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).