Cases reported "Autoimmune Diseases"

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21/80. Overlap syndrome of autoimmune pancreatitis and cholangitis associated with secondary sjogren's syndrome.

    In approximately 25% of patients with acute or chronic pancreatitis the cause remains unclear. Despite progress in understanding so-called idiopathic pancreatitis, more diagnostic criteria are needed. We report on a patient who presented with jaundice, but without pain or fever. Under the assumption of pancreatic cancer the patient underwent hemipancreatoduodenectomy. Histological examination showed chronic sclerosing inflammation of the pancreas and bile ducts without any signs of malignancy. Ten weeks later he developed bilateral parotid swelling and recurrent bouts of fever. Again liver enzymes were elevated and unsuccessfully treated with antibiotics for bacterial cholangitis. Further biopsies from submandibular gland, lymph nodes and liver again showed chronic sclerosing inflammation with lymphoplasmacytic infiltration. For sicca symptoms the diagnosis of a primary sjogren's syndrome was proposed. However, with corticosteroid treatment the patient improved remarkably but after tapering he relapsed. On the basis of established criteria, we diagnosed autoimmune pancreatitis with (1) diffuse swelling of the pancreas, (2) irregularities of the pancreatic duct, (3) lymphoplasmacytic infiltration, (4) response to corticosteroids, (5) hypergammaglobulinaemia, and (6) disproportionately raised IgG4. In addition, the patient fulfilled the criteria for secondary sjogren's syndrome. Autoimmune pancreatitis may present as an isolated or syndromic disease. It is an autoimmune disorder of unknown cause and should be included in the differential diagnosis of pancreatic disorders.
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keywords = submandibular gland, submandibular, gland
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22/80. alopecia areata associated with idiopathic primary hypophysitis.

    alopecia areata has been reported in association with various autoimmune diseases. Idiopathic primary hypophysitis is an organ specific autoimmune disease affecting the pituitary gland. We report a case of alopecia areata occurring in a patient of idiopathic primary hypophysitis. The constellation of the two diseases can be explained by autoimmunity, which is a major aetiologic factor in both diseases. To the best of our knowledge, this is the first report of such an association.
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ranking = 0.02612153518831
keywords = gland
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23/80. lymphoma in sjogren's syndrome.

    sjogren's syndrome is an autoimmune disease with a known predisposition for lymphoma development. Eight of 120 patients with primary sjogren's syndrome followed at the University of Ioannina over the past 7 years developed non-Hodgkin's lymphoma diagnosed according to the Kiel classification. The lymphomas differed by location and grading. Six were called low grade (immunocytoma) and two intermediate grade non-Hodgkin's lymphomas. Five of the immunocytomas involved the minor salivary or lacrimal glands. Immunoperoxidase staining for light chains revealed monoclonal populations. Two patients showed spontaneous regression not previously reported in sjogren's syndrome. Thus, in sjogren's syndrome, low grade non-Hodgkin's lymphomas and especially immunocytomas are the most common lymphomas. These lymphomas tend to evolve very slowly and may regress spontaneously. Given these facts, a conservative approach to treatment is indicated in those patients with only localized disease.
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ranking = 0.02612153518831
keywords = gland
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24/80. Autoimmune phenomena following prostatectomy.

    BACKGROUND: Benign prostatic hypertrophy is the most common benign tumor in males, resulting in prostatectomy in 20-30% of men who live to the age of 80. There are no data on the association of prostatectomy with autoimmune phenomena in the English-language medical literature. OBJECTIVES: To report our experience with three patients who developed autoimmune disease following prostatectomy. patients: Three patients presented awith autoimmune phenomenon soon after a prostectomy for BPH or prostatic carcinoma: one had clinically diagnosed temporal arteritis, one had leukocytoclastic vasculitis, and the third patient developed sensory Guillian-Barre syndrome following prostatectomy. CONCLUSIONS: In view of the temporal association between the removal of the prostate gland andthe autoimmune process, combined with previously known immunohistologic features of BPH, a cause-effect relationship probably exists.
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ranking = 0.02612153518831
keywords = gland
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25/80. Autoimmune pancreatitis with IgG4-positive plasma cell infiltration in salivary glands and biliary tract.

    A 62-year-old male was referred to our hospital because of liver dysfunction, diffuse pancreatic swelling, and trachelophyma. At admission, the patient was free of pain. physical examination showed enlarged and palpable bilateral submandibular masses, but no palpable mass or organomegaly in the abdomen. Laboratory findings were as follows: total protein 90 g/L with gamma-globulin of 37.3% (33 g/L), total bilirubin 4 mg/L, aspartate aminotransferase 39 IU/L, alanine aminotransferase 67 IU/L, gamma-glutamyl transpeptidase 1 647 IU/L, and amylase 135 IU/L. autoantibodies were negative, and tumor markers were within the normal range. serum IgG4 level was markedly elevated (18 900 mg/L). Computed tomography (CT) showed diffuse swelling of the pancreas and dilatation of both common and intra-hepatic bile ducts. Endoscopic retrograde pancreatography (ERP) revealed diffuse irregular and narrow main pancreatic duct and stenosis of the lower common bile duct. biopsy specimens from the pancreas, salivary gland and liver showed marked periductal IgG4-positive plasma cell infiltration with fibrosis. We considered this patient to be autoimmune pancreatitis (AIP) with fibrosclerosis of the salivary gland and biliary tract, prescribed prednisolone at an initial dose of 40 mg/d. Three months later, the laboratory data improved almost to normal. Abdominal CT reflected prominent improvement in the pancreatic lesion. Swelling of the salivary gland also improved. At present, the patient is on 10 mg/d of prednisolone without recurrence of the pancreatitis. We present here a case of AIP with fibrosclerosis of salivary gland and biliary tract.
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ranking = 0.33004252473803
keywords = submandibular, gland
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26/80. A case of IgG4-related autoimmune disease with multiple organ involvement.

