ANCA-associated vasculitis (AAV) - is a group of rare diseases with a total incidence of 46-186 cases per million inhabitants. In all cases, diseases are characterized by inflammation and necrosis of small blood vessels and in many cases anti-neutrophil cytoplasmic antibodies (ANCA) are detected in the blood serum of the patient. The severity of the disease may range from local form to systemic and life-threatening condition and patient’s survival prediction mainly depends on timely and aggressive treatment.
We report a case of a 47-year-old woman, who was diagnosed with histologically confirmed ANCA negative granulomatosis with polyangiitis (GPA), and achieved clinical remission after being treated with rituximab. The disease manifested as upper respiratory tract inflammation. With suspicion of GPA, the patient was operated and the diagnosis of GPA was histologically confirmed. Chest CT scan revealed a granuloma-like nodule in the left lung, which is why the GPA was evaluated as a high-activity disease and patient was prescribed with Cyclophosphamide (100 mg) and methylprednisolone (32 mg). Even with the treatment, the patient began to complain about progressive dyspnea and coughing. High levels of inflammatory blood parameters and hepatic enzymes were detected. Due to insufficient treatment and persistent elevated liver enzymes, cyclophosphamide was discontinued. Patient was started to be treated with rituximab which reduced inflammation in the upper respiratory tract. Shortness of breath remained present due to scarring inthe bronchi and pulmonary atelectasis. Bronchodilation procedure led to a significant improvement in the patient’s condition and the patient was further treated with azathioprine (100 mg) and methylprednisolone (8 mg). In the absence of rituximab, the patient began to complain about frequent respiratory infections, coughing, polyneuropathy and general weakness. Continuation of rituximab reduced previously mentioned symptoms, and fibrobronchoscopy has shown no signs of respiratory tract obstruction.
Remission induction for high-activity AAV is usually initiated in combination with methylprednisolone and cyclophosphamide therapy, which is effective in many cases. Despite the fact that none clinical trials have shown rituximab’s superiority to cyclophosphamide, in cases of cyclophosphamide resistant disease treatment with rituximab can be effective.