Case Report: An Unusual Case of Acute Lower Limb Ischemia as Precursor of the Asherson's Syndrome

Abstract

Introduction: Asherson's Syndrome, also defined as Catastrophic Antiphospholipid Syndrome (CAPS), represents the most severe manifestation of Antiphospholipid Antibody Syndrome. Rarely, the first CAPS diagnosis is based on macro-thrombotic event as acute limb ischemia.Case Presentation: We present a case of a 65-year-old woman admitted with an acute lower limb arterial ischemia with a complete occlusion of all the three tibial vessels. Three endovascular recanalization procedures were performed contemporary to 48 h intraarterial thrombolysis administration. The patency of tibial arteries was restored with a near-complete absence of digital arteries and microvessel perfusion of the foot. In the following days, an aggressive foot gangrene was established, leading to a major lower-limb amputation. Due to the general clinical status worsening and aggressiveness of ischemic condition, further investigations were performed leading to the diagnosis of an aggressive Asherson's Syndrome that was also complicated by a severe heparin-induced thrombocytopenia. Medical management with a high dose of intravenous steroids and nine sessions of plasma exchange led to a clinical condition stabilization.Conclusion: In our case, the presence of a “sine causa” acute arterial occlusion of a large vessel represented the first manifestation of an aggressive form of Asherson's Syndrome that could represent a fatal disease. Due to the extreme variety of manifestations, early clinical suspicion, diagnosis, and multidisciplinary management are essential to limit the life-threatening consequences of patients.

A young lady presenting with acute limb ischemia secondary to systemic lupus erythematosus, antiphospholipid syndrome and infective endocarditis

“…Although it is rare, APLS can sometimes present as acute limb ischemia. 7 Previous case reports have shown a link between arterial thrombosis in the lower limbs and both SLE and APLS. 8 , 9 However, upper limb thrombosis is a rare occurrence and only a few case reports have been published.…”

Hallux Partial Necrosis Associated with Antiphospholipid Syndrome: The Importance of Early Accurate Diagnosis

“…In patients with previous episodes of venous or arterial thrombosis in whom APS is suspected, early diagnosis and multidisciplinary management are essential to avoid potentially fatal consequences. An early diagnosis can also prevent the appearance of CAPS, the accelerated presentation form of APS that is characterized by multiple organ failure and very poor prognosis [10,11].…”