    A 52-year-old male was admitted with autoimmune pancreatitis (AIP), showing mononuclear cell infiltration in both the pancreas and salivary glands with both normal sialography and anti-SS-A/SS-B antibodies. Although the AIP improved with glucocorticoid treatment, subsequent abdominal computed tomography (CT) revealed a nodular shadow in the bilateral kidneys, which was confirmed as interstitial nephritis by renal biopsy. The patient's serum immunoglobulin G4 (IgG4) level was 10 times higher than the upper limit of the normal range. IgG4-positive mononuclear cell infiltration was detected in the salivary gland, pancreas, and kidney. A new entity proposed as 'IgG4-related autoimmune disease' was considered.
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ranking = 0.05224307037662
keywords = gland
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27/80. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease.

    We report three elderly men with high serum IgG4 concentrations and multiple lymphoplasmacytic inflammation of the salivary glands, lymph nodes, pancreas, and renal interstitium. The infiltrating plasma cells had strong immunoreactivity for IgG4, even in patients without pancreatic lesions. These cases show that IgG4 is associated not only with autoimmune pancreatitis, but also with other systemic lymphoplasmacytic disease.
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ranking = 0.02612153518831
keywords = gland
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28/80. EBV lymphoepithelial carcinoma of the parotid gland in Mexican Mestizo patients with chronic autoimmune diseases.

    Lymphoepithelial carcinomas of the salivary gland are rare tumors constantly associated with Epstein-Barr virus (EBV) and mainly identified in Asiatic and Greenlander population. Four cases have been described in Caucasians, only two with EBV infection. We describe two cases of parotid gland lymphoepithelial carcinomas in Mexican mestizo women in which chronic latent EBV infection was documented by immunohistochemistry and in situ hybridization. One patient had primary sjogren's syndrome and the other systemic lupus erythematosus of six and three years of evolution, respectively. Epithelial neoplastic cells showed latency pattern II (LMP1 , EBNA-2-, EBER ) with a dense inflammatory infiltrate composed mainly by CD8 T lymphocytes. Follow-up excluded nasopharyngeal involvement in both patients. This report expands the ethnic groups in which salivary lymphoepithelial carcinomas associated with chronic latent EBV infection have been described, and illustrates for the first time its association with autoimmune diseases in two women living in a region non-endemic for this unusual neoplasm.
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ranking = 0.15672921112986
keywords = gland
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29/80. Complete anterior pituitary failure and postpartum cardiomyopathy.

    OBJECTIVE: To present a case of presumed autoimmune hypophysitis that occurred concurrently with severe postpartum cardiomyopathy and pneumonitis. methods: We describe the clinical, laboratory, and imaging findings in a young postpartum woman who presented with decompensated heart failure. RESULTS: Two weeks after childbirth, a 37-year-old previously healthy woman required urgent pericardiocentesis and inotropic support because of new-onset left ventricular systolic dysfunction, pericardial effusions, and hypotension. Analysis of pericardial fluid was negative for malignant cells and culture, and no cardiac tamponade or thrombus was evident. Results of a rheumatologic serology survey were negative, as was an assessment for antithyroid antibodies. Chest radiography revealed bilateral pleural effusions. magnetic resonance imaging of the pituitary showed a homogeneously enlarged gland, consistent with the postpartum state, but no discrete pituitary lesions. Laboratory results included low levels of thyrotropin, free triiodothyronine, free thyroxine, and cortisol and a high erythrocyte sedimentation rate. The patient's symptoms responded to prednisone therapy (60 mg/day) as well as an angiotensin-converting enzyme inhibitor and a b -adrenergic blocking agent. Follow-up magnetic resonance images showed an atrophic pituitary with an empty sella turcica. CONCLUSION: To our knowledge, this is the first reported case of concomitant presumed autoimmune hypophysitis, complete anterior pituitary failure, postpartum cardiomyopathy, and pneumonitis.
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ranking = 0.02612153518831
keywords = gland
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30/80. An informative case of Graves' disease with implications for schizophrenia.

    The aetiology of schizophrenia and the other psychoses is not yet established. The Knight model, based on genetic and other evidence, proposes that schizophrenia is an autoimmune disease, caused by the development of forbidden clones of B lymphocytes that secrete autoantibodies that accidentally stimulate cell surface receptors on certain neurons, affecting the limbic system of the brain. An unusual defect in a Maori man with Graves' disease rendered him unresponsive to the usually effective antithyroid drugs, prompting his being treated with prednisone, a non-specific immunosuppressant agent. This was highly successful, reducing the blood level of the causative thyroid-stimulating autoantibodies with reduction of thyroid hormone levels and thyroid gland size. Unfortunately, high dosage prednisone can be used for only a month, because of steroid toxicity. A research pathway to effective therapy of receptor-mediated autoimmune diseases, which probably include the psychoses, is now apparent. It involves finding the autoantibodies, then cloning of their antigenic targets, as has been done for Graves' disease. This will provide knowledge of the peptide sequences necessary for constructing therapeutic agents for selectively destroying the pathogenic forbidden clones. Meanwhile, usage of short-term therapy with prednisone could be helpful in the management of schizophrenia and should be explored.
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ranking = 0.02612153518831
keywords = gland
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