1/2. Eosinophilic fasciitis associated with autoimmune phenomena after bone marrow transplantation: report of two cases.Between January 1992 and May 2004, 189 patients underwent allogeneic bone marrow transplantation (BMT) for haematological malignancies from HLA-identical sibling donors in our transplantation unit. Of the 189 patients, 2 developed eosinophilic fasciitis (EF). The first patient developed Hashimoto's thyroiditis and EF 11 and 21 months after BMT, respectively. In the second patient EF occurred 9 months after BMT, accompanied by antinuclear antibodies, antiextractable nuclear antigens and antigliadin antibodies. Both patients were treated with extracorporeal photochemotherapy (ECP), resulting in improvement of fasciitis in both and normalization of antithyroid antibodies in the first patient. Our data confirm the rarity of fasciitis after BMT and the efficacy of ECP, recently applied experimentally in one patient for the treatment of fasciitis after BMT. Moreover, we report for the first time the association of fasciitis with autoimmune phenomena after BMT. The correlation between the two entities is supported by remission of Hashimoto's thyroiditis after ECP treatment for fasciitis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/2. Clinical and diagnostic aspects of encephalopathy associated with autoimmune thyroid disease (or Hashimoto's encephalopathy).Encephalopathy associated with autoimmune thyroid disease, currently known as Hashimoto's encephalopathy, but also defined as corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis, is a relatively rare condition observed in a small percentage of patients presenting with autoimmune thyroid disease. It consists of a subacute, relapsing-remitting, steroid-responsive encephalopathy characterised by protean neurologic and neuropsychiatric symptoms, diffuse electroencephalographic abnormalities and increased titres of antithyroid antibodies in serum and/or in cerebrospinal fluid. Most of the cases presenting this neurologic complication are affected by Hashimoto's thyroiditis or, less frequently, by other autoimmune thyroid diseases, chiefly Graves' disease. The pathogenesis of this encephalopathy is still unknown and largely debated, because of extremely varied clinical presentation, possibly referable to different aetiologic and pathophysiologic mechanisms, as confirmed by the two clinical cases we report in this paper. Autoimmune aetiology is, however, very likely in view of the well established favourable response to corticosteroid administration. Both vasculitis and autoimmunity directed against common brain-thyroid antigens represent the most probable aetiologic pathways. Clinical manifestations include consciousness changes, neurologic diffuse or focal signs, headache, and altered cognitive function. Although unspecific, cerebral oedema has also been described. cerebrospinal fluid examination often discloses an inflammatory process, with a mild increase in protein content and occasionally in lymphocyte count. In this review, clinical criteria for the diagnosis of defined, probable, or possible encephalopathy associated with autoimmune thyroid disease are suggested. Corticosteroid therapy currently allows us to obtain rapid remission of disease symptoms, but adverse outcomes as well as spontaneous remissions have also been reported.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |