Una inusual causa de estrechez de la vía aérea laringotraqueal / An unusual cause of laryngotracheal airway narrowing

La enfermedad relacionada con inmunoglobulina (Ig) G4 es una enfermedad de reciente conocimiento que puede comprometer cualquier órgano teniendo preferencias por ciertas regiones del cuerpo, donde la región de cabeza y cuello es uno de sus principales puntos afectados, pudiendo comprometer tanto la órbita, glándulas salivales, glándulas lagrimales, glándula tiroides, cavidades paranasales, hueso temporal, faringe y laringe. Este último órgano es infrecuentemente comprometido, solo existiendo 12 casos registrados en la literatura antes de la publicación de este escrito. Presentamos un caso de una mujer de 49 años con historia de disnea frente a esfuerzo, diagnosticándose una estenosis subglótica la cual fue manejada quirúrgicamente con una reconstrucción laringotraqueal. En el estudio histopatológico se evidenció histología compatible con enfermedad relacionada con IgG4, por lo que se inició tratamiento médico con corticotera- pia oral por un lapso de 2 meses en conjunto con inmunología. Paciente luego de 4 años de seguimiento, no ha presentado recaídas, manteniendo un lumen subglótico adecuado.

Immunoglobulin (Ig) G4-related disease is a medical condition of recent knowledge that can compromise any organ, having preferences for certain regions of the body, where the head and neck region is one of the main affected points, being able to affect orbit, salivary glands, lacrimal glands, thyroid gland, paranasal cavities, temporal bone, pharynx and larynx. The latter is infrequently compromised, with only 12 cases registered in the literature before the publication of this writing. We present a case of a 49-year-old woman with a history of exertional dyspnea, diagnosed with a sub- glottic stenosis which was managed surgically with laryngotracheal reconstruction. The histopathological study revealed histology compatible with IgG4-related disease, so medical treatment with oral corticosteroid therapy was started for a period of 2 months in conjunction with immunology. After 4 years of follow-up, the patient has not presented relapses, maintaining an adequate subglottic lumen.

Enfermedad por IgG4 multisistémica: a propósito de un caso: revisión de la literatura / Multisystemic IgG4 disease: a case report: literature review

We report a 63-year-old male patient who consulted for a 4-month history of xerophthalmia, xerostomia and cervicalgia, associated with jaundice, choluria, low weight and night sweats. Exams show an obstructive pattern and abdominal ultrasound describes a possible mass in the pancreatic head with secondary dilation of the bile duct. Colangio-MRI (magnetic resonance imaging) confirms a focal pancreatic head lesion that determines intrapancreatic bile duct stenosis, proximal dilation of the bile duct and stenosis of the main pancreatic duct. Pancreatic neoplasia versus autoimmune pancreatitis (PAI) is proposed, complementing a study with a count of IgG subclasses where IgG4 stands out at 1050 mg/dl (normal range: 3.9-86) and computed tomography (CT) of the thorax, abdomen and pelvis, that in addition to the biliary-pancreatic alterations, shows thickening of the vesicular wall, multiple bilateral lesions in the renal parenchyma and peri-aortic soft tissue. Salivary gland biopsy reports lymphoplasmacytic infiltrate characteristic of IgG4 disease. A disease related to IgG4 (ER-IgG4) is diagnosed with pancreatic, renal, biliary, vascular, lymphatic, salivary and lacrimal glands involvement. It is treated with corticoidal therapy, evolving favorably with resolution of the symptomatology and regression of imaging alterations.

Se reporta caso de paciente de sexo masculino de 63 años que consulta por cuadro de 4 meses de xeroftalmia, xerostomía y cervicalgia, asociado a ictericia, coluria, baja de peso y sudoración nocturna. En exámenes destaca pruebas hepáticas con un patrón obstructivo y ecografía abdominal que muestra una posible masa en la cabeza pancreática con dilatación secundaria de la vía biliar. Colangio-resonancia (resonancia magnética-RM) confirma una lesión focal en la cabeza pancreática que determina estenosis del colédoco intrapancreático, dilatación proximal de la vía biliar y estenosis del conducto pancreático principal. Se plantea neoplasia de páncreas versus pancreatitis autoinmune (PAI), complementando estudio con recuento de subclases de IgG donde destaca IgG4 de 1.050 mg/dl (rango normal: 3,9-86) y tomografía computada (TC) de tórax, abdomen y pelvis, que además de las alteraciones bilio-pancreáticas, demuestra engrosamiento de la pared vesicular, lesiones múltiples bilaterales en el parénquima renal y tejido de partes blandas peri-aórtico. Biopsia de las glándulas salivales informa sialoadenitis crónica e inespecífica. Se diagnostica una enfermedad relacionada a IgG4 (ER-IgG4) con compromiso pancreático, renal, biliar, vascular, linfático, de glándulas salivales y lagrimales. Se trata con terapia corticoidal, evolucionando favorablemente con resolución de la sintomatología y regresión de las alteraciones imagenológicas.

Gastropatía hipertrófica por IgG4: reporte del primer caso en Chile / IgG4 related hypertrophic gastropathy: report of one case

IgG4 related disease is a systemic autoimmune disease, which can affect different organs. The most common digestive manifestation is autoimmune pancreatitis (AIP), followed by involvement of bile ducts and the major papilla. The stomach is only rarely involved. We report a 71-year-old diabetic woman consulting for jaundice and weight loss. Abdominal CAT scan, cholangio resonance imaging, endosonography and a serum IgG4 of five times the normal value, lead to the diagnosis of an autoimmune pancreatitis. An upper gastrointestinal endoscopy showed a diffuse thickening of gastric folds. The pathological study confirmed the presence of IgG4 positive plasma cells. The patient was successfully treated with steroids.

Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune: enfermedades relacionadas con IgG4. Caso clínico / Hypophysitis and retroperitoneal fibrosis associated with autoimmune polyglandular syndrome and IgG4-related disease: report of one case

We report a 23 year old woman presenting with a nephrotic syndrome due to minimal change disease, central diabetes insipidus, primary hypothyroidism, vitiligo and universal alopecia. Eleven years later, she presented secondary amenorrhea due to hypogonadotropic hypogonadism, with mild hyperprolactinemia and central adrenal insufficiency. A magnetic resonance imaging of the sella turcica showed a pituitary mass with suprasellar extension that was resected using a transsphenoidal approach. Pathology confirmed the presence of a lymphoplasmacytic hypophysitis. She needed a second surgical resection due to mass growth and neuro-ophthalmologic impairment. One year later, systemic lupus erythematosus, arterial hypertension and type 2 diabetes mellitus were diagnosed. Two years later, due to back pain, constipation and renal failure, retroperitoneal fibrosis was found, satisfactorily treated with glucocorticoids and colchicine. Hence, this clinical vignette shows the coexistence of autoimmune polyglandular syndrome with retroperitoneal fibrosis and lymphoplasmacytic hypophysitis. Tissue analysis showed the presence of IgG4 producing plasma cells in the pituitary and retroperitoneum, which constitute a basis for the diagnosis of IgG4 related disease.