Methotrexate in a low dose intermittent regimen has become popular as a therapeutic choice for refractory psoriatic arthritis, polymyositis, Reiter's disease and more recently rheumatoid arthritis. As a folate analogue, it inhibits the formation of reduced folate cofactors which participate in a host of important reactions including DNA synthesis. It has been shown to have both immunosuppressive and anti-inflammatory properties although its precise mechanism of action in these diseases is not known. It may be variably absorbed especially in psoriatics and its action may be antagonized by folate supplements. Its major route of metabolism appears to be via the enterohepatic circulation. Numerous drugs, including salicylates, may increase serum levels by displacing the drug from protein binding sites and by competing for renal excretion. It has fewer short term side effects than the other immunosuppressives used in rheumatic disease. Its major long term toxicity is liver fibrosis or cirrhosis which appears to increase with greater cumulative dosage and treatment duration. Several recent reports of hypersensitivity pneumonitis reinforce the previous literature on this topic. Pulmonary toxicity may be more common than suggested as more patients are treated with methotrexate for rheumatic diseases. Clinical studies in general have been uncontrolled and lacking in scope and size. Nevertheless, the literature to date appears to show this to be an excellent drug for use in the diseases mentioned. Prospective double blind controlled studies on psoriatic arthritis and rheumatoid arthritis should help establish this drug as the immunosuppressive of choice in these diseases.