1/946. Pigmented purpura-like eruption as cutaneous sign of mycosis fungoides with autoimmune purpura.We describe the clinical and laboratory findings of a young man with mycosis fungoides. The disease was associated, since the early stages, with autoimmune purpura. Interferon alfa (IFN-alpha) administration improved this patient's condition, both the purpuric eruption and patchy cutaneous lesions, thus suggesting T-cell abnormalities may be responsible for the development of the disease.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
2/946. diagnosis of phacoanaphylactic endophthalmitis by fine needle aspiration biopsy.diagnosis of phacoanaphylactic endophthalmitis (or lens induced uveitis), a rare autoimmune disease, is difficult due to variable clinical presentation. We sought to diagnose a case based on the cytopathology of the anterior chamber aspirate. This is a case report of spontaneous phacoanaphylactic endophthalmitis in a 79-year-old woman with no history of eye trauma or surgery. After clinical examination, diagnostic anterior chamber paracentesis was performed. Cytologic examination of the aspirate revealed polymorphonuclear leukocytes, histiocytes, and plasma cells surrounding amorphous lens material. A mature cataract was removed subsequently, and the eye has remained free of inflammation postoperatively. As the clinical diagnosis of phacoanaphylactic endophthalmitis is often difficult, cytopathology of an anterior chamber aspiration specimen may be useful in diagnosing this rare, treatable condition. As far as we know, this is the first case report of the diagnosis of phacoanaphylactic endophthalmitis solely by anterior chamber fine needle aspiration biopsy.- - - - - - - - - - ranking = 0.5keywords = disease (Clic here for more details about this article) |
3/946. Autoimmune cholangitis in a patient with celiac disease: a case report and review of the literature.Autoimmune cholangitis is a rare chronic cholestatic liver disease. We describe the case of a 65-year-old woman with celiac disease who presented to us with fever, jaundice and weight loss. serum biochemical study showed marked increase in alkaline phosphatase and gammaGT levels. Antinuclear antibodies were positive, while antimitochondrial and anti-smooth-muscle antibodies were negative. Liver biopsy was compatible with primary autoimmune cholangitis. The patient was successfully treated with azathioprine and methylprednisolone. We describe here the uncommon association of autoimmune cholangitis with celiac disease and review the prevalence of liver diseases in patients with celiac disease.- - - - - - - - - - ranking = 4.5keywords = disease (Clic here for more details about this article) |
4/946. Hepatocellular carcinoma with fibrolamellar pattern in a patient with autoimmune cholangitis.A 75-year-old woman with a 15-year history of autoimmune cholangitis underwent orthotopic liver transplantation because of progressive liver decompensation. A clinically unsuspected hepatocellular carcinoma was found. A portion of the tumor showed fibrolamellar differentiation. Hepatocellular carcinoma, either with the usual pattern or with a fibrolamellar pattern, is rare in the setting of primary biliary cirrhosis, but has been seen in the setting of autoimmune hepatitis. Autoimmune cholangitis is a relatively recently recognized form of autoimmune liver disease whose association with hepatocellular carcinoma has yet to be determined.- - - - - - - - - - ranking = 0.5keywords = disease (Clic here for more details about this article) |
5/946. An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome.The autoimmune lymphoproliferative syndrome (ALPS) affords novel insights into the mechanisms that regulate lymphocyte homeostasis and underlie the development of autoimmunity. This syndrome arises early in childhood in persons who inherit mutations in genes that mediate apoptosis, or programmed cell death. The timely deletion of lymphocytes is a way to prevent their accumulation and the persistence of cells that can react against the body's own antigens. In ALPS, defective lymphocyte apoptosis permits chronic, nonmalignant adenopathy and splenomegaly; the survival of normally uncommon "double-negative" CD3 CD4- CD8- T cells; and the development of autoimmune disease. Most cases of ALPS involve heterozygous mutations in the lymphocyte surface protein Fas that impair a major apoptotic pathway. Detailed immunologic investigations of the cellular and cytokine profiles in ALPS show a prominent skewing toward a T-helper 2 phenotype; this provides a rational explanation for the humoral autoimmunity typical of patients with ALPS. Prospective evaluations of 26 patients and their families show an ever-expanding spectrum of ALPS and its major complications: hypersplenism, autoimmune hemolytic anemia, thrombocytopenia, and neutropenia. Defective apoptosis may also contribute to a heightened risk for lymphoma.- - - - - - - - - - ranking = 0.5keywords = disease (Clic here for more details about this article) |
6/946. Acute hemorrhagic leukoencephalitis in patients with acute myeloid leukemia in hematologic complete remission.The authors describe the cases of three patients affected by acute myeloid leukemia, in complete remission, who rapidly developed neurologic symptoms leading to death. Neither clinical characteristics, nor radiological or microbiological procedures, allowed an etiological diagnosis of the neurologic syndrome. Post-mortem examination of the brain showed both macroscopic and microscopic findings compatible with acute hemorrhagic leukoencephalitis. The difficulty in distinguishing this entity from other CNS disease-related complications (e.g. leukemia infiltration, drug toxicity, hemorrhages) should not lead to an underestimation of the true incidence of this complication. We believe that with more attention to the possibility of this complication there would probably be both a greater possibility of collecting clinical informations about the real impact of this dramatic disease and a stronger hope of finding the right treatment for it.- - - - - - - - - - ranking = 1keywords = disease (Clic here for more details about this article) |
