Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos / IGG4-related disease. Report of 52 patients

BACKGROUND: IgG4-related disease (IgG4 RD) is an immune-mediated fibro-inflammatory disorder, with tissue infiltration of IgG4+ plasma cells. It causes pseudotumors, tumors, and a wide spectrum of clinical manifestations. AIM: To report the clinical, laboratory, histopathological and treatment characteristics of a group of Chilean patients with IgG4 RD. MATERIAL AND METHODS: Review of medical records of 52 patients aged 18 to 76 years with IgG4 RD seen at six medical centers. RESULTS: Elevated IgG4 serum levels (> 135 mg/dl) were found in 18 of 44 (41%) patients. There was histological confirmation of the disease in 46 patients. The most common sites of involvement were lungs, eyes and kidneys. Eighteen (35%) patients had only one organ involved, 34 (65%) patients had two organs and 13 (25%) patients had three or more organs. The involvement of two organs was significantly more common in men (p < 0.05). In patients with only one organ involvement, the most frequent location was orbital and meningeal. All patients with kidney or lung disease had multiorgan involvement. All patients received corticosteroid therapy, 67% synthetic immunosuppressants, and 16% rituximab. CONCLUSIONS: ER-IgG4 can affect any tissue. Multiorgan involvement was more common in this series, with preference for lungs, eyes and kidneys. An excellent response to steroids is characteristic of the disease, but with a high relapse rate that requires additional immunosuppression.

Evidencia clínica e histológica de regeneración de tejido blando a partir de una matriz colágena porcina. Reporte de Caso / Histological and clinical evidence of soft tissue regeneration from a porcine collagen matrix. Case Report

RESUMEN: La pérdida de un diente resulta en la pérdida de volumen de tejidos duros y blandos lo que dificulta lograr resultados estéticamente satisfactorios. Con el fin de disminuir la morbilidad que provoca un injerto autólogo en el sellado del alveolo se puede reemplazar por una matriz reabsorbible de colágeno. El presente reporte de caso evaluó clínica e histológicamente una matriz colágena de porcino, en la regeneración de tejido blando, durante la instalación de un implante inmediato a una extracción dentaria. A los 6 meses clínicamente se obtuvo un tejido con una apariencia estética final óptima e histológicamente se evidenció la formación de un tejido epitelial y conjuntivo compatible con la de una mucosa normal.

ABSTRACT: Tooth loss results in loss of hard and soft tissue volume, making it difficult to achieve aesthetically pleasing results. In order to decrease the morbidity caused by an autologous graft in the alveolus seal, it can be replaced by a resorbable matrix of collagen. The present case report evaluated clinically and histologically a porcine collagen matrix, in soft tissue regeneration, during the installation of an implant immediately after dental extraction. At 6 months, clinically, a tissue with an optimal final aesthetic appearance was obtained and histologically, the formation of an epithelial and connective tissue compatible with that of a normal mucosa was evidenced.

[IGG4-related disease. Report of 52 patients]. / Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos.

BACKGROUND: IgG4-related disease (IgG4 RD) is an immune-mediated fibro-inflammatory disorder, with tissue infiltration of IgG4+ plasma cells. It causes pseudotumors, tumors, and a wide spectrum of clinical manifestations. AIM: To report the clinical, laboratory, histopathological and treatment characteristics of a group of Chilean patients with IgG4 RD. MATERIAL AND METHODS: Review of medical records of 52 patients aged 18 to 76 years with IgG4 RD seen at six medical centers. RESULTS: Elevated IgG4 serum levels (> 135 mg/dl) were found in 18 of 44 (41%) patients. There was histological confirmation of the disease in 46 patients. The most common sites of involvement were lungs, eyes and kidneys. Eighteen (35%) patients had only one organ involved, 34 (65%) patients had two organs and 13 (25%) patients had three or more organs. The involvement of two organs was significantly more common in men (p < 0.05). In patients with only one organ involvement, the most frequent location was orbital and meningeal. All patients with kidney or lung disease had multiorgan involvement. All patients received corticosteroid therapy, 67% synthetic immunosuppressants, and 16% rituximab. CONCLUSIONS: ER-IgG4 can affect any tissue. Multiorgan involvement was more common in this series, with preference for lungs, eyes and kidneys. An excellent response to steroids is characteristic of the disease, but with a high relapse rate that requires additional immunosuppression.

