Pemphigus vulgaris: a therapeutic option for disease control.

Pemphigus vulgaris (PV) is an autoimmune intra-epithelial dermatologic disease that is characterized by epithelial blistering and affected cutaneous and/or mucosal surfaces. The disease is caused by circulating immunoglobulin G (IgG) antibodies directed against desmosomes. These antibodies interfere with keratinocyte adhesion, causing acantholysis, which results in blister formation. At present, death from pemphigus infection is unusual; however, morbidity and mortality from chronic corticosteroid use remain considerable, indicating a need for additional therapies. This article presents a case of PV in a female patient that was treated with conventional corticosteroid therapy and discusses recent adjuvant therapy.

Autoimmune pancreatitis: an adolescent case and review of literature.

We present a case of a 16-year-old adolescent boy with autoimmune pancreatitis and a review of the related literature. The patient was sent from a peripheral medical center, presenting with jaundice, pruritus, weight loss, and hyperglycemia of 20 days' duration. At admission, the patient was icteric, choluric, and acholic. His abdomen was soft and nontender, and the patient felt no pain in his abdomen. He had skin lesions because of scratching. Laboratory findings showed a blood glucose level of 135 mg%; total serum bilirubin, 29.4 mg%; direct bilirubin, 23 mg%; and alkaline phosphatase, 1100 IU/L. Abdominal ultrasound showed an enlarged head of the pancreas that was 30 x 35 mm. The parenchyma was slightly heterogeneous. Abdominal computed tomography showed an enlarged head of the pancreas with a normal body and tail, thickened duodenal wall, and dilated intra- and extrahepatic biliary tract, and the distal choledochus was not visible. Magnetic resonance imaging showed dilated intra- and extrahepatic biliary tract. The choledochus was not visible, and the cystic duct ended abruptly. The pancreas head was enlarged and homogenous, and there were no changes with contrast. Wirsung's duct was not dilated. Laparotomy was performed with a presumed diagnosis of pancreatic head tumor. The pancreas was diffusely indurated and enlarged; biopsy and intraoperative cholangiography were performed. The biliary tract was dilated with no duodenal passage of contrast, and the Wirsung's duct was not observed. Cholecystectomy was performed, and a transcystic drain was positioned. The histopathology was compatible with autoimmune pancreatitis. Prednisone treatment was started with good response. Autoimmune pancreatitis is a very rare entity among children and adolescents. It should be suspected when characteristic clinical signs and radiographic images are associated with a higher level of IgG4. Autoimmune pancreatitis is confirmed by biopsy. Treatment with prednisone often alleviates all the symptoms, as what happened in this case.

[Hematopoietic stem cell transplantation in autoimmune diseases]. / Trasplante de células progenitoras hematopoyéticas en enfermedades autoinmunes.

Transplantation of hematopoietic stem cells, which are capable of self renewal and reconstitution of all types of blood cells, can be a treatment for numerous potential lethal diseases, including leukemias and lymphomas. It may now be applicable for the treatment of severe autoimmune diseases, such as therapy-resistant multiple sclerosis, lupus and systemic sclerosis. Studies in animal models show that the transfer of hematopoietic stem cells can reverse autoimmunity. The outcome of ongoing clinical trials, as well as of studies in patients and animal models, will help to determine the role that stem-cell transplantation can play in the treatment of autoimmune diseases.

Trasplante de células progenitoras hematopoyéticas en enfermedades autoinmunes / Hematopoietic stem cell transplantation in autoimmune diseases

El trasplante de células progenitoras hematopoyéticas, células con capacidad de autorrenovación y reconstitución de todos los tipos de células sanguíneas, se utiliza en el tratamiento de numerosas enfermedades potencialmente letales incluyendo leucemias y linfomas. Hoy en día es posible además aplicarlo en el tratamiento de enfermedades autoinmunes graves, como esclerosis múltiple, lupus eritematoso sistémico o esclerosis sistémica, resistentes a la terapia convencional. Estudios en modelos animales nos demuestran que la transferencia de células progenitoras hematopoyéticas podría revertir el proceso de autoinmunidad, un fenómeno que puede explicarse mediante diferentes mecanismos. El resultado de los estudios clínicos que se están llevando a cabo, así como también estudios en pacientes y modelos animales, ayudarán a determinar el rol que el transplante de células progenitoras hematopoyéticas puede jugar en el tratamiento de enfermedades autoinmunes.

