Cases reported "Autoimmune Diseases"

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1/39. High anti-golgi autoantibody levels: an early sign of autoimmune disease?

    IgG anti-Golgi complex antibodies were detected by means of indirect immunofluorescence in the sera of five patients during routine investigation for suspected systemic autoimmune disease. The typical picture of paranuclear fluorescence was observed on the HEp-2 cell line and in tissue sections; anti-Golgi specificity was confirmed on the HEp-2 cells using the immunoperoxidase method. The phenomenon was transient in three patients with a probable viral infection and whose sera had low titre antibodies; however, it was persistent and in high concentration in the other two who, five years later, developed an autoimmune disease. Only in the sera of these last two patients were specific bands of 123, 72, 46, 37 and 26 kilodaltons found by the immunoblotting technique on cytoplasmic extracts. Although the detection of anti-Golgi autoantibodies is rare, and may represent a transitory epiphenomenon in patients with a viral infection, their presence in high titre in the absence of a clear clinical picture may constitute an early sign of systemic autoimmune disease.
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2/39. Coexistent lymphoid interstitial pneumonia, pernicious anemia, and agammaglobulinemia.

    immunologic factors have been incriminated in the pathogenesis of lymphoid interstitial pneumonia. The discovery of a patient with coexistent lymphoid interestitial pneumonia, pernicious anemia, and common variable hypogammaglobulinemia focused attention on the possible autoimmune nature of this pulmonary disease. Extensive immunologic studies demonstrated a noticeably impaired bonemarrow-dependent (B cell) system and intact thymus-dependent (T cell) system. No evidence of humoral or cellular hypersensitivity to homologous lung determinants was found.
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3/39. Identification of monoclonal insulin autoantibodies in insulin autoimmune syndrome associated with HLA-DRB1*0401.

    We have characterized HLA and insulin autoantibodies in a Japanese female patient with insulin autoimmune syndrome. Serological HLA typing demonstrated the patient had HLA-DR4, and dna typing showed she had HLA-DRB1*0401 which has not been reported in patients with insulin autoimmune syndrome in japan. A single binding affinity of insulin autoantibodies was demonstrated by Scatchard analysis and immunoglobulin class of insulin autoantibodies was exclusively IgG-kappa. HLA-DRB1*0406 is strikingly associated with patients with insulin autoimmune syndrome who have polyclonal insulin autoantibodies. The present report demonstrated the first Japanese patient with insulin autoimmune syndrome carrying HLA-DRB1*0401 who was revealed to have monoclonal insulin autoantibodies. The present results indicate that HLA molecules are the major determinants of polyclonal insulin autoantibodies and monoclonal insulin autoantibodies in insulin autoimmune syndrome.
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4/39. Unmasking of acquired autoimmune C1-inhibitor deficiency by an angiotensin-converting enzyme inhibitor.

    BACKGROUND: A majority of angioedema arise from unknown etiologies. angioedema may also arise from medications or deficiency of C1-esterase inhibitor (C1-INH); either of these may lead to recurrent, sometimes life-threatening attacks of subcutaneous or submucosal edema if the angioedema involves the tongue, throat, or larynx. We describe a patient with unknown acquired C1-INH deficiency, who experienced only mild attacks of angioedema before treatment with an angiotensin-converting enzyme (ACE) inhibitor. This therapy led to life-threatening respiratory distress. OBJECTIVE: To investigate this patient's life-threatening angioedema. methods: serum protein electrophoresis and immunofixation were performed. The titer of anti-C1-inhibitor autoantibody was determined by ELISA, and the specificity of the autoantibody demonstrated by using purified C1-INH to block binding in the ELISA. Finally, fractions from the immunoelectrophoresis gel were tested for C1-INH autoantibody by ELISA. RESULTS: complement activation was documented by reduced C1-INH, C1q, and C4, and the patient was found to have an autoantibody of IgG2 isotype specific for C1-INH. After discontinuation of the ACE inhibitor, he continued to have decreased C1-INH and positive C1-INH autoantibodies. CONCLUSIONS: This case describes a patient who had a history of mild facial and extremity swelling with abdominal symptoms before ACE inhibitor treatment; this medication resulted in life-threatening respiratory distress. The use of the ACE inhibitor may have unmasked this patient's acquired autoimmune C1-INH deficiency.
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5/39. A novel lymphoproliferative/autoimmune syndrome resembling murine lpr/gld disease.

