A case of ureteral stenosis due to ureteritis probably associated with rheumatoid arthritis

Abstract

Ureteritis associated with immunological disorder is rarely reported, and most cases in this category are small vessel vasculitis and immunoglobulin G4 (IgG4)-related disease. Rheumatoid arthritis (RA) associated ureteritis is uncommon and underlying etiology is unclear. We present a patient of ureteritis who had medical history of RA and successfully treated with steroid and immunosuppressant. A 49-year-old woman who had been treated for rheumatoid arthritis (RA) and atopic dermatitis (AD) suffered from gross hematuria for five successive days. Contrast enhanced-computed tomography (CE-CT) showed right dominant upper urinary tract dilatation with enhanced thickened wall. The hematuria continued accompanied with intermittent right back and lower abdominal pain, and following CT image taken after three months presented the progression to bilateral hydronephrosis. Ureteral stents were placed and antibiotic therapy was introduced for obstructive pyelonephritis. Ureterocystoscopy and following biopsy from the upper ureteral tract showed chronic inflammatory change in the histopathology, and we finally considered the stenosing ureteritis to be caused by immune-mediated mechanism related to RA. After starting steroid therapy with methotrexate, therapeutic response was obtained to remove the stents. In the cases of ureteritis or ureteral stenosis of unknown etiology with medical history of immunological disorders, we should consider the underlying immune-activated state and try to test CE-CT and histological examination before performing surgical procedure. After excluding the common causes of ureteritis or ureteral stenosis, these tests would support the appropriate diagnosis.

Clinical Images: Ureteritis in VEXAS syndrome

“…A ureteric stent was inserted with prompt symptom resolution; it was removed 3 weeks later. Ureteritis is generally caused by infection; however, there are case reports associated with IgA vasculitis, granulomatosis with polyangiitis, systemic lupus erythematosus, IgG4‐related disease, and rheumatoid arthritis 1 . VEXAS syndrome is a recently described autoinflammatory condition caused by a somatic mutation in the UBA1 gene 2 .…”

“…Ureteritis is generally caused by infection; however, there are case reports associated with IgA vasculitis, granulomatosis with polyangiitis, systemic lupus erythematosus, IgG4‐related disease, and rheumatoid arthritis. 1 VEXAS syndrome is a recently described autoinflammatory condition caused by a somatic mutation in the UBA1 gene. 2 Activation of innate immune inflammatory signalling in myeloid cells leads to a plethora of dramatic inflammatory manifestations such as chondritis, pulmonary infiltrates, neutrophilic dermatosis, and vasculitis.…”