How Common Is Retroperitoneal Fibrosis in Patients with Sjogren Syndrome?

Introduction: Cases of retroperitoneal fibrosis resulting in end organ damage like hydronephrosis are rare. The common denominator in all these fibrotic manifestations in autoimmune diseases appears to be the activation of an epithelial to mesenchymal transition (EMT) process following chronic inflammatory stimulation. Case Description: A 54 year old female is known to have Hypertension, G6PD defecincy, Sjogren syndrome with ANA positive, sicca symptom and multiple miscarriages during 2nd trimesters, appendectomy and history of hernia repair. She had a significant history of multiple obstructive acute kidney injury episodes due to bilateral hydronephrosis and treated with double J stents with dramatic imporvement in her renal function. A diagnosis of secondary retroperitoneal fibrosis was done but no biopsy was obtained. and her IgG4 was negative. Her ENA results are showed in the attached photo. Discussion: Because symptoms of IRF are nonspecific, there is often a delay in diagnosis resulting in end-organ disease such as ureteral obstruction or hydronephrosis. Although IRF is uncommon, it should be considered in patients presenting with abdominal or flank pain, especially in patients with concomitant autoimmune disorders. Early recognition of disease can prevent end-organ damage and, as more cases are diagnosed, its relationship to SS may be elucidated leading to further advances in treatment and surveillance.