Case presentation
A 39-year-old female patient with a history of SLE since 18 years ago and lupus nephritis since six years ago which treated with Mycophenolic Acid 2 grams daily, Hydroxychloroquine 400 mg daily, and low doses of Prednisolone. Also, Mycophenolic Acid has discontinued for him five months ago due to the reduction of proteinuria and the control of the disease.
The patient came to the clinic complaining of fever, shortness of breath, and malar rash for a few days. His laboratory test showed 3100 mg per 24 hours proteinuria, and with diagnosed with SLE flare-up and evaluated for LAD admitted to the Rheumatology ward.
During the physical examination in the ward, blood pressure: 120/80, respiratory rate: 20, body temperature: 38.7, Pulls rate: 89, malar rash, 2+ pitting edema, and crackles in the middle and lower right lungs were detected. Also, numerous LADs were found in the bilateral axillary and cervical area, while other physical examinations were normal. Table 1 illustrates the laboratory tests.
Due to the fever and shortness of breath, a chest CT scan and Covid PCR were performed for the patient, and the CT scan findings confirmed pneumonia; also, there is no evidence of LAD in the mediastinum, and the PCR test result was negative. So, broad-spectrum antibiotics were started for the patient for two weeks, and the patient’s respiratory symptoms improved. Furthermore, according to the diagnosis of SLE flare-up, Prednisolone 1mg/kg was started for the patient; hence, her edema, malar rash, and respiratory symptoms improved over time. Also, an ultrasound was performed based on LAD, which was detected during the physical examination. Multiple LADs were found in the cervical and both sides of Axillary areas; the largest was reported in the left axillary area with a size of 43mm x 19mm. Due to the suspicion of lymphoma, a CT scan of the abdomen and pelvis with oral and intravenous contrast was also performed for lymphadenopathy, but no lymphadenopathy was found.
For evaluation of the LADs, a biopsy was performed on one of the axillary lymph nodes. The pathology results and IHC (IHC results: CD20: positive in lymph nodules, Pax 5: positive in lymph nodules, BCL 6: positive in GCs, EMA: Negative, CD30: Negative, Ki 67: High in GCs, CD3: Positive in small T cells) confirmed the diagnosis of CD type Hylan vascular.(figure 1 )