Cases reported "Autoimmune Diseases"

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11/80. Rapid increase in bone mineral density in a child with osteoporosis and autoimmune hypoparathyroidism treated with PTH 1-34.

    We describe a 16-year-old girl with autoimmune polyglandular syndrome type 1 including hypoparathyroidism, who had osteoporosis that improved rapidly with parathyroid hormone replacement therapy. patients with hypoparathyroidism usually have high bone mass. Our patient developed vertebral compression fractures at age 10, shortly after hypoparathyroidism was diagnosed. She continued to have low lumbar bone mass until age 16, when a dual energy x-ray absorptiometry (DEXA) revealed a Z score of - 2.2 SD. Several factors including decreased physical activity, total body magnesium depletion, and intermittent ketoconazole and short-term prednisone treatment, may have contributed to the development and progression of osteoporosis. Therapy with synthetic human parathyroid hormone (PTH) 1-34 rapidly normalized lumbar bone mass, as assessed by DEXA.
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keywords = gland
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12/80. Autoimmune cholangitis in patients with primary sjogren's syndrome. A case report.

    sjogren's syndrome is a very frequent autoimmune disease, characterised by exocrine gland involvement. Immunologic disorders are also responsive for extraglandular manifestations of the disease, mostly for the digestive involvement. We report a case of primary sjogren's syndrome with multiple extraglandular manifestations: vasculitis, cryoglobulinaemia, hepatic involvement and presumably neurologic involvement. The particularities of the case are the typical pattern of autoimmune cholangitis, hypogammaglobulinaemia, the absence of antinuclear antibodies and the association with vasculitis. To the best of our knowledge, this is the first case report of autoimmune cholangitis in sjogren's syndrome
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keywords = gland
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13/80. Recurrent and reversible cerebellar ataxia with concomitant episodes of hyperthyroidism: a new autoimmune syndrome.

    A new disorder with possible autoimmune background is described in a 67-year-old man. He had experienced episodes of cerebellar ataxia induced by infections over the last 40 years. In 1963 he had thyrotoxicosis. The patient underwent bilateral subtotal thyroidectomy. Recently it has been possible to detect involvement also of his remaining thyroid gland, with findings compatible with hyperthyroidism concomitant with his cerebellar atixia.
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keywords = gland
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14/80. First male case of lymphocytic hypophysitis in korea.

    Lymphocytic hypophysitis is a rare inflammatory disorder which is caused by autoimmune destruction of the pituitary gland. Almost all reported cases have been in women and the disease is often associated with pregnancy. We describe here the first male case of lymphocytic hypophysitis in korea. The patient presented with headache, impotence, decreased libido, and deteriorated vision. Endocrinologic studies showed panhypopituitarism, and pituitary MRI imaging revealed a homogeneously enhanced pituitary mass with a thickened stalk. Treatment with prednisolone and thyroid hormone for five months was ineffective. Transsphenoidal resection of the pituitary mass was performed successfully with normalization of the visual field defect. Histologic examination revealed diffuse lymphocytic infiltration with dense collagenous fibrosis, consistent with lymphocytic hypophysitis. Lymphocytic hypophysitis should be considered in differential diagnosis even in men with hypopituitarism and an enlarged pituitary gland.
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keywords = gland
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15/80. Gamma-heavy chain disease: review of 23 cases.