Abordaje quirúrgico y protésico para resolución de defecto Clase IV C de Zucchelli en el maxilar anterior. Reporte de Caso / Surgical and prosthetic approach for resolution of Zucchelli Class IV C defect in the anterior maxilla. Case report

RESUMEN: Se describe el caso clínico de una paciente de 37 años, sexo femenino, sana, con sonrisa gingival, la cual presentaba problemas estéticos en relación, a un implante en la 1.1, instalado hace 7 años, el implante no presentaba una correcta posición tridimensional, correspondiendo a una Clase IVc de Zucchelli. El presente reporte clínico describe los pasos quirúrgicos y protésicos para resolver la estética del maxilar anterior pasando de un PES/WES inicial de 8 a un valor final de 16.

ABSTRACT: We describe the clinical case of a 37-year-old female patient, healthy, with a gingival smile, who had aesthetic problems in relation to an implant in 1.1, installed 7 years ago. The implant did not present a correct three-dimensional position, corresponding to a Class IVc according to Zucchelli. This clinical report describes the surgical and prosthetic steps to resolve the aesthetics of the anterior maxilla, going from an initial PES / WES of 8 to a final value of 16.

Manejo multidisciplinario de rinosinusitis bacteriana aguda posterior a elevación de seno maxilar: reporte de caso rehabilitado a tres años de seguimiento / Multidisciplinary management of acute bacterial rhinosinusitis after maxillary sinus lift: three years follow-up: case report

La cirugía de elevación de seno maxilar se considera como la técnica de aumento óseo más predecible. Sin embargo, no está exenta de complicaciones las cuales deben ser manejadas adecuadamente. Se presenta un caso clínico rehabilitado, con un seguimiento de 3 años, en el cual se realizó un manejo multidisciplinario y resolutivo de complicaciones asociadas a la técnica quirúrgica de elevación de membrana sinusal vía ventana lateral. Frente a una infección postoperatoria, como la presentada en el caso, hay que considerar iniciar un tratamiento antibiótico en el momento adecuado para impedir el agravamiento del cuadro clínico o un cambio de esquema en caso de resistencia antimicrobiana.

Maxillary sinus lift surgery is considered the most predictable bone augmentation technique. However, this procedure is not without complications, which must be handled properly. We present a rehabilitated clinical case, with a 3-year follow-up, in which a multidisciplinary and resolutive management of complications associated with the surgical technique of sinus lift procedure, using lateral window approach, was performed. In case of postoperative infection, such as the one presented in this report, it is necessary to consider starting an antibiotic treatment at the adequate moment to prevent the aggravation of the illness or change the pharmacological treatment in case of antimicrobial resistance.

Infección por citomegalovirus en pacientes reumatológicos: análisis de un caso y revisión de la literatura / Cytomegalovirus infection in rheumatologic patients: a case report and literature review

El Citomegalovirus es un microorganismo capaz de generar infecciones severas en pacientes inmunosuprimidos. Existe abundante información respecto a la infección en pacientes inmunosuprimidos por VIH o en relación a trasplante de órganos sólidos o hematopoyéticos. No ocurre lo mismo con los pacientes portadores de enfermedades autoinmunes. Si bien la clínica puede ser inespecífica y dificultar la sospecha diagnóstica, la clave está en determinar al paciente de riesgo para la infección y así realizar un diagnóstico precoz. Se presenta el caso de una mujer de 56 años, portadora de una polimiositis de difícil tratamiento, que en un contexto de terapia en base a corticoides e inmunosupresores (azatioprina y metotrexato), desarrolla cuadro febril asociado a fatiga, cuyo estudio concluyó una infección por Citomegalovirus, tratado exitosamente con Valganciclovir.

Cytomegalovirus is a microorganism associated with severe infections in immunosuppressed patients. There is abundant information regarding infection in HIV immunosuppressed patients or in relation to solid or hematopoietic organ transplantation. The same does not happen with patients with rheumatic diseases. Although the clinic can be nonspecific and hinder diagnostic suspicion, the key is to determine the patient at risk for the infection and thus make an early diagnosis. We present a case of a 56-year-old woman with a difficult-to-treat polymyositis, who, in a context of corticosteroid and immunosuppressive agents (azathioprine and methotrexate), develops a fever associated with fatigue, whose study con-cluded an infection due to Cytomegalovirus, successfully treated with Valganci-clovir.

