Case presentation
A 43-year-old woman was admitted to our rheumatology outpatient clinic with a primary complaint of lower limb edema and dyspnea on exertion. Clinical findings in a hospitalized patient include: tachycardia (Heart rate [HR] 110) , tachypnea (respiratory rate [RR] 26 per min), blood pressure [BP] : 110/75, Body temperature: 38 , pale conjunctiva, decrease in the intensity of heart sounds ,decreased breath sounds at the base of the right lung and the left half of the chest, as well as nonpitting edema in the right lower extremity with normal and symmetric pulses .
The pericardial effusion and thickened pericardium were reported in the echocardiography. The CT scan revealed left-sided pneumothorax and right-sided pleural effusion. Additionally, collapse consolidation of the lower lobes was reported in both lungs.
Abdominal ultrasound and Doppler ultrasound of the right lower limb veins were reported as normal. Broad-spectrum antibiotics were initiated for the patient, and they underwent pericardiocentesis. Pericardial fluid drainage and pericardial biopsy were performed. The pericardial fluid analysis showed exudative fluid with negative cultures, and no malignant cells were observed in the cytology examination. The biopsy results were consistent with pericardial fibrinosis. The patient was discharged without a definitive diagnosis and was prescribed colchicine medication.
During follow-up, the patient did not show improvement and continued to experience palpitations and exertional dyspnea. Further investigations were conducted, including CT angiography and CT scan of the abdomen and pelvis, which were reported as normal. Cardiac magnetic resonance imaging (MRI) was also performed, revealing thickened pericardium along with signal changes in the ascending aorta. Considering the overall findings, there is a possibility of rheumatological problems, and the patient will undergo evaluation and examination by a rheumatologist.
Based on the further examinations, the patient has been diagnosed with weakness (3/5) in the right lower extremity. Considering the signal changes in the ascending aorta on the MRI, PET scan was performed. PET scan findings include: several intense hypermetabolic foci in pericardium, mild hypermetabolism in walls of ascending aorta. Moderately hypermetabolic gastric wall thickening in the pylorus/antrum regions. Diffuse intense hypermetabolism in muscles of neck, chest wall and trunk abdomen, pelvis and right tight (especially in vastus intermedius).
Based on the increased uptake in the muscles of the right thigh, further investigations were requested for the muscles of the right leg and thigh using MRI and electromyography (EMG)- NCS (Nerve Conduction Studies) for the patient. The findings indicated inflammatory myopathy, and the MRI results were reported as follows: diffuse muscular edema along right tight muscles. Based on these findings, further tests were requested for the patient to investigate the possibility of inflammatory myopathy. )LDH: 1083/230UL , Aldolase : 33.5/7.6 U/L )(table 1)
The patient underwent muscle biopsy, which revealed severe inflammatory myopathy with fascicular necrosis and prominent chronic fasciitis with MHC1 markedly upregulated. (Fig 1&2)
The patient was placed under treatment with IVIG (intravenous immunoglobulin) and MTX (methotrexate) for a duration of 3 months for the diagnosis of inflammatory myopathy. Subsequently, the patient underwent corticosteroid therapy as part of the treatment plan.
The patient had a favorable response to treatment, and significant clinical improvement was observed. However, due to severe hair loss, methotrexate was discontinued, and Mycophenolate mofetil was started but due to inadequate clinical response, patient was admitted to the hospital to receive rituximab medication.
During the hospitalization, the patient developed acute abdominal symptoms. Based on the imaging findings and examination, the diagnosis of bowel perforation was suggested in the context of vasculitis. The patient was scheduled for surgery, and a biopsy of the intestine was obtained, which was consistent with high grade diffuse large B-cell lymphoma.
The patient was referred to an oncologist for further management after receiving this diagnosis. They were placed under the care of an oncologist and started treatment with chemotherapy. The patient will continue to be monitored and treated by the oncologist.
In the latest follow-up reports, the patient’s condition remained stable.