Transplantation of hematopoietic stem cells, which are capable of self renewal and reconstitution of all types of blood cells, can be a treatment for numerous potential lethal diseases, including leukemias and lymphomas. It may now be applicable for the treatment of severe autoimmune diseases, such as therapy-resistant multiple sclerosis, lupus and systemic sclerosis. Studies in animal models show that the transfer of hematopoietic stem cells can reverse autoimmunity. The outcome of ongoing clinical trials, as well as of studies in patients and animal models, will help to determine the role that stem-cell transplantation can play in the treatment of autoimmune diseases.

[Autologous bone marrow transplantation as a treatment for autoimmune disease: mechanisms and results]. / Trasplante autólogo de médula ósea como tratamiento de enfermedades autoinmunes: mecanismos y resultados.

Autoimmune diseases are characterized by immune response against self antigens. One of the current research interests in this field is oriented toward development of tolerance. One of the newest options in the search for tolerance is autologous bone marrow transplantation: a variant of bone marrow transplant in which the patient's own hematopoietic stem cells are reinfused after myeloablative therapy. The idea of using bone marrow transplant in treatment of autoimmune diseases derived from observing remission in autoimmune diseases in patients transplanted due to coexisting neoplastic disease. Although an isolated initial report of bone marrow transplant as treatment for autoimmune disease questioned the utility of this procedure, over all, results are encouraging. To compile information in a programmed and systematic manner, it is necessary to send more patients in all stages of immune diseases to specialized centers to be included in large multicenter randomized trials. In time, the role for this procedure in autoimmune diseases will become clear.

Trasplante autólogo de médula ósea como tratamiento de enfermedades autoinmunes: mecanismos y resultados / Autologous Bone Marrow Transplantation as a Treatment for Autoimmune Disease: Mechanisms and Results

Las enfermedades autoinmunes se caracterizan por una respuesta del sistema inmune del individuo hacia tejidos propios. Una línea de investigación actual es el tratamiento de estas enfermedades y el desarrollo de tolerancia. Una de las opciones en la búsqueda del desarrollo de tolerancia es el trasplante autólogo de médula ósea: la variantes del trasplante de médula ósea que hace uso de células progenitoras hematopoyéticas propias. La posibilidad de usar este tipo de trasplante como tratamiento de enfermedades autoinmunes se originó en los hallazgos de remisiones de enfermedades autoinmunes coexistentes, en pacientes que eran trasplantados por enfermedades oncológicas. En esta revisión presentemos el fundamento teórico de este tratamiento, así como una recopilación de los estudios preclínicos y clínicos más relevantes en esta materia. Aunque algún reporte inicial puso en duda la utilidad de dicho procedimiento, en general, los resultados son alentadores. Es necesario que más pacientes en diversos estadios de las enfermedades autoinmunes sean referidos a centros especializados de manera que sea posible recopilar la información de manera ordenada y sistemática, y se pueda arribar a un conocimiento sobre el papel que juega este tipo de tratamiento en las enfermedades autoinmunes.

Autoimmune diseases are characterized by immune response against self antigens. One of the current research interests in this field is oriented toward development of tolerance. One of the newest options in the search for tolerance is autologous bone marrow transpiantation: a variant of bone marrow transplant in which the patient's own hematopoietic stem cells are reinfused after myeloablative therapy. The idea of using bone marrow transplant in treatment of autoimmune diseases derived from observing remission in autoinmune diseases in patients transplanted due to coexisting neoplastic disease. Although an isolated initial report of bone marrow transplant as treatment for autoimmune disease questioned the utility of this procedure, over all, results are encouraging. To compile information in a programmed and systematic manner, it is necessary to send more patients in all stages of immune diseases to specialized centers to be included in large multicenter randomized trials. In time, the role for this procedure in autoimmune diseases will become clear.