    In mice, the two distinct autosomal recessive genes lpr and gld can induce a syndrome characterized by autoantibody formation and the progressive accumulation of an unusual CD4-CD8- T cell population in peripheral lymphoid tissue. This phenotype does not precisely mirror any human disease. In this report we describe two patients with a progressive lymphoproliferative disorder associated with autoimmunity. The peripheral blood and lymph nodes of these patients contained large numbers of an unusual CD4-CD8- T cell population. These CD4-CD8- T cells express surface markers characteristic of mature peripheral blood T cells (CD3, CD2, CD5), express the alpha/beta form of the T cell receptor, and do not express surface markers characteristic of immature thymocytes (CD1) or NK cells (CD16, CD56). Functionally, these cells exhibited deficient proliferation and lymphokine production upon stimulation with mitogenic antibodies to CD3 or CD2. Both proliferation and lymphokine production could be augmented by co-stimulation with an antibody directed at the CD28 determinant. The clinical and immunological features of this syndrome resemble the lymphoproliferative/autoimmune disease seen in lpr and gld mice.
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6/39. transferrin-immune complex disease.

    A 71-year-old woman showed a highly unusual pattern of iron distribution in the organism which was associated with iron overload. The hallmark of this disease was an extreme hypersiderinemia, the serum iron reaching about 800 mug/100 ml. There was a pigment cirrhosis of the liver, bronzed skin containing hemosiderin, and diabetes mellitus. Paradoxically, hemosiderin was not detectable in bone marrow macrophages, sideroblasts and erythrocytes were reduced, and there was a decrease in radioiron utilization of erythropoiesis, thus indicating insufficient iron supply. The pathogenesis of this disorder based on the formation of an autoantibody with specificity for transferrin thus producing a circulating immune complex which bound the majority of serum iron. immunosuppression achieved a partial remission including a recovery of the patient's general state, a rise in free transferrin, a decrease in serum iron, disappearance of hemosiderin in the liver, and a rise in erythrocyte production.
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7/39. Development of alloanti-Jka in a patient with hemolytic anemia due to autoanti-Jkb.

    Autoanti-Jkb and a transient autoanti-E were identified in a patient with autoimmune hemolytic anemia, and red blood cells negative for Jkb and E antigens were transfused. Twelve weeks after transfusion, the autoantibody appeared to have developed broad specificity. However, autoadsorption studies revealed that the broad reactivity was due to the development of alloanti-Jka in addition to the autoanti-Jkb. Distinguishing this combination of alloanti-Jka plus autoanti-Jkb from an autoantibody with broad specificity will be important in selecting Jka antigen-negative red cells for subsequent transfusions. This case emphasizes the importance of additional serologic testing to detect alloantibodies in patients with broadly reactive warm autoantibodies.
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8/39. Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype.

    BACKGROUND: autoantibodies in linear immunoglobulin a (IgA) disease (LAD) are reported to be of IgA class and directed against a 97-120 kDa epidermal antigen. methods: We report a 39-year-old woman with clinical features of LAD and with circulating IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180). RESULTS: Histopathology of lesional skin revealed a subepidermal blister with mixed inflammatory cell infiltrate. Direct immunofluorescence of perilesional skin showed linear deposits of IgA along the dermal-epidermal junction. The antigen specificity of the patient's circulating antibodies was determined by Western blotting and enzyme-linked immunoabsorbent assay (ELISA) using various antigen sources, including cultured human keratinocytes, dermal protein lysates, and purified laminin-5, as well as proteins corresponding to BP180, the 230 kDa bullous pemphigoid antigen (BP230), laminin-5 subunits, and collagen IV alpha1-alpha6 chains. IgA and IgG antibodies in the patient's serum were directed against BP180, and no IgA or IgG reactivity was found against the other skin antigens. CONCLUSIONS: These data provide evidence for the presence of a subtype of LAD with dual IgA and IgG autoimmune response to BP180.
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9/39. A case of 'auto-immune thrombocytopenic purpura' serologically similar to post-transfusion purpura.

    The report describes a HPA-1a (Zwa)-negative woman with thrombocytopenia and antibodies in serum and eluate from autologous platelets with an operational anti-HPA-1a specificity. The results of the serological investigations were similar to the findings in most patients suffering from post-transfusion purpura. However, the present patient had no history of blood transfusion prior to the unset of purpura and the thrombocytopenia had persisted 6 months before splenectomy.
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10/39. Acquired Glanzmann's thrombasthenia without thrombocytopenia: a severe acquired autoimmune bleeding disorder.

    Acquired Glanzmann's thrombasthenia (GT) is an uncommon accompaniment to immune thrombocytopenic purpura. Rarely, GT may present as an acquired autoimmune disorder of platelet function, with rapid onset of a moderate-to-severe bleeding tendency, a prolonged bleeding time, but with a normal platelet count and normal platelet glycoprotein (GP) expression. This is caused by an autoantibody with specificity for platelet GP IIb/IIIa or an epitope close to that of the GP, resulting in partial or complete refractoriness of the patient's platelets to ADP, collagen and arachidonic acid. We describe two patients with acquired GT and a normal platelet count, who presented with severe bleeding. The first patient responded gradually to immunosuppressive treatment but eventually developed non-Hodgkin's lymphoma. The second patient had no other underlying conditions and remitted spontaneously within 2 years.
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