The Journal of Foot & Ankle Surgery xxx (2016) 1–5
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
Case Reports and Series
Acute Limb-Threatening Ischemia Associated With Antiphospholipid Syndrome: A Report of Two Cases Kiwako Suzuki, MD 1, 2, Tetsuji Uemura, MD 3, Mamoru Kikuchi, MD 4, Yasuhiro Ishihara, MD 1, Shigeru Ichioka, MD 5 1
Assistant Professor, Department of Plastic and Reconstructive Surgery, Saga University School of Medicine, Saga, Japan Assistant Professor, Department of Plastic and Reconstructive Surgery, Saitama Medical University, Saitama, Japan Professor, Department of Plastic and Reconstructive Surgery, Saga University School of Medicine, Saga, Japan 4 Lecturer, Department of Plastic and Reconstructive Surgery, Saga University School of Medicine, Saga, Japan 5 Professor, Department of Plastic and Reconstructive Surgery, Saitama Medical University, Saitama, Japan 2 3
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Acute limb ischemia results from sudden deterioration in the arterial supply to the limb, occasionally leading to limb loss or fatality. Antiphospholipid syndrome (APS) is known to induce acute limb ischemia among the various etiologies responsible for arterial obstruction. APS is a systemic autoimmune disorder characterized by a combination of arterial and/or venous thrombosis and limb loss. It is often accompanied by a mild-tomoderate thrombocytopenia and elevated titers of antiphospholipid antibodies, including the lupus anticoagulant and the anticardiolipin antibodies. In the present report, we present 2 cases of acute limb ischemia due to APS associated with systemic lupus erythematosus. Angiography revealed arterial obstruction distal to the popliteal artery in both patients, and each patient eventually underwent below-the-knee amputation. Surgeons treating acute limb ischemia should remember APS, although this disease might not be common in daily clinical practice. Ó 2016 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: acute arterial occlusion antiphospholipid syndrome major amputation systemic lupus erythematosus
Acute limb ischemia represents one of the toughest challenges that extremity surgeons encounter in their clinical practice. Its diagnosis and initial assessment are largely clinical, and a diagnostic error risks serious consequences to the patient, including amputation of the limb or even death. Acute limb ischemia is often the endof-life condition that confronts a patient with multiple medical comorbidities. A careful clinical assessment is therefore very important for preservation of the limb and, in many cases, life salvage. Acute limb ischemia develops when an abrupt interruption of blood flow to an extremity occurs, usually because of either embolic or thrombotic vascular occlusion. Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the clinical features of recurrent thrombosis in the venous and/or arterial circulation, often associated with loss of the fetus in a pregnant female (1). The Euro-Phospholipid project estimates that the manifestations of APS in the lower extremities include deep vein thrombosis in 38.9%, superficial thrombophlebitis in 11.7%, and arterial thrombosis in 4.3% (2). In the present report, we describe 2 cases of lower
Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Kiwako Suzuki, MD, Department of Plastic and Reconstructive Surgery, Saga University School of Medicine, Saga 849-8501, Japan. E-mail address: [email protected] (K. Suzuki).
extremity arterial thrombosis associated with APS in patients with systemic lupus erythematosus (SLE). Case Reports Patient 1 A 48-year-old Japanese male diagnosed with SLE-associated APS had had his left leg scratched 2 months earlier, and he was referred to the Saga University Hospital for treatment of a refractory leg ulceration with cellulitis that had been present from February 2008 to August 2008. Three days before his referral, he had experienced sudden pain in his left limb and a high fever. He presented with widespread cutaneous necrosis (Fig. 1), and leg angiography was obtained. The angiogram revealed obstructions in both the anterior and the posterior tibial arteries of his left leg (Fig. 2). We identified the condition as acute limb ischemia with infection, with no indication for revascularization. Instead, we recommended major amputation for control of the pain and infection. After 14 days of medical stabilization and supportive therapy, the patient underwent surgery. We confirmed the presence of emboli in the anterior and posterior tibial arteries and venae comitantes during the operation (Fig. 3). Histopathologic examination disclosed organized thrombi, hypertrophy of the intima with crystal-form cholesterol, invasion of foam cells, and
1067-2516/$ - see front matter Ó 2016 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2016.01.002
2
K. Suzuki et al. / The Journal of Foot & Ankle Surgery xxx (2016) 1–5
Fig. 1. View of patient 1 showing widespread cutaneous necrosis.
