A 62-year-old woman presented with a 3-month history of vesical tenesmus without dysuria, vaginal bleeding, and low grade fever was referred to gynaecological and urological units. Her past medical history was significant for hypertension, osteoporosis, and B-thalassemia. She underwent hysterectomy and adnexectomy 12 years before due to fibroids. Clinical examination revealed abdominal pain and irregular hardening of anterior vagina encompassing urethra. No disorders involving respiratory tract, cardiovascular system, ear, or eyes were noted. A test of cervical smear and colposcopic evaluation were normal. Vital signs were normal, except temperature 37.5 C. Laboratory test revealed a hemoglobin level of 9,8 g/dL, 13430 leukocytes, and 630000 platelets. Urinalysis and intravenous urography were normal. Ultrasonography, abdomen computed tomography (CT), and pelvic magnetic resonance showed an irregular mass with crenulated margins occupying the urethra and vagina (Figures 1 and 2). Under the presumptive diagnosis of tumor, biopsy was carried with several specimens obtained. The biopsy showed a granulomatous inflammatory reaction, with small- and medium-sized vessels vasculitis and a hemorrhagic and necrotic background and without the characteristic features of caseating necrosis. The diagnosis of limited GPA was considered and patient was referred to Rheumatology Unit.
We proceeded to extend the study. Chest X-rays were normal and a skin test for tuberculosis was negative. Laboratory test revealed a sedimentation rate of 31 mm/h and C-reactive protein level of 20.7 mg/dL. The serum chemistry was unremarkable with creatinine of 1.0 mg/dL. Tumor markers were all in normal range. Anemia, thrombocytosis, and leukocytosis persisted. Anti-neutrophil cytoplasmic antibodies (c-ANCA) at a titer of 1/80 were noted. Rest of immunological studies was negative.
Prednisone (0,5/mg/kg/day) and oral Methotrexate (10 mg per week) were started with resolution of the mass and the symptoms. Sustained remission was achieved. Two years later, the patient was admitted to hospital for septic shock of urinary tract origin. Methotrexate was suspended and treatment with Prednisone (5 mg per day) was continued. The patient remained well; however two years later she began with a 2-week history of abdominal pain and vaginal bleeding. MRI showed mucosal edema at bladder neck and proximal urethra and persistence of vaginourethral fistula. Laboratory tests including ANCAs were normal. Prednisone dose was increased (0,5/mg/kg/day) and Azathioprine was started but had to be discontinued for hepatotoxicity. At this moment, five years after the onset of the symptoms, patient is in remission under low dose of Prednisone (2,5 mg per day), Methotrexate (7,5 mg per week), and Trimethoprim/Sulfamethoxazole three days per week for recurrent urinary tract infections prophylaxis.