Regeneración de tejido blando en una clase 3 mediante un colgajo desplazado lateral e injerto de tejido conjuntivo. Reporte de un caso clínico / Elian class 3 regeneration of soft tissue using a laterally moved flap and connective tissue graft. A clinical case report

RESUMEN: Se describe el caso clínico de una paciente de 60 años, sexo femenino, sana, la cual presentaba en el diente 1.1 una recesión de 6 mm de longitud con extensa pérdida ósea en vestibular correspondiente a una clase 3 de Elian. En el presente reporte se expone los pasos que se siguieron para llegar a una Clase 2 de Elian, mediante un colgajo desplazado lateral con injerto de tejido conjuntivo subepitelial.

ABSTRACT: We describe the clinical case of a 60-year-old female patient, healthy, who presented a 6mm-long recession on tooth 1.1 with extensive vestibular bone loss corresponding to an Elian class 3. In the present report, the steps followed to reach an Elian class 2,by means of a laterally moved flap with subepithelial connective tissue graft, are shown.

Atraumatic Extraction and immediate implant placement into infected site with the "ice cream cone" technique and L-PRF: A Case Report

ABSTRACT: We describe a case report of a 53 years old patient with osteopenia treatment, which presented a longitudinal root fracture in relation to 9 tooth Single Fixed Prothesis (SFP), an active fistula and a bucal plate loss. It was prescribed the tooth extraction and the immediate Biohorizons® Tapered Internal® implant installation. The regeneration of the bucal plate was performed using the technique of "ice cream cone" using Mineross®, Mem Lok® and L-PRF.

Necrosis de injerto de tejido conectivo subepitelial asociado a incompetencia labial: reporte de un caso clínico / Necrosis of a subepitelial connective tissue graft associated with lip incompetence: case report

Se describe el reporte del caso de una paciente sana con incompetencia labial portadora de un implante dental en la zona de 1.2, a la cual se le realizó un injerto de tejido conectivo utilizando la técnica del «sobre¼ para mejorar un leve defecto estético existente. El injerto sufrió una necrosis, posiblemente debido a la condición de deshidratación a la que están sometidos los tejidos en estos pacientes que presentan incompetencia labial y respiración bucal. Se describe la resolución quirúrgica del caso mediante una segunda cirugía de injerto de tejido conectivo, y se entrega el manejo clínico para evitar la aparición de esta complicación.

We describe a case report of a patient with lip incompetence and a healthy carrier of a dental implant in the tooth 1.2, which was performed using a connective tissue graft technique for improving an existing slight cosmetic defect. The graft necrosis was possibly due to the dehydration that the tissues are subjected to in these patients with mouth breathing and labial incompetence. The surgical resolution of the case by a second connective tissue graft, and its clinical management to prevent the occurrence of this complication is presented.

Clinical and genetic features of hereditary periodic fever syndromes in Hispanic patients: the Chilean experience.

Hereditary periodic fever syndromes (HPFS) are rare genetic diseases characterized by recurrent episodes of inflammation. Little information is available concerning HPFS in Latin American Hispanic population. The purpose of this study was to determine the clinical and genetic features of HPFS in Chilean population. A multicenter retrospective study of Hispanic Chilean patients with genetically confirmed HPFS was performed. We included 13 patients, 8 with familial Mediterranean fever (FMF) and 5 with TNF receptor-associated periodic syndrome (TRAPS), evaluated at rheumatology or pediatric rheumatology clinics between January 2007 and December 2010. Median age of symptoms onset was 8 years (range 1-35) and 8 years (range 0.3-21) for FMF and TRAPS, respectively. Median duration of fever was 3 days (range 2.5-15) for FMF and 21 days (range 9.5-30) for TRAPS. Genotyping of the MEFV gene in FMF patients revealed a homozygous M694V missense mutation in one patient, and heterozygous missense mutations in seven patients: M694V (n = 3), E148Q, R717H, A744S, and A511V. Sequencing of the TNFRSF1A gene in TRAPS patients revealed heterozygous missense mutations in four patients: T50M, C30R, R92Q, and IVS3+30:G→A, and a two-base pair deletion (IVS2-17_18del2bpCT) in one patient. Mutation in MEFV R717H and mutations in TNFRSF1A IVS2-17_18del2bpCT and IVS3+30:G→A are novel and have not been described previously. This study reports the largest series of genetically confirmed HPFS in Latin America, and adds evidence regarding the clinical and genetic characteristics of patients with FMF and TRAPS in Hispanic population. Mutations identified in MEFV and TNFRSF1A genes include defects reported in other ethnicities and novel mutations.