In June 22, 2002, the patient received a renal transplant from a 29-year-old woman unrelated live donor. Her initial immunosuppressive regimens were prednisolone, 7.5 mg, po, qd; cyclosporine 100 mg, po, bid; and Cellcept, 1 g, po, bid. She was also treated with co-trimoxazole 40/400 mg po, bid and nifedipine, 30 mg, po, qd, to treat post-transplantation hypertension. Her serum creatinine was always less than 1.3 mg/dL; the serum cyclosporine A level was in acceptable range for 12 years post-transplantation.
For seven years, she continued to suffer from recurrent sinusitis and frequent episodes of left knee swelling, otolaryngology consultation was done three times. Sinus computed tomography was ordered and blood work and urine culture for infection were done. Also cerebrospinal fluid (CSF) and synovial fluid analysis, VDRL, and PPD were done, all being negative. There was an excellent improvement with steroid therapy. However, her ANCA remained negative.
On February 10, 2009, with no changes in immunosuppressive regimen, she presented with a high grade fever, cloudy nasal discharge, neck rigidity, severe headache, diplopia, and bilateral papillary edema. Lumbar puncture was performed revealing high CSF pressure (>40 cm H2O). CSF analysis revealed high protein, 400 RBC, 50 WBC with 20% PMN and 80% lymphocytic predominance. Brain MRI showed a lesion suggestive of lymphocytic hypophysitis (LYH). Accordingly, she was treated successfully with high dose steroids.
In March 2009, the patient was admitted with severe headache. While taking her immunosuppressive, as usual and regularly, her C-ANCA became positive for the first time accompanied by proteinuria of <900 mg/24 hrs. Neurology consultation reported "bilateral papillary edema and high CSF pressure (>40 cm H2O)," Cerebral pseudo-tumor was therefore suggested and frequent lumbar puncture was recommended. Laboratory data including HBS Ag, HBC Ab, HIV Ab, CMV Ab (IgG and IgM), VDRL, and AFB for Mycobactrium tuberculosis, C3, C4, CH50, Wright and Coombs Wright reported within normal limits. In all CSF analyses, WBC was high with lymphocyte predominance, high protein and normal to low glucose. The patient developed cavernous sinus thrombosis, which was treated successfully using heparin followed by warfarin. Headache was subsided with increasing the dose of corticosteroid. In follow up, the patient was doing well and stable until September 2014 when her serum creatinine was 1.3 mg/dL and urinalysis was normal.
In November 2014, while on cyclosporine A, according to blood level and Cellcept 2 g/day plus 7.5 mg prednisolone, the patient developed acute renal failure:an increase in serum creatinine from 1.3 to 2.01 mg/dL:with the therapeutic range of cyclosporine A trough level. In work up for the cause of acute kidney injury in transplanted kidney, color Doppler ultrasonography reported positive for a significant renal artery stenosis of the transplanted kidney at the anastomosis site of the donated renal transplant artery to the right internal iliac artery of the recipient (Fig 1). At this time, there was no evidence of hydronephrosis of the transplanted kidney in ultrasonography.
A stent was inserted via angioplasty procedure, to bypass significant proximal renal artery stenosis in the transplanted kidney on February 18, 2015. Following insertion of the stent in the stenotic site, renal function improved. In June 2015, serum creatinine increased again. Renal ultrasonography revealed a moderate to severe hydronephrosis due to uretero-pelvic junction obstruction (UPJO) in the transplanted kidney. Considering positive C-ANCA with simultaneous inflammation of the right knee and ankle joint, diagnosis of recurrent vasculitis in ureteo-pelvic junction of the transplanted kidney was established. Following percutaneous nephrostomy and antegrade pyelography (Fig 2), initiation of corticosteroid therapy as well as 500 mg prednisolone daily, and rituximab every week for four consecutive weeks, complete resolution was achieved. On July 4, 2015, nephrostomography of the transplanted kidney revealed resolution of the uretero-pelvic stenosis in the transplanted kidney (Fig 3) and no more hydronephrosis, thus, nephrostomy catheter was removed. In March, renal ultrasound revealed grade 1-2 hydronephrosis in pyelocaliceal system and hydroureter. Simultaneous laboratory work showed a WBC count of 1500, hemoglobin of 11.9 g/dL, BUN of 25 mg/dL, creatinine of 1.6 mg/dL, and serum potassium of 4.7 mEq/L.