Cases reported "Autoimmune Diseases"

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11/14. Recurrent demyelinating transverse myelitis in a high titer HBs-antigen carrier.

    A 46-year-old man who was an extremely high titer hepatitis B surface (HBs)-antigen carrier had three attacks of acute demyelinating transverse myelitis associated with signs of meningeal irritation. Each episode showed good response to corticosteroid therapy. The cerebrospinal fluid was characterized by elevated levels of myelin basic protein (MBP) and predominating CD4 CD29 helper-inducer T cells during the acute stages, and also by persistently positive HBs antigen. There were neither autoantibodies nor evidence of vasculitis. However, circulating immune complexes composed of HBs antigen disappeared after treatment, indicating that immunity to hepatitis b virus played a role in the demyelinative lesion formation in the central nervous system (CNS). In light of the animal studies demonstrating autoimmunity triggered by molecular mimicry between MBP and hepatitis b virus antigens, this patient may serve as a rare example of CNS demyelination in humans with the same autoimmune etiology.
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12/14. Late development of anti-La/SS-B antibodies in a patient with sjogren's syndrome and high titer anti-Ro/SS-A antibodies.

    The frequent coexistence of anti-Ro and anti-La autoantibodies is well described, however, there is little evidence of sequential development of these two autoantibodies. We report a case of typical sjogren's syndrome with high titer anti-Ro antibodies, who subsequently developed anti-La antibodies later in the course. This case suggests that the anti-La antibodies may actually follow the anti-Ro antibodies in some cases as hypothesized in the concept of linked set of autoantibodies, analogous to development of anti-Sm in certain anti-nRNP antibody positive SLE patients and animal models.
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13/14. sarcoidosis with thyroid involvement, polymyalgia rheumatica and breast carcinoma. A case report.

    polymyalgia rheumatica developed in a 71-year-old patient within 2 years of the onset of acute sarcoidosis with biopsy-verified involvement of the thyroid, and concomitant autoimmune thyroiditis with hyperthyroid symptoms. Three years after the onset of muscle symptoms a non-metastasizing breast carcinoma was discovered and treated surgically. Neither the long interval between the onset of polymyalgia rheumatica and the discovery of the breast tumour, nor the good response of muscle symptoms to a one-year maintenance treatment with corticosteroids, was consistent with a paraneoplastic mechanism of the polymyalgia rheumatica syndrome. It was therefore hypothesized that the various disorders suffered by this patient might be related to a partly age-dependent depression of T-lymphocyte function, leading to an altered immunological reactivity to which the various clinical manifestations could be attributed. Such a hypothesis is supported by recent reports showing that in old people and in ageing experimental animals, a decrease in T-lymphocyte function and in the number of circulating T-cells occurs concomitantly with an increase in the incidence of a variety of neoplasms and autoimmune disorders.
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14/14. Autoimmune sensorineural hearing loss: a human temporal bone study.

    PURPOSE: To describe histopathologic findings in temporal bones of a patient whose clinical history suggests a sensorineural hearing loss (SNHL) of autoimmune origin. MATERIALS AND methods: Temporal bones from a patient with a history of ulcerative colitis, leukemia, and SNHL were examined by light microscopy. RESULTS: Histopathologic findings included: (1) organs of Corti missing or absent in all cochlear turns; (2) cells decreased in spiral ganglia, and lymphocytic infiltration; (3) absence of portions of the spiral prominence; (4) endolymphatic hydrops in basal, middle, and apical cochlear turns and in the saccule and utricle; (5) fibrosis and osteoneogenesis of a scala tympani of the basal turn of the cochlea, the posterior semicircular canal, and the canal of Cotugno; (6) fibrosis of the vestibular aqueduct and endolymphatic sac; and (7) lymphocytes in the endolymphatic sac, perisaccular area, inferior cochlear vein, and Rosenthal's canal. CONCLUSION: Histopathologic findings in the temporal bones of this patient with ulcerative colitis, sensorineural hearing loss, and vestibular symptoms closely parallel those in a previously reported animal study of autoimmunity and suggest the possibility of a SNHL of autoimmune origin.
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