A 57-year-old female, not smoker, was admitted on March 29, 2014, with hemoptysis and history of recurrent sinusitis for one year. She had also positive history of recurrent eye inflammation epistaxis, dizziness, and left ear dry perforation. The right ear and audio assessment were normal. There was no arthritis. Other systemic reviews and physical examinations were unremarkable. Chest X-ray showed bilateral chest infiltrates. CT-scan revealed mucosal thickening of the floor of the left maxillary antrum, otherwise normal sinuses, and it also showed collapsed consolidation of the right lung with air bronchogram. The left lung showed ill-defined patches of ground glass and tree in bud appearance. Bronchoscopy showed mild pulmonary hemorrhage. Lung biopsy showed no granuloma but small vessels inflammation. Bronchoalveolar lavage (BAL) was negative for malignancy or any specific lesion.
Laboratory findings revealed high titers of c-ANCA 1 : 40 where it should be absent or of level less than 1 : 20, and p-ANCA was negative. Rheumatoid Factor (RF), ESR, and CRP were elevated of 225 IU/mL, 115 mmHr, and 92 mg/dL, respectively (Table 1). On the other hand, antinuclear antibody (ANA), Hepatitis B antigens, Hepatitis C antigens, and TB AFB and PCR were all negative and complements C3 and C4 were normal. Urine analysis identified hematuria and proteinuria. Serum albumin was low (2.5 g/dL). Renal function tests showed high level of creatinine (299 mg/dL).
She was diagnosed as ANCA-associated vasculitis, most likely granulomatosis polyangiitis, and her Birmingham Vasculitis Activity Score (BVAS) was 10 according to BVAS Version 3 form.
Patient was started on Pulse Steroid 1 gm IV for 3 days and then 60 mg orally daily.
On April 28, 2014, she was on Essential D-3 50,000 international units once weekly, Feroglobin capsules twice a day, and folic acid tablets 5 mg once weekly.
She was started on rituximab 1000 mg as induction of remission in May 2014. It was given after 30 minutes of its premedications methylprednisolone 100 mg, diphenhydramine 50 mg per month, and acetaminophen 975 mg per month. It was given each week for two weeks similar to Rheumatoid Arthritis (RA) induction regimen according to REFLEX trial.
Methotrexate was given as 12.5 mg orally once per week, but it was shortly tapered afterwards. Prednisone was tapered to 15 mg per month twice a day.
On March 4, 2015, the patient was given the maintenance dose of rituximab according to MAINRITSAN trial. Rituximab was administered at dose of 500 mg IV once weekly for 2 weeks with the premedications mentioned earlier. While infusing, she developed reaction to rituximab appearing as skin rashes and redness over cheeks only. The infusion stopped for 30 minutes and resumed once her vital signs were stabilized with slow rate infusion.
On April 8, 2015, she came complaining of pain and redness in the left eye, and similar symptoms of her initial presentation of the disease. She was diagnosed with anterior scleritis by ophthalmology service. Therefore, she was started on 30 mg of prednisolone. Symptoms were improved and the drug was tapered by 5 mg per week. She was also on calcium and vitamin D, but the plan for continuing rituximab as maintenance therapy was a question mark.
On May 27, 2015, she came complaining of ear pain, decreased hearing, tinnitus in the left side, and bilateral eye redness. That was attributed probably to the tapering of the prednisone as she at that time was on 10 mg once daily. Laboratory c-ANCA titers were positive (18.2). It was considered as rituximab maintenance failure. The plan was to increase the dose of prednisone to 30 mg/day and to add mycophenolate mofetil (MMF) 500 mg twice per day as an alternative maintenance therapy. The dose was increased to 1 gm twice per day to control symptoms. She was hypertensive as well; amlodipine 5 mg once daily was given.
On June 3, 2015, symptoms improved and ANCA titers decreased but hearing was still reduced. MMF dose was increased to 1 gm in the morning and 500 mg in the evening, and prednisone decreased to 25 mg/day. She was still hypertensive; amlodipine was increased to 10 mg once daily.
On July 1, 2015, there was no new complaint and symptoms improved, therefore tapering prednisone 2.5 mg per week according to symptoms.
On August 26, 2015, c-ANCA turned negative and the patient is doing well on the same regimen.
Upon her last followup on October 28, 2015, her prednisone was kept at 10 mg/day and mycophenolate at 1 gm twice a day with no complaint.