Painful blue fingers

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doi:doi: 10.12788/jfp.0557

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Painful blue fingers

J Fam Pract. 2023 March;72(2):E9-E11 | doi: 10.12788/jfp.0557

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References

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2. Duarte-García A, Pham MM, Crowson CS, et al. The epidemiology of antiphospholipid syndrome: a population-based study. Arthritis Rheumatol. 2019;71:1545-1552. doi: 10.1002/art.40901

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5. Love PE, Santoro SA. Antiphospholipid antibodies: anticardiolipin and the lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders: prevalence and clinical significance. Ann Intern Med. 1990;112:682-698. doi: 10.7326/0003-4819-112-9-682

6. Merkel PA, Chang YC, Pierangeli SS, et al. The prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue diseases. Am J Med. 1996;101:576-583. doi: 10.1016/s0002-9343(96)00335-x

7. Petri M. Epidemiology of the antiphospholipid antibody syndrome. J Autoimmun. 2000;15:145-151. doi: 10.1006/jaut. 2000.0409

8. Bozlar U, Ogur T, Khaja MS, et al. CT angiography of the upper extremity arterial system: Part 2—Clinical applications beyond trauma patients. AJR Am J Roentgenol. 2013;201:753-763. doi: 10.2214/AJR.13.11208

9. Ruiz-Irastorza G, Hunt BJ, Khamashta MA. A systematic review of secondary thromboprophylaxis in patients with antiphospholipid antibodies. Arthritis Rheum. 2007;7:1487-1495. doi: 10.1002/art.23109

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Diagnosis: Antiphospholipid syndrome

Given our patient’s SLE, left subclavian artery thrombosis, digital ischemia, and high-titer antiphospholipid antibodies, we had significant concern for antiphospholipid syndrome (APS). The diagnosis of APS is most often based on the fulfillment of the revised Sapporo classification criteria. These criteria include both clinical criteria (vascular thrombosis or pregnancy morbidity) and laboratory criteria (the presence of antiphospholipid antibodies on at least 2 separate occasions separated by 12 weeks).¹ Our patient met clinical criteria given the evidence of subclavian artery thrombosis on CTA as well as digital plethysmography findings consistent with digital emboli. To meet laboratory criteria, she would have needed to have persistent high-titer antiphospholipid antibodies 12 weeks apart.

Previous studies have estimated that 30% to 50% of patients with SLE who test positive for antiphospholipid antibodies will develop thrombosis.

APS is an autoimmune disease in which the presence of antiphospholipid antibodies is associated with thrombosis; it can be divided into primary and secondary APS. The estimated prevalence of APS is 50 per 100,000 people in the United States.² Primary APS occurs in the absence of an underlying autoimmune disease, while secondary APS occurs in the presence of an underlying autoimmune disease.

The autoimmune disease most often associated with APS is SLE.³ Among patients with SLE, 15% to 34% have positive lupus anticoagulant and 12% to 30% have anticardiolipin antibodies.^4-6 This is compared with young healthy subjects among whom only 1% to 4% have positive lupus anticoagulant and 1% to 5% have anticardiolipin antibodies.⁷ Previous studies have estimated that 30% to 50% of patients with SLE who test positive for antiphospholipid antibodies will develop thrombosis.^5,7

Differential includes Raynaud phenomenon, vasculitis

The differential diagnosis for digital ischemia in a patient with SLE includes APS, Raynaud phenomenon, vasculitis, and septic emboli.

Raynaud phenomenon can manifest in patients with SLE, but the presence of thrombosis on CTA and high-titer positive antiphospholipid antibodies make the diagnosis of APS more likely. Additionally, Raynaud phenomenon is typically temperature dependent with vasospasm in the digital arteries, occurring in cold temperatures and resolving with warming.

Systemic vasculitis may develop in patients with SLE, but in our case was less likely given the patient did not have any evidence of vasculitis on CTA, such as blood vessel wall thickening and/or enhancement.⁸

Septic emboli from endocarditis can cause digital ischemia but is typically associated with positive blood cultures, fever, and other systemic signs of infection, and/or vegetations on an echocardiogram.

Continue to: Thrombosis determines intensity of lifelong antiocagulation Tx