    We report the cases of 23 patients with gamma-heavy chain disease seen at our institution (8 patients previously reported, 15 new patients). There were 15 women and 8 men; the median age at diagnosis was 68 years (range, 42-87 yr). Sixteen patients had an associated lymphoplasma cell proliferative disorder, 3 had a lymphoplasma cell proliferative disorder and an autoimmune disorder, another 3 had an autoimmune disorder only, and 1 had no underlying disease. The lymphoplasma cell proliferative disorder was disseminated in 10 patients and localized in 6. patients with localized lymphoplasma cell proliferative disorder included 3 with plasmacytoma (1 tongue, 1 submandibular area, and 1 thyroid), 2 with lymphoplasma cell proliferative disorder involving the bone marrow only, and 1 with amyloid of the skin. At the time of diagnosis, lymphadenopathy was present in 8 patients, splenomegaly in 7, and hepatomegaly in 1. A monoclonal spike on serum protein electrophoresis was documented in 19 patients. gamma-Heavy chain was documented by immunofixation in the serum of all patients; 2 had an additional immunoglobulin m-lambda. gamma-Heavy chain was present in the urine in 19 of 22 patients. Sixteen patients were treated for lymphoplasma cell proliferative disorder or autoimmune disorder (14 with chemotherapy, 1 splenectomy, and 1 thyroidectomy followed by radiation therapy). For 5 patients, treatment was not felt to be necessary; 2 patients were thought to be too sick for treatment. Of the 16 patients treated, 6 had a complete clinical response (in 2, gamma-heavy chain disappeared; in 2, gamma-heavy chain persisted; and for 2, no serologic follow-up was available); in 10 patients, clinical disease persisted (in 3, gamma-heavy chain disappeared; in 6, it persisted; and for 1, no serologic follow-up was available). Of 7 patients not treated, 2 died within 5 months; 1 died after 15 months; 2 had no clinical disease at latest follow-up, although gamma-heavy chain persisted; and 2 had no change in clinical and serologic status. The median duration of follow-up was 33 months (range, 1-261 mo). Median survival was 7.4 years.
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ranking = 4.6348823818643
keywords = submandibular
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16/80. Activating antibodies to the calcium-sensing receptor in two patients with autoimmune hypoparathyroidism.

    Autoimmune hypoparathyroidism is thought to result from immune-mediated destruction of the parathyroid glands. We encountered two patients with hypoparathyroidism and other autoimmune conditions (Graves' disease and Addison's disease, respectively) in whom autoimmune destruction of the parathyroid glands had not taken place. In the first, a histologically normal parathyroid gland was observed at the time of subtotal thyroidectomy; and in the second, the hypoparathyroidism remitted spontaneously. Both patients had antibodies that reacted with the cell surface of bovine parathyroid cells and human embryonic kidney (HEK293) cells transfected with the extracellular calcium-sensing receptor (CaR) but not with nontransfected hek293 cells. The antibodies also reacted with the same bands on Western analysis of extracts of bovine parathyroid tissue and CaR-transfected hek293 cells that were identified by an authentic, polyclonal, anti-CaR antiserum and reacted with several peptides with sequences from the CaR's extracellular domain. These anti-CaR antibodies activated the receptor based on their ability to increase inositol phosphate accumulation, activate MAPK, and inhibit PTH secretion. These results, therefore, demonstrate that patients with the biochemical findings of primary hypoparathyroidism can harbor activating antibodies to the CaR, which, in the two cases studied here, did not produce irreversible destruction of the parathyroid glands.
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keywords = gland
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17/80. Lymphocytic hypophysitis with a long latent period before development of a pituitary mass.

    BACKGROUND: Lymphocytic hypophysitis is an autoimmune condition that commonly presents in women of childbearing age as hypopituitarism and a sellar mass. CASE REPORT: A 66-year-old woman presented with anterior pituitary dysfunction. Computed tomography imaging revealed a small hypodensity that was not felt to be the cause of the pituitary dysfunction. Eight years later, her vision rapidly deteriorated and MRI showed a pituitary mass lesion causing optic chiasm compression. Histological examination of the partially resected gland revealed evidence of lymphocytic hypophysitis. CONCLUSION: Our patient is an example of the variable presentation and course of lymphocytic hypophysitis. Such a long latent period between the initial presentation of adenohypophysial hypofunction and optic chiasm compression due to an enlarging pituitary mass has not been reported.
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18/80. Preoperative diagnosis of lymphocytic hypophysitis (adenohypophysitis) unresponsive to short course dexamethasone: case report.

