A 39 year old female presented with rapidly enlarging goitre, minimal obstructive symptoms and no constitutional symptoms. Clinical examination revealed diffusely enlarged, firm, non-tender thyroid gland. Biochemical investigations showed subclinical hypothyroidism, positive thyroid antibodies and unremarkable inflammatory markers. Ultrasound examination and CT scan of the neck were suspicious ...

Idiopathic retroperitoneal fibrosis (RPF) is a rare disease that involves non-specific inflammation and fibrosis surrounding the aorta. As a result, RPF is a challenging diagnosis to make; patients often seek medical attention after complications arise, such as nephrotic syndrome. The patient in our case report initially complained of flank pain. Laboratory evaluation revealed acute renal failu...

Ormond's disease (OD) is an uncommon process with an annual incidence nearing 1 per million inhabitants. The etiology in most of the cases is unknown and several pathogenic mechanisms are implicated in secondary OD. Ormond disease is characterized by a fibrotic and inflammatory mass with three different clinical features: i) retroperitoneal fibrosis, ii) perianeurysmatic retroperitoneal fibrosi...

A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to eluc...

Four patients with IgG4-related disease (IgG4-RD) showed increased percentages of RP105-negative B cells in the peripheral blood. Case 1: A 66-year-old man having retroperitoneal fibrosis had 18.8% of RP105-negative B cells. Oral prednisolone improved the affected lesions and the percentage of RP105-negative B cells decreased (3.2%) after the treatment. Case 2: A 53-year-old man with retroperit...

We report a case of IgG4-related disease presenting both tubulointerstitial nephritis and retroperitoneal fibrosis causing acute renal failure in a 63-year-old male. He was admitted to our hospital because of acute renal failure requiring emergent hemodialysis. Computed tomography showed a soft-tissue density mass with an irregular border in the retroperitoneum. The mass involved bilateral uret...

The salient pathological findings in 40 patients with idiopathic retroperitoneal fibrosis are summarized. The findings tend to confirm previous suggestions that this chronic inflammatory disease is potentially widespread, predominantly peri-aortic, associated with systemic disturbances, and of some fundamental interest. The name ;systemic idiopathic fibrosis' is suggested for the disease comple...