thromboarteritis (Fig. 4). After surgery, anticoagulant therapy was initiated with oral doses of warfarin 5 mg/day, aspirin 100 mg/day, and sarpogrelate 300 mg/day. Immunosuppressive therapy, under the
guidance of the rheumatologist, was initiated with oral doses of prednisolone 35 mg/day, which was gradually decreased, and intermittent pulse intravenous cyclophosphamide therapy (750 mg/day). He survived the surgery and has been walking with appliances for >5 years since the amputation. Case 2 A 65-year-old Japanese female presented with the sudden onset of gangrene of the right forefoot and heel in October 2012 (Fig. 5). She had 2 children and no history of a miscarriage. A contrast
Fig. 2. Angiogram showing obstruction.
Fig. 3. The presence of emboli in the anterior and posterior tibial arteries and venae comitantes was confirmed during surgery.
K. Suzuki et al. / The Journal of Foot & Ankle Surgery xxx (2016) 1–5
3
Fig. 4. Histopathologic view showing organized thrombi, hypertrophy of the intima with crystal form cholesterol, invasion of foam cells and thromboarteritis.
computed tomography image revealed occlusion of the anterior and posterior tibial arteries (Fig. 6). Because the gangrene progressed rapidly and her platelet count was 13,000 mL secondary consumption due to embolism, an urgent below-the-knee amputation was performed after 3 days of hospitalization. During the surgery, we confirmed the presence of emboli in the right popliteal artery and veins (Fig. 7). After surgery, collagen disease specialists were consulted, and serology revealed an elevated antinuclear antibody level of 1:640, positive double-stranded DNA-IgG antibody, lupus anticoagulant, and positive anticardiolipin b2-glycoprotein complex antibody. Pathologic examination revealed only 1 thrombus in the venous and arterial circulation, with no other findings of note (Fig. 8). She was diagnosed with SLE and secondary APS. We initiated her treatment with oral doses of prednisolone 35 mg, warfarin 2 mg/day, and aspirin 100 mg/day. At 1 year after the operation, she was able to walk with appliances and remained under treatment for her collagen disease.
Discussion
Fig. 5. (A and B) Sudden onset of gangrene of the right forefoot and heel. Posterior tibial and dorsalis pedis pulse was not palpable.
Fig. 6. Contrast computed tomography showing occlusion of anterior and posterior tibial arteries.
Acute limb ischemia is the result of a sudden deterioration in the arterial supply to the limb. There are 2 main reasons for acute ischemia: arterial embolism or thrombosis. The usual source of an embolus is the heart, and the material is mural thrombus that has detached after an accumulation in the wall of the vessel. The other main cause of acute ischemia is atherosclerotic debris from a diseased proximal artery, often the thoracic aorta, in individuals with a heavy burden of atherosclerotic disease. Thrombosis results from blood clotting within an artery, which can be caused by progressive
4
K. Suzuki et al. / The Journal of Foot & Ankle Surgery xxx (2016) 1–5
Fig. 7. The presence of emboli in the right popliteal artery and veins was confirmed during surgery.
atherosclerotic obstruction, hypercoagulability, Burger’s disease, dehydration, and trauma. Some localized problems could also be present in the vessel itself (1).
