When dealing with unknown etiology for deep vein thrombosis, look for a systemic disease

Introduction: Inferior vena cava thrombosis is a rare presentation of deep vein thrombosis, representing diagnosis and therapeutic challenges. Whenever there are important abnormalities of the surrounding structures, especially involving the aorta, identifying a com-mon setting is essential for the appropriate management. The patient, a 50 years old male presented in the ER for hypogastric pain and significant edema in both legs, associating psoriasiform rash located on the elbows. The patients also had tachycardia, polypnea and hypotension. The laboratory findings showed D-Dimer tests over the lab limit, anemia, thrombocytopenia, renal dysfunction and elevated inflammation markers. The echocardiography showed no significant findings and the chest computed tomography scan excluded thromboembolism. Suspecting deep vein thrombosis, an ultrasound was performed and it didn’t identify any blood flow in the femoral common veins – in both legs.

Case presentation: The abdominal and pelvic CT scan described an important thickening of the aortic wall in the infrarenal segment, in close contact with the inferior vena cava, forming together an adherent block, without contrast flow in the vein, an aspect suggesting aortitis and probably secondary inferior vena cava thrombosis. This very rare description of the two big vessels lead us to the suspicion that we are dealing with a pathology that primarily affects the aorta and then, secondary to the local inflammation, a thrombosis occurred. Next step was excluding neoplastic and hematologic (like thrombophilia) etiologies responsible for the structural abnormalities of the aorta and vena cava. By putting together clinical elements (skin lesions, joint pain), paraclinical findings (aortitis, bicytopenia, elevated in-flammatory markers, renal dysfunction) we thought it might be a systemic disease, most likely systemic lupus erythematosus – a diagnosis that was eventually confir-med with the immunology tests in a specialized cen-ter. The challenge of this case was not only diagnosing the etiology of the vascular abnormalities, but also the short and long term therapeutic management as well as the complications of the main pathology.

Case particularity: The first therapeutic step was local catheter thrombolysis, using a jugular approach, a pro-cedure that had to be interrupted due to a large cervical hematoma, extended in the mediastinum. We continued with unfractionated heparin under aPTT control. The immediate clinical evolution was favorable, with the gradual remission of the symptoms (including the edema, tachycardia or hypotension). For the long term anticoagulation regimen we chose VKA, considering the clinical special setting, the inflammatory context causing the thrombotic event and the procoagulant status in lupus. We also decided for 6 months of initi-al treatment, followed by clinical and imagistic assess-ment in order to establish the need for its extension. The particularity of the case comes from a very unique manifestation of deep vein thrombosis, associated with lupus aortitis, with two possible underlying mecha-nisms: the inflammation of the adjacent vessel and the hypercoagulable state, challenging as well the short and long term therapeutic management.

ISSN
ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
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CNCSIS B+
CODE: 379
CME Credits: 10 (Romanian College of Physicians)
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