CASE PRESENTATION
The case was a 31-years old man, a known case of seronegative peripheral arthritis with involvement of knees, hand wrists, and metacarpophalangeal joints from five years ago that was treated with methotrexate and prednisolone. During the years of illness, the patient had no evidence of skin nodules, exertional dyspnea, sinusitis, or kidney insufficiency, but evidence of scleritis was found during the course of the disease that improved with a gradual increase in the dose of the drug. About 6 months before the last visit, the patient voluntarily stopped his medication regimen, and after that, about two months before, he developed bilateral enlargement of the parotid and submandibular glands. At the time of the visit, the swelling of the mentioned areas was evident in the form of a thundering mass without erythema on the surface of the glands. On examination, the left mandibular margin was not well palpable. The patient did not complain of dry mouth, but he complained of dry eyes without any evidence of visual impairment, pain, or eye inflammation. Also, there was no evidence of purulent or bloody discharge from the nose. Also, there were no signs of fever or shortness of breath. In initial laboratory assessment, cell count, serum biochemistry, thyroid test, and 24-hour urine analysis were found to be normal, however, raised inflammatory indices including C-reactive protein and erythrocyte sedimentation rate were evident. The purified protein derivative (PPD) skin test was also negative. In sonography assessment, a cystic mass with internal echogenic foci measuring 20 x 27 mm was seen in the distal part of the left submandibular gland. For this reason, the patient was a candidate for MRI with contrast indicating a cystic-like lesion with fluid-fluid level and peripheral enhancement in parotid and submandibular glands bilaterally suggestive of hematoma (Figure 1). Pulmonary nodules were also evident in the patient’s CT scan (Figure 2). There was also a severe enlargement of bilateral parotid and submandibular glands probably due to vasculitis. Based on the aforementioned findings, the patient underwent fine needle aspiration (FNA) under ultrasound guidance, which indicated the presence of degenerated squamoid cells, giant cells, and inflammatory cells with a priority of neutrophils in the submandibular gland, as well as the presence of a cyst containing fluid without evidence of malignancy in the parotid gland. The differential diagnosis proposed for the patients included Sjogren’s syndrome, sarcoidosis, IgG4-related disease, and Wegener’s granulomatosis (GPA). As additional evaluations, an ultrasound of the abdomen and pelvis was requested, the only positive finding of which was grade 1 fatty liver. CT scan of the sinuses was also normal. In the CT scan of the lung, a lung nodule with a diameter of 14 mm was seen in the medial apical segment of the lung, which was not seen in the CT scan of the patient four years ago. The echocardiography of the patient also showed a normal ventricular functional state with normal pulmonary artery pressure and mild MR. In cytological assessment, raised serum levels of immunoglobulin-G4 (IgG4) along with positivity for the C-ANCA marker without any positivity for other rheumatologic markers were revealed. Also, the viral markers of hepatitis and HIV were found to be negative. Due to the size of the submandibular gland containing necrosis, the presence of pulmonary nodules, and positive markers, the diagnosis of GPA was finally made for the patient. He was treated with methotrexate, prednisolone, and rituximab which led to a gradual reduction in the size of the glands and the improvement of the patient’s clinical symptoms within one month after the treatment (Figure 3).