1/34. Serial brain SPECT images in a case of Sydenham chorea.BACKGROUND: The pathophysiological nature of Sydenham chorea (SC) has been presumed to be an autoimmune-mediated inflammatory process. Positron emission tomography in SC has revealed a striatal hypermetabolism that might explain the transient neuronal dysfunction. However, any focal hyperperfusion in the striatum or its related structures has not been demonstrated in previous single photon emission computed tomographic (SPECT) imaging studies, which raised a concern about the pathogenesis of the striatal hypermetabolism. OBJECTIVE: To investigate the cerebral perfusion patterns of the subcortical structures by using serial technetium Tc 99m-ethyl cysteinate dimer SPECT in a case of SC, which may provide a clue for the pathophysiological mechanisms. DESIGN: A case report and serial SPECT studies. CASE PRESENTATION: A girl aged 4 years 3 months showed severe generalized choreic movements with concomitant signs of acute pharyngitis. Results of a laboratory study taken 7 days after the onset of chorea showed elevated antistreptolysin O titer, c-reactive protein levels, and erythrocyte sedimentation rate. Other laboratory data, throat culture, echocardiography, brain magnetic resonance imaging, and electroencephalography did not reveal any abnormalities. Five days after treatment with haloperidol and penicillin, the chorea began to improve slowly, and completely resolved in 2 months. RESULTS: Three serial SPECT images and semiquantitative analysis of cerebral perfusion were obtained. Cerebral perfusion in the striatum and thalamus was markedly increased bilaterally during the stage of active chorea and then returned nearly to its baseline level during the convalescent phase. These cerebral perfusion patterns were concordant with semiquantitative analysis. CONCLUSIONS: Hyperperfusion in both the striatum and thalamus in our patient may reflect the subcortical inflammatory processes in SC. The unequivocal SPECT findings in our patient are difficult to reconcile with the negative findings of previous SPECT studies but may suggest the heterogeneity of the perfusion patterns in SC.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/34. Therapeutic plasma exchange for the acute management of the catastrophic antiphospholipid syndrome: beta(2)-glycoprotein I antibodies as a marker of response to therapy.We describe two patients with the catastrophic antiphospholipid syndrome associated with elevation of beta(2)-glycoprotein I antibodies and fulminant thrombotic diatheses. Both patients were treated with therapeutic plasma exchange (TPE), which resulted in a marked decrease in antibody titer accompanied by an improved clinical outcome in one patient (IgG antibody). In the second patient, the outcome was poor despite TPE (IgA antibody). There were no significant complications of TPE in either case. Because of the fulminant nature of the catastrophic antiphospholipid syndrome, we conclude that a trial of TPE is warranted for the acute management. Further studies are needed to clarify which patients may benefit from this treatment.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/34. adoptive transfer of vitiligo after allogeneic bone marrow transplantation for non-Hodgkin's lymphoma.vitiligo developed in a 50-year-old man 9 months after allogeneic transplantation from his HLA-identical sister who had had this disease for several years. Our findings suggest adoptive transfer of vitiligo by haematopoietic stem cell transplantation, and lend support to the autoimmune nature of this disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/34. 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.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/34. Acquired factor viii autoantibody: four cases demonstrating the heterogenous nature of this condition and problems involved in diagnosis and treatment.The development of an autoantibody to human factor viii is rare and presents many problems for diagnosis and treatment. We have seen several cases at our institution recently with widely heterogenous clinical and laboratory presentations. A wide range of treatment modalities were used in these cases with no gold standard of treatment or widely accepted guidelines existing. This has prompted us to examine all cases of this condition presenting at Fremantle Hospital over the last decade. We describe four cases which demonstrate the heterogeneity of this condition and its treatment and review the recent literature on the subject.- - - - - - - - - - ranking = 4keywords = nature (Clic here for more details about this article) |
6/34. Cogan's syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature.Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms. Typically, young adults suffer from interstitial keratitis and sudden onset of tinnitus and hearing loss. Few cases (around 150) have been published and thus it is difficult to determine the percentage of patients with underlying systemic disorders such as systemic vasculitis. The variety of systemic manifestations is large and includes fever, splenomegaly, lymphadenopath, and musculoskeletal complaints. systemic vasculitis can be seen in around 10% of cases and may involve the large vessels, appearing as Takayasu-like vasculitis with affection of the aortic valve but also the coronary arteries and the small kidney vasculature. Evaluating the exact extension of the systemic features determines the choice of treatment. While corticosteroids have proved to be of short-term benefit, long-term treatment with immunosuppressive drugs is controversial. Auditory function in deaf patients has often been restored successfully with cochlear implants. To illustrate the nature of the syndrome, we present two patients with a wide clinical spectrum of symptoms from local disease restricted to the eyes and ears to a widespread vasculitis affecting arteries of the brain, kidney and the upper and lower