    Lymphocytic hypophysitis (adenohypophysitis) is a rare lymphocytic infiltration of the pituitary gland that usually occurs during pregnancy. Because of its rarity, it has seldom been diagnosed preoperatively, and no trials of therapeutic treatment have been reported to date. We describe a 29-year-old woman with a pituitary mass and visual-field defects during pregnancy. The patient's pituitary profile revealed an abnormal thyroid axis and relatively low prolactin for her stage of pregnancy. This finding suggested adenohypophysitis, and the patient was given a trial course of dexamethasone. The progression of the visual-field defects, however, indicated that the steroids, both in dosage and duration, were not effective. Thus, the patient underwent a partial hypophysectomy for decompression. The pathology report confirmed adenohypophysitis, and steroids were continued for the remainder of the pregnancy, with slow resolution of the visual-field defects to normal. This report is the first case of adenohypophysitis where the diagnosis was suspected preoperatively, and a trial course of steroids was given. The steroids at the dosage and duration used were not effective. Further evaluation of the use of steroids in this disease is warranted.
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19/80. Primary salivary gland lymphoma: a clinicopathologic study of 23 cases in taiwan.

    Twenty-three patients with primary salivary gland lymphoma were diagnosed between 1990 and 2001. The sites of involvement were the parotid gland in 13, the submandibular gland in 9 and the minor salivary gland in 1. The sites of lymphoma involvement beyond the salivary glands were the cervical lymph nodes in 7, bone marrow in 3, the axillary lymph nodes in 3, the nasopharynx in 2, the abdominal lymph nodes in 2, the palate, the subconjunctiva, and the spleen in 1 each patient. Histologically, 19 patients had lymphomas of mucosa-associated lymphoid tissue (MALT) with myoepithelial sialadenitis in 13, 3 patients had diffuse large cell lymphomas and 1 had follicular lymphoma. Six patients were in stage I, 4 in II, 1 in III and 12 in IV. Eight of 23 patients (35%) had autoimmune diseases before or after the diagnosis of NHL and all suffered from MALT lymphoma. Four patients with parotid MALT lymphoma had primary or secondary sjogren's syndrome. One each patient suffered from hyperthyroidism, systemic lupus erythematosus, membranoproliferative glomerulonephritis and cryoglobulinemia, respectively. All the 6 stage I patients had achieved complete remission (CR) without relapses 17-84 months (median 44 months) after treatment. Excluding a stage IV patient with follicular lymphoma who died at 3.5 months without treatment, CR was achieved in all of the remaining 16 patients. However, a high relapse rate (9/16, 56%) was noted in stage II-IV patients. These patients tended to relapse in the original sites, but achieved CR again after chemotherapy or radiotherapy. One patient with MALT lymphoma developed histologic transformation into diffuse large lymphoma during relapse and died of refractory disease. overall, only 2 patients succumbed. The overall survival and relapse-free survival rates at 5 years were 94.7 and 51.4%, respectively. Thus, salivary gland lymphoma proved to be an indolent disease.
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ranking = 47.282589166027
keywords = submandibular gland, submandibular, gland
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20/80. Autoimmune polyglandular syndrome type III in monozygotic twins: a case report.

    Autoimmune polyglandular syndrome is characterized by the coexistence of several autoimmune diseases, affecting predominantly the endocrine glands. Autoimmune polyglandular syndrome type III, as a subdivision of autoimmune polyglandular syndrome type II, is the co-occurrence of autoimmune thyroid disease with other autoimmune disorders without addison disease. We present a rare case of autoimmune polyglandular syndrome type III in monozygotic twins. One of the twins also had autoimmune leukopenia. To our knowledge, leukopenia is the first mentioned coexistence in the literature of autoimmune polyglandular syndrome.
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keywords = gland
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