Upper Arm Deep Vein Thrombosis in a Patient with Active Lupus

Abstract

Upper extremity deep vein thrombosis (UEDVT) is relatively rare, and much less as an initial presentation of systemic lupus erythematosus (SLE). Primary UEDVT should be considered in individuals with unilateral arm swelling where the brachial, axillary, and subclavian veins are frequently involved. SLE is a chronic autoimmune disease that predominantly affects women of childbearing age and of African descent. Patients present with clinical features ranging from arthritis and arthralgias (over 90% of patients with SLE) to life-threatening hematologic, or central nervous system involvement. Individuals have an increased risk of arterial and/or venous thrombosis where the most important risk factor is the presence of antiphospholipid antibodies. Even within this condition, thrombotic events are typically seen in the legs, and UEDVT remains an unusual presentation. Here, we present a case of a 36-year-old female of African descent with a recent medical history of small joint arthralgia and vaginal bleeding due to uterine fibroids, for which she was prescribed a short course of prednisolone and norethisterone, respectively. She presented with a 2-week history of unilateral swelling in the left arm. Doppler ultrasound and later computed tomography scan with contrast indicated left UEDVT. Further investigations throughout her admission led to the diagnosis of SLE, while antiphospholipid syndrome - a common contributor to thrombosis in SLE - was notably ruled out. The patient was initiated on anticoagulants. The patient went on to later rapidly develop lupus nephritis and started on high-dose prednisolone. Given the high risk of bleeding, the decision to postpone the kidney biopsy was taken. There is limited data available about UEDVT when compared to lower extremity DVT and even fewer studies on SLE patients with thrombosis in the absence of antiphospholipid syndrome. Keeping this in mind, clinicians need to recognize idiopathic UEDVT as a potential early sign of SLE and maintain a high level of suspicion.

Learning points: To highlight the possibility of idiopathic upper extremity deep vein thrombosis (UEDVT) in spontaneous unilateral arm swelling.Idiopathic UEDVT might indicate a serious underlying autoimmune condition as SLE in this case (in the absence of antiphospholipid syndrome), that requires intensive thorough investigation by a multidisciplinary team.Initial treatment of both UEDVT and lupus nephritis with standard dose anticoagulants and steroids might be the proper initial management, whereas kidney biopsy might not be necessary as the risk of bleeding is high while on anticoagulants.

Keywords: Paget-Schroetter syndrome; Upper extremity deep venous thrombosis; antiphospholipid syndrome; lupus nephritis; systemic lupus erythematosus.

Figure 1

Coronal CT scan shows left axillary vein and left subclavian vein thrombosis (blue arrow) and pericardial effusion (red arrow).

Figure 2

Axial CT scan shows pericardial effusion (red arrow).

Figure 3

Axial CT scan shows a left lower lobe pulmonary artery embolus (red arrow).

Figure 4

Echocardiography image showing pericardial effusion of 11.4 mm.

Figure 5

Follow-up coronal CT scan after 4 months of treatment, shows resorbed thrombus (blue arrow) and pericardial effusion (red arrow).