APS, 1 of the acquired thrombotic diseases in our 2 patients, can also cause acute limb ischemia. APS is a systemic autoimmune disorder characterized by a combination of arterial and/or venous thrombosis and recurrent fetal loss, often accompanied by mild-to-moderate thrombocytopenia and elevated titers of antiphospholipid antibodies (i.e., the lupus anticoagulant and/or anticardiolipin antibodies). First recognized in patients with SLE and later, although less frequently, in other autoimmune disorders, it is now well-known that development of this syndrome can also be independent of any underlying disease. In such cases, it is called primary APS. More recently, a subset of APS cases with outstanding features has been described, including multiple occlusive events that usually affect the small vessels supplying organs and that manifest themselves within a short period. This subset has been termed catastrophic APS (2). Arterial thrombosis associated with APS first appeared in the published data in 1983 in the description of a stroke case. Involvement of other noncerebral arteries (both large and small) has subsequently been documented (3–5). Small vessel occlusions (e.g., glomerular, retinal, and nail fold) have been included in the latest consensus statements regarding APS (3,5). Although a great variety of clinical features have been described in patients with APS, gangrene leading to the amputation of a limb is 1 of the most devastating complications. Dubois and Arterberry (6) and Asherson et al (3) estimated the incidence of limb gangrene in SLE to be 1.3%. Yang et al (7), in their report on gangrene and SLE in 1996, reviewed 14 such cases from published studies (3). The Euro-Phospholipid project noted a 4.3% incidence of arterial thrombosis in the lower limbs (2). As a disease causing gangrene of the limbs, APS is very important clinically.
Fig. 8. Pathologic examination revealed only 1 thrombus in the venous and arterial circulation.
K. Suzuki et al. / The Journal of Foot & Ankle Surgery xxx (2016) 1–5 Table Patient characteristics Variable
Patient 1
Patient 2
Sex Age (y) Disease Duration (y) Precipitating factors Amputation Pathologic finding ANA positive dsDNA positive Complement level (mg/dL) C3 C4 CH50 aPL Obstruction
Male 48 SLE 20 DM, HL, smoking Below the knee Thromboarteritis Yes Yes
Female 65 SLE 0 None Below the knee Thrombus Yes Yes
41 7.9 14 b2-Glycoprotein I antibodies ATA, PTA
68 11 35 Anticardiolipin antibodies ATA
Abbreviations: ANA, antinuclear antibody; ATA, anterior tibial artery; aPL, antiphospholipid antibodies; DM, diabetes mellitus; HL, hyperlipidemia; PTA, posterior tibial artery; SLE, systemic lupus erythematosus.
5
In conclusion, we have described 2 cases of limb loss in patients with acute limb ischemia associated with SLE and APS. From our experience with these patients and our understanding of the published data, we have concluded that the following points are important to consider when treating a patient with acute limb ischemia due to APS: 1. Acute limb ischemia should be diagnosed carefully and without error. Once the diagnosis is firm, a therapeutic strategy should be established in consultation with a cardiovascular surgeon. If the limb is deemed nonsalvageable, its amputation must be a part of the strategy. 2. If APS is suspected, the patient should be treated in cooperation with a collagen disease specialist, such as a rheumatologist. 3. Vascular insufficiency can develop in both males and females, irrespective of their history of disease. The presence of antiphospholipid antibody positivity should be regarded as a major risk factor for the future development of vascular occlusions. References
The clinical and serologic features of our 2 patients are summarized in the Table. Originally diagnosed with SLE, both of our patients developed secondary APS. Patient 1 had been receiving treatment for SLE for 20 years before the onset of APS. In contrast, APS in patient 2 was incipient in the episode. The great majority of SLE cases are in females, as attested by the ratio of males to females of 1:9. However, males are afflicted, although rarely, with SLE just as in patient 1. However, nothing in his medical history of the disease showed an indication that might have led to gangrene. In both cases, the location of obstruction was distal to the popliteal artery; therefore, below-theknee amputation was applicable. Acute limb ischemia due to secondary APS can appear with no relation to the patient’s history of disease, and both males and females can experience its sudden onset. APS might not be a common cause of acute ischemia of the limb. However, surgeons treating a case of acute limb ischemia should remember the possibility of APS. Surgeons often have very limited choice regarding when an amputation is necessary (6,8,9). The rare indication of a devastating major amputation is recognized only when it is a documented situation in which obstruction of large- and medium-size arteries will surely result in lower extremity ischemia.
