Idiopathic versus Provoked Renal Infarction: Characteristics and Long-Term Follow-Up of a Cohort of Patients in a Tertiary Hospital.

BACKGROUND: There is limited evidence on the etiology and outcomes of renal infarction. A provoking factor is identified only in one- to two-thirds of patients. METHODS: This is a retrospective observational study. The clinical characteristics and outcomes of patients with acute renal infarction were studied; the sample was divided into two groups according to the presence of at least one provoking factor at the time of diagnosis (atrial fibrillation, flutter, major thrombophilia, or renal artery malformations). RESULTS: The study comprised 59 patients with a mean age of 63 (±16.7) years and a follow-up period of 3.1 (±2.8) years. An identifiable provoking factor was found for 59.3% of the renal infarctions at the time of diagnosis, and atrial fibrillation was the most frequent one (in 49.2% of all patients). Renal impairment was found in 49.2% of the patients at diagnosis and in 50.8% of the patients 6 months after the event (p = 0.525). When compared with the idiopathic group, the patients with provoked infarction were older (69.8 vs. 57.9 years, p = 0.014) and had a higher rate of recurrence of arterial thrombosis during follow-up (18.8 vs. 0%, p = 0.028), but there were no differences in the rest of the baseline characteristics or in mortality rates. Six patients (10.2%) in the idiopathic group were diagnosed with atrial fibrillation during follow-up. CONCLUSIONS: Atrial fibrillation, both at diagnosis and at follow-up, is the most common identifiable cause of renal infarction; however, a significant number of patients are idiopathic, and these are younger, but they have a similar burden of cardiovascular disease and a lower risk of arterial recurrence.

Retroperitoneal fibrosis associated with orbital pseudotumor without evidence of IgG4: A case report with review of literature.

Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic inflammation and periaortic fibrosis that affects retroperitoneal structures and often entraps the ureters. The idiopathic form has an incidence of 0.1-1.3/100,000 person-years. A substantial percentage of patients with idiopathic retroperitoneal fibrosis (IRF), as well as patients with orbital pseudotumor, is associated with IgG4-related disease (IgG4-RD). It is not clear what percentage of IRF is related to the spectrum of the IgG4-RD or if both represent different stages of the same disease (especially in those cases with extra-retroperitoneal involvement). Histopathological features such as storiform fibrosis, obliterative phlebitis and tissue infiltration of IgG4-positive plasma cells (ratio IgG4+/IgG higher than 0.4) are essential to identify this association. Extra-retroperitoneal manifestations are often presented among patients with IgG4-related RPF. About 90% of cases of IRF have a good prognosis, with adequate response to treatment. We report a case of a 59-year-old woman with history of past occupational asbestos exposure and smoking habit. She was diagnosed with RPF, periaortitis and orbital pseudotumor, without histopathologic or serologic features of IgG4- related disease. This could be related to the fact that the biopsy was done in a place with scarce inflammatory activity but high fibrosis. We want to emphasize the usual need to perform several biopsies or to be guided by positron emission tomography (PET-CT) in order to achieve a histopathological confirmation. Our case differs from the main IgG4 international cohorts in the involvement of the retroperitoneum, aorta and eye, whereas the usual involvement includes liver, pancreas, lymph nodes and salivary glands. Our patient had lower IgG4 serum levels than those described in the international cohorts. However, they were similar to those of the Spanish population.