7/946. headache and bilateral visual loss in a young hypothyroid Indian man.We describe the exceptional association of Vogt-Koyanagi-Harada syndrome (VKHS) and hypothyroidism in a 29-year-old man of Indian heritage. VKHS is a rare uveomeningoencephalitic syndrome with probably autoimmune pathogenesis. Nontraumatic uveitis, aseptic meningoencephalitis, vitiligo, alopecia and poliosis are the leading clinical features of VKHS. The reported patient presented with bilateral visual loss and progressive frontal headache. VKHS was diagnosed due to characteristic ophthalmological findings and the diagnosis of aseptic meningitis. Due to the autoimmune pathogenesis, VKHS may be rarely associated with other autoimmune disorders. association of VKHS with autoimmune thyroid disease has been described in the literature in three patients. In the reported case hypothyroidism due to chronic autommune thyroiditis was diagnosed in association with VKHS. Routinely determination of thyroid function in patients with VKHS is recommended.- - - - - - - - - - ranking = 0.5keywords = disease (Clic here for more details about this article) |
8/946. Autoimmune atrophic gastritis with hypergastrinemia.Elevation in fasting serum gastrin levels was found in three patients being evaluated for persistent upper abdominal pain without radiographic evidence of peptic ulcer disease. Fiberoptic endoscopy of the upper gastrointestinal tract in each patient revealed characteristic changes of chronic atrophic gastritis. Gastric biopsies showed diffuse chronic inflammation in the lamina propria, a decrease in the number of parietal cells, and "intestinalization" of gastric mucosa. Total achlorhydria was demonstrated after a maximal histalog stimulus; however, serum levels of vitamin B12 and schilling test values were normal in all three patients. Parietal cell antibodies were found in the serum in all patients in a dilution of 1:20 to 1:80. These cases represent autoimmune (type A) chronic atrophic gastritis and should be distinguished from chronic simple (type B) gastritis, in which serum gastrin levels are normal and no parietal cell antibodies are found in the serum. patients with autoimmune gastritis should be observed at frequent intervals for the occurrence of pernicious anemia or gastric carcinoma.- - - - - - - - - - ranking = 0.5keywords = disease (Clic here for more details about this article) |
9/946. autoimmunity and extranodal lymphocytic infiltrates in lymphoproliferative disorders.OBJECTIVE: To examine the relationship between autoimmunity and extranodal lymphocytic infiltrates in different lymphoproliferative disorders with immunoglobulin alterations. SUBJECTS AND DESIGN: A clinical review combined with a retrospective cohort study of 380 patients, 28 with monoclonal gammopathy of undetermined significance, three with common variable immunodeficiency, 147 with chronic lymphocytic leukaemia, 57 with Waldenstrom's macroglobulinaemia and 145 with non-Hodgkin's malignant lymphoma. SETTING: A university hospital and The State serum Institute in Copenhagen. INTERVENTION: Clinical examination of each patient with special attention to chronic inflammatory and autoimmune manifestations. Biopsies were taken from non-infectious infiltrates, some of which were additionally tested with PCR analysis for gene rearrangements. Serological screening with a test battery for various autoantibodies was used in combination with techniques for the detection of M-components and monoclonal B-cell proliferation. MAIN OUTCOME MEASURES: Clinical and/or serological autoimmune manifestations, M-component and other immunoglobulin alterations, and inflammatory tissue changes were studied in patients with chronic inflammatory, polyclonal or oligoclonal pseudolymphomas and in monoclonal, malignant extranodal lymphomas. RESULTS: In 380 consecutive patients, 49 (12.9%) had extranodal manifestations, of whom 47 also had autoimmune manifestations. Nearly half of the 47 patients had more than one autoimmune manifestation. There was a strong correlation between clinical signs and corresponding autoantibodies such as anti-SSA and -SSB antibodies in sjogren's syndrome (10 cases), antithyroid peroxidase antibodies in thyroiditis and Graves' disease (10 cases), and parietal cell antibodies in gastric ulcers with maltoma (12 cases). Clinical and serological signs of autoimmunity correlated strongly with female sex (34, 72% women; and 13, 28% men) and with immunoglobulin alterations. CONCLUSIONS: To our knowledge this is the first systematic review of B-lymphoproliferative and autoimmune disorders indicating that pseudolymphoma and malignant lymphomas, including maltomas, may develop in the context of a permanent autoantigenic drive.- - - - - - - - - - ranking = 0.5keywords = disease (Clic here for more details about this article) |
10/946. Three different autoimmune bullous diseases in one family: is there a common genetic base?We report an unusual familial occurrence of autoimmune bullous diseases. Three members of a family suffered from three different autoimmune bullous diseases: pemphigus vulgaris (PV), linear IgA disease (LAD) and cicatricial pemphigoid (CP). The HLA type was determined in five family members: all were positive for HLA-DQ5/DR6, which is reported to be associated with susceptibility to PV. The CP patient was DQ7(3) positive, which is in concordance with enhanced susceptibility to ocular CP and CP. The LAD patient was B8 and DR3 negative but positive for HLA-A1. Our study supports the hypothesis that there is a genetically transmitted susceptibility to autoimmune bullous diseases but that additional factors seem necessary actually to develop a particular disease.- - - - - - - - - - ranking = 4.5keywords = disease (Clic here for more details about this article) |
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