1. Earnshaw JJ. Evaluation and decision making. In: Rutherford’s Vascular Surgery, ed 7., pp. 2389–2398, edited by JL Cronenwett, KW Johnston, WB Saunders, Philadelphia, 2010. 2. Cervera R, Boffa M-C, Khamashta MA, Hughes GRV. The Euro-Phospholipid project: epidemiology of the antiphospholipid syndrome in Europe. Lupus 18:889–893, 2009. 3. Asherson RA, Cervera R, Stojanovic L, Bucciarelli S, Levy R. Amputation of digits or limbs in patients with antiphospholipid syndrome. Semin Arthristis Rheum 38:124–131, 2008. 4. Hughes GRV. Thrombosis, abortion, cerebral disease and the “lupus anticoagulant.”. BMJ 287:47–51, 1983. 5. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, Derksen RH, de Groot PG, Koike T, Meroni PL, Reber G, Shoenfeld Y, Tincani A, Viachoyiannopoulos PG, Krilis SA. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome. J Thromb Hemost 4:295–306, 2006. 6. Dubois EL, Arterberry JD. Gangrene as a manifestation of systemic lupus erythematosus. JAMA 181:366–374, 1962. 7. Yang SG, Kim KH, Park KC, Youn SW, Joh GY. A case of systemic lupus erythematosus showing acute gangrenous changes of fingertips. Br J Dermatol 134:178– 192, 1996. 8. Jou IM, Liu MF, Chao SC. Widespread cutaneous necrosis associated with antiphospholopid syndrome. Clin Rheumatol 15:394–398, 1996. 9. Dubois EL. Gangrene of the extremities. In: Lupus Erythematosus, ed 2, pp. 296– 305, edited by EL Dubois, University of Southern California Press, Los Angeles, 1976.
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
Case Reports and Series
Acute Limb-Threatening Ischemia Associated With Antiphospholipid Syndrome: A Report of Two Cases Kiwako Suzuki, MD 1, 2, Tetsuji Uemura, MD 3, Mamoru Kikuchi, MD 4, Yasuhiro Ishihara, MD 1, Shigeru Ichioka, MD 5 1
Assistant Professor, Department of Plastic and Reconstructive Surgery, Saga University School of Medicine, Saga, Japan Assistant Professor, Department of Plastic and Reconstructive Surgery, Saitama Medical University, Saitama, Japan Professor, Department of Plastic and Reconstructive Surgery, Saga University School of Medicine, Saga, Japan 4 Lecturer, Department of Plastic and Reconstructive Surgery, Saga University School of Medicine, Saga, Japan 5 Professor, Department of Plastic and Reconstructive Surgery, Saitama Medical University, Saitama, Japan 2 3
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Acute limb ischemia results from sudden deterioration in the arterial supply to the limb, occasionally leading to limb loss or fatality. Antiphospholipid syndrome (APS) is known to induce acute limb ischemia among the various etiologies responsible for arterial obstruction. APS is a systemic autoimmune disorder characterized by a combination of arterial and/or venous thrombosis and limb loss. It is often accompanied by a mild-tomoderate thrombocytopenia and elevated titers of antiphospholipid antibodies, including the lupus anticoagulant and the anticardiolipin antibodies. In the present report, we present 2 cases of acute limb ischemia due to APS associated with systemic lupus erythematosus. Angiography revealed arterial obstruction distal to the popliteal artery in both patients, and each patient eventually underwent below-the-knee amputation. Surgeons treating acute limb ischemia should remember APS, although this disease might not be common in daily clinical practice. Ó 2016 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: acute arterial occlusion antiphospholipid syndrome major amputation systemic lupus erythematosus
Acute limb ischemia represents one of the toughest challenges that extremity surgeons encounter in their clinical practice. Its diagnosis and initial assessment are largely clinical, and a diagnostic error risks serious consequences to the patient, including amputation of the limb or even death. Acute limb ischemia is often the endof-life condition that confronts a patient with multiple medical comorbidities. A careful clinical assessment is therefore very important for preservