What is Wegener’s granulomatosis with sinusitis as the first diagnosis?

A patient with Wegener’s granulomatosis (WG) was admitted to our department, and a review of his past medical history revealed that he had been diagnosed with “sinusitis” 3 months ago, but treatment was delayed due to lack of detailed investigation at that time, resulting in passive treatment. This is reported below to further understand the characteristics of this disorder.

Patient, female, 54 years old. She was admitted to the hospital on 05-11-17 with “swelling of both lower extremities, oliguria for 1 week, aggravated by shortness of breath for 3 days”. The patient had swelling of both lower extremities and decreased urine output with increased nocturia without any obvious cause for the past 1 week, but did not pay attention to it. In the last 3 days, the above symptoms worsened, and he developed epigastric distension, frequent nausea and vomiting, accompanied by chest tightness, palpitations, shortness of breath, and gradually developed into inability to lie down, so he came to our hospital. The chest X-ray suggested “acute respiratory distress syndrome” and was immediately treated with emergency hemodialysis and non-invasive ventilator-assisted ventilation. The patient was given sinus irrigation outside the hospital and underwent pathological examination of sinus secretions, which indicated “chronic inflammation of the left maxillary sinus with focal acute infection and tissue necrosis”, and the chest X-ray indicated “right lower pneumonia “At that time, it was considered as “sinusitis” and “right lower pneumonia”, and was discharged after anti-infection and other symptomatic treatment. We combined the above history with the patient’s previous sinus secretions, and then performed pathological examination, and found granuloma tissue, highly suspecting “Wegener’s granuloma”. The patient was immediately treated with methylprednisolone and cyclophosphamide shock therapy, as well as continuous hemofiltration, nutritional support and other symptomatic treatments. 12-6 The patient’s symptoms were significantly relieved, and the chest radiograph suggested improvement. However, the patient’s symptoms worsened again 1 week later and he died after resuscitation.

Discussion Systemic vasculitis, since it was first identified in 1982, with increased awareness of it, there is now a growing emphasis on the occurrence of this type of immune disease. It includes Wegener’s granulomatosis, microscopic polyangiitis, and others. This type of disease has a high rate of clinical underdiagnosis and misdiagnosis, and is not easily detected at an early stage. In 2001, the statistics of Shanghai Ruijin Hospital found that the underdiagnosis and misdiagnosis rate of these diseases in their hospital was over 66%. This kind of disease often manifests as various organs of the body damage, with lung and kidney more common, and less nasal involvement. Overseas studies have concluded that the incidence of ocular and nasal involvement is significantly higher in cANCA-positive patients than in pANCA-positive patients, and most cANCA-positive patients have Wegener’s granulomatosis, which is associated with nasal, ocular and upper respiratory tract lesions and needs to be taken seriously. If diagnosed early and treated early, the 5-year survival rate will be mentioned to more than 75%. In addition, the recurrence rate of Wegener’s granulomatosis is also statistically the highest among all vasculitis treatments, almost above 70%.