Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans cells histiocytosis characterized by multiorgan involvement, with renal-ECD documented in over one-third of patients. Renal disease is generally asymptomatic, rarely causing hydronephrosis and kidney impairment. In addition, the diverse clinical picture of Erdheim-Chester disease arises slowly with sequential manifestations. We present a rare case of a 75-year-old woman on long-term treatment for panhypopituitarism and steroid therapy for vasculitis, presenting to the emergency department with bilateral renal colic and acute kidney injury. Abdominopelvic CT scan revealed renal infiltration with signs of retroperitoneal fibrosis and hydronephrosis. Kidney CT-guided biopsy and 18-fluorodeoxyglucose (FDG) positron emission tomography whole body scan as well as the history of hypopituitarism and vasculitis confirmed the diagnosis of Erdheim-Chester disease. Proper therapy with interferon-α was started. This case describes the multifaced manifestation of this disease and the difficulty to establish the diagnosis, as well as the pivotal role that a urologist can play in its management.

Survivorship Issues in Adult Patients With Histiocytic Neoplasms

“…Emergent issues such as severe prerenal and postrenal obstruction in ECD may require invasive medical or surgical intervention. 1,45,[48][49][50] of obstructed ureters, the placement of nephrostomy tubes, or use of other urinary drainage systems may preserve renal function. Patients and caregivers require education about the care and risks of these drainage systems.…”

IgA Nephropathy in a Patient With Metastatic Carcinoma of Unknown Primary

“…Chemotherapy was then initiated, and the patient was still in treatment at the time of writing this report. Diffuse uptake of FDG from bilateral kidneys has been reported both in malignant and benign diseases, such as lymphoma, 3–5 Erdheim-Chester disease, 6 antineutrophil cytoplasmic antibody–associated vasculitis, 7 IgG4-related disease, 8 and genitourinary tuberculosis 9 . The imaging features of these disease demonstrated diffuse heterogeneous FDG accumulation, except for lymphoma.…”