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.

Retroperitoneal fibrosis: case series of five patients and review of the literature / Fibrose retroperitoneal: série de cinco casos e revisão da literatura

ABSTRACT Chronic periaortitis (CP) is an umbrella term used to describe a group of nosologically allied conditions that include idiopathic retroperitoneal fibrosis (Ormond's disease), inflammatory abdominal aortic aneurysm, and perianeurysmal retroperitoneal fibrosis. Retroperitoneal fibrosis encompasses a range of diseases characterized by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighboring structures-ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Here we describe a 5 years follow up (2006-2011) of 5 patients admitted to our hospital with symptoms, laboratory, imaging and pathologic finding compatible with retroperitoneal fibrosis. We review our clinical course of our patient with respect to the literature.

RESUMO Periaortite crônica (PC) é um termo genérico usado para descrever um grupo de condições nosologicamente ligadas que incluem a fibrose idiopática retroperitoneal (doença de Ormond), o aneurisma da aorta abdominal inflamatório e a fibrose retroperitoneal perianeurismática. O termo fibrose retroperitoneal engloba uma gama de doenças que se caracterizam pela presença de um tecido fibroinflamatório que geralmente envolve a aorta abdominal e as artérias ilíacas, se estende ao retroperitôneo e envolve estruturas ureterais vizinhas. A fibrose retroperitoneal geralmente é idiopática, mas pode também ser secundária ao uso de determinados fármacos, doenças malignas, infecções e cirurgia. Este estudo descreve o seguimento por cinco anos (2006-2011) de cinco pacientes internados em nosso hospital que apresentavam sintomas e achados laboratoriais, de imagem e patológicos compatíveis com a fibrose retroperitoneal. Revisou-se a evolução clínica dos pacientes, que foi comparada com os achados da literatura.

Multifocal idiopathic fibrosclerosis mimicking tuberculosis of the abdomen.

We present a 13 year old girl from Assam who had been treated as abdominal tuberculosis for 2 years due to the presence of refractory lymphocyte-predominant ascites and multiple small bowel strictures associated with significant anorexia and weight loss. On evaluation she was found to have retroperitoneal fibrosis with hydroureteronephrosis, mediastinal fibrosis and a retro-orbital pseudotumour. Based on these findings the diagnosis of Multifocal Idiopathic Fibrosclerosis (MIFS) was made. Ascites and multiple bowel strictures have been only rarely been described in association with MIFS. The other unique features in this patient were the early age of presentation, the presence of mediastinal fibrosis in association with retroperitoneal fibrosis, extensive soft tissue fibrosis of the neck, axillae and the presence of trismus. In a country like ours where Tuberculosis is commonplace, one would not think twice about treating such a case with antituberculous therapy. However, with a constellation of findings suggestive of a diffuse fibrotic process, MIFS should be an important consideration.©

Fibrose retroperitoneal: relato de caso / Retroperitoneal fibrosis: report of a case

Os autores relatam um caso de fibrose retroperitoneal diagnosticada por laparotomia exploradora a qual havia sido indicada por uma hipótese diagnóstica de aneurisma de aorta fissurado. Dada a raridade da doença, fazem o presente relato e revisam a literatura

A propósito de un caso de enfermedad de Ormond: fibrosis retroperitoneal idiopática / Apropos of a case of Ormond's disease: idiopathic retroperitoneal fibrosis

Este trabajo presente un caso de enfermedad de Ormond en un hombre de 56 años de edad. El cuadro se caracterizó por presentar un síndrome general, edemas en ambos miembros inferiores, insuficiencia renal y anemia. No presentó dolor abdominal o lumbar, ni hubo ingestión previa de metisergida, betabloqueantes u otras drogas. Tampoco había evidencias de enfermedad asociada alguna. Seis meses antes, el paciente no había presentado este síndrome y después de 37 días de postoperatorio, fue dado de alta hospitalaria. Por ecografía y tomografía computada se constató una masa retroperitoneal, cuya histopatología demostró que se trataba de una fibrosis retroperitoneal idiopática (IRF). La ureterolisis presentó dificuldades técnicas. El paciente comenzó a ser tratado con progesterona después de un mes de la operación. Seis meses después de su egreso, se efectuó una nueva TC que demuestra remisión total de su enfermedad hasta la fecha, encontrándose el paciente asintomático y con función renal normal

Fibrose retroperitoneal idiopática forma difusa: com oclusäo vascular periférica e fibrose pulmonar difusa (Síndrome de Ormond) / Diffuse idiopathic retroperitoneal fibrosis: with peripheral vascular occlusion and diffuse pulmonary fibrosis (Ormond's syndrome)

Relata-se um caso de doença de Ormond diagnosticado pela autópsia sem uropatia obstrutiva, cujo quadro inicial de gangrena de pododáctilo foi confundido com aterosclerose tendo concomitantemente RX pulmonar de padräo intersticial com fibrose difusa em vários territórios, supra e infradiafragmático

Progesterone as therapy for retroperitoneal fibrosis

Se presenta un caso de fibrosis retroperitoneal con obstrucción ureteral e infiltración vesical con remisión completa después del tratamiento con progesterona. La exploración abdominal y retroperitoneal, en una segunda laparatomía por un tumor inflamatorio apendicular, permitió constatar la ausencia de fibrosis. Cuatro años después de la última laparotomía, el paciente se encuentra asintomático y con estudios radiológicos y de laboratorio normales