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.