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UP-PGH Division of 
Cardiovascular Medicine

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Case Vignette: 49/M with Takayasu Arteritis and Upper Extremity Phlegmasia Cerulea Dolens

By Bryan F. Elvambuena
History

A 49-year-old Filipino male presented with acute worsening of left arm pain, swelling, weakness, and violaceous discoloration. His history was notable for Takayasu arteritis diagnosed after a prior right subclavian artery pseudoaneurysm, with subsequent recurrent left upper-extremity ischemic symptoms that improved with but recurred after discontinuation of immunosuppressive therapy. Over the year preceding admission, progressive left arm ischemia culminated in severe pain, cyanosis, edema, and a reticular rash, prompting hospital evaluation.

Physical Examination

On examination, the patient had edema of the left face, chest, breast, and upper arm with prominent veins over the left neck and upper chest. Blood pressures were slightly higher in the lower extremities, while Doppler signals were triphasic in all major arteries with a normal ankle–brachial index of 1.29, indicating preserved arterial flow. He also had violaceous, lace-like rashes with dusky discoloration and fingertip cyanosis on the left arm, accompanied by mild motor weakness (4/5) and partial sensory loss.

Diagnosis

Upper Extremity Phlegmasia Cerulea Dolens from Subclavian Vein Compression and Thrombosis due to Subclavian Artery Pseudoaneurysm

Takayasu Arteritis

Approach to Diagnosis

The diagnostic approach for this patient integrated clinical, echocardiographic, and advanced imaging findings.

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Laboratory tests were largely normal except for mildly elevated liver enzymes, with normal inflammatory markers and cardiac evaluation. Imaging showed arterial wall thickening and venous obstruction on duplex studies, and CT angiography revealed a large saccular aneurysm of the left subclavian artery with chronic thrombus. This caused compression and near-occlusion of the left subclavian vein, thrombosis of the internal jugular vein, and associated stenoses of nearby arteries with collateral vessel formation.

Discussion

Takayasu arteritis (TA) is a chronic, immune-mediated large-vessel vasculitis that causes panarteritis, fibrosis, thrombus formation, and eventual arterial weakening leading to aneurysms. Subclavian artery involvement is uncommon in TA, and subclavian aneurysms are extremely rare, with only two cases previously reported (Bhandari et al. 2023)

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This case is also notable for upper-extremity phlegmasia cerulea dolens (PCD), a rare and severe form of venous thrombosis not previously described in TA due to combined DVT and mass effect from a subclavian pseudoaneurysm. Early recognition is crucial because upper-extremity PCD carries high morbidity and mortality. Management of TA centers on suppressing inflammation, while vascular complications may require endovascular or surgical interventions, and venous thrombosis necessitates anticoagulation (Alnabwani et al. 2021)

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Clinical Pearls

  • Q: What are the most common vessels affected by TA?

A: Aorta and its major branches

  • Q: What is the triad of Phlegmasia Cerulea Dolens?

A: Severe form of proximal deep vein thrombosis causing PAIN, CYANOSIS and EDEMA.

  • Q: What venous duplex ultrasound finding would suggest a more proximal level of obstruction?

A: Reduced waveform phasicity 

  • Q: What is the primary goal of treatment of TA?

A: Preservation of vasculature by suppression of inflammation

  • Q: What is the standard of treatment for PCD?

A: Revascularization through a combination of anticoagulation or thrombolysis and surgery 

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