extremities. We then review the typical aspects as well as the etiology of the disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/34. Successful treatment of autoimmune chronic idiopathic urticaria with intravenous cyclophosphamide.BACKGROUND: A 45-year-old woman presented with a 20-year history of chronic idiopathic urticaria (CIU) unresponsive to H1- and H2-antagonists and combinations of other anti-inflammatory agents but controlled with daily prednisone (35 mg) for more than 13 years. Intracutaneous testing to autologous serum revealed an 8 x 10-mm wheal/flare reaction consistent with the presence of anti-Fc epsilonRIalpha autoantibodies. These autoantibodies have been reported to be present in >45% of patients with CIU. Their functional role in the pathogenesis of CIU remains poorly understood. OBJECTIVE: Because of the therapeutic refractory nature of this patient's CIU requiring high doses of corticosteroids, it was decided to initiate treatment with intravenous cyclophosphamide (CTX) in an attempt to eradicate autoantibody-producing B-lymphocyte clones. This therapeutic approach has been previously successful in other autoantibody-mediated disorders such as type II acquired angioedema and factor viii deficiency. RESULTS: Initial treatment consisted of 500 mg CTX intravenously followed by increases of 100 mg every 2 weeks, with the maximum dose reaching 1,500 mg once a month, which represents approximately 20% of the dose administered during systemic cancer chemotherapy. Within 7 months there was a complete clinical remission and prednisone was discontinued. Repeat intracutaneous testing to autologous serum was negative, consistent with an abrogated autoantibody response. The patient has not experienced a recurrence of CIU at the time of this report. CONCLUSION: This index case suggests that intravenous CTX may be effective in alleviating autoantibody-associated CIU in corticosteroid-dependent patients refractory to conventional therapies.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/34. Primary sclerosing cholangitis associated with autoimmune pancreatitis.We present 2 cases of primary sclerosing cholangitis complicated with autoimmune pancreatitis with narrowing of the pancreatic duct. In both cases, endoscopic retrograde cholangiography showed the characteristic findings of primary sclerosing cholangitis. In addition, positivity for autoantibody, hypergamma-globulinemia and narrowing of the pancreatic duct shown on endoscopic retrograde pancreatography were noticed in both. They were thu diagnosed as autoimmune pancreatitis. Whereas it is well known that primary sclerosing cholangitis is often complicated with chronic pancreatitis, it has rarely been studied whether the chronic pancreatitis is autoimmune pancreatitis or not. In English literature, possibly 7 equivalent cases have been reported so far. In those cases, the onset was in the post-prime of life, and the disease was frequently accompanied with sjogren's syndrome, but not with ulcerative colitis; frankly the nature of the disease was slightly different from what has traditionally been thought as a typical primary sclerosing cholangitis. In primary sclerosing cholangitis complicated with autoimmune pancreatitis, the autoimmune mechanism seems to be much involved, and thus steroid therapy might be effective as in Case 2 in our series.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/34. Autoimmune phenomena in patients with myelodysplastic syndromes and chronic myelomonocytic leukemia.Autoimmune paraneoplastic syndromes are commonly encountered in patients with myelodysplastic syndromes (MDS). A review of case reports and small series suggest as many as 10% of MDS patients may experience various autoimmune syndromes. Clinical manifestations of such phenomena may include an acute systemic vasculitic syndrome, skin vasculitis, fever, arthritis, pulmonary infiltrates, peripheral polyneuropathy, inflammatory bowel disease, glomerulonephritis, and even classical connective tissue disorders, such as relapsing polychondritis. On the other hand, asymptomatic immunologic abnormalities have also been reported in these patients. These autoimmune manifestations frequently respond to immunosuppressive agents including steroids and occasional hematologic responses to steroid therapy have also been reported. We report five patients with history of MDS who manifested different spectrums of autoimmune phenomena including: pyoderma gangrenosum (PG), vasculitis, Coombs negative hemolytic anemia, idiopathic thrombocytopenia, and chronic inflammatory demyelinating polyneuropathy (CIDP). We also review the incidence, nature, course and response to therapy of these manifestations and discuss potential pathogenic mechanisms.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/34. Autoimmune disease-associated lymphadenopathy from dermatomyositis. A case report.A case of autoimmune disease-associated lymphadenopathy (ADAL) in the lymph nodes linked to well documented dermatomyositis is presented. A 49-year-old Japanese woman was affected by a left axillary and a left inguinal lymphadenopathy at the onset of disease. A biopsy specimen taken from a left inguinal lymph node showed distortion of the nodal architecture and polymorphous lymphoid infiltration containing many plasma cells and plasmacytoid cells with scattered transformed lymphocytes, as well as a large number of capillaries. The polytypic nature of b-lymphocytes was demonstrated by immunohistochemistry and polymerase chain reaction. Epstein-Barr virus-associated lymphoplasmacytic infiltrates may occur in patients immunosuppressed by methotrexate administered for the treatment of dermatomyositis. However, Epstein-Barr virus-encoded small rna-positive cells were not identified by in situ hybridization. Moreover, the patient had no history of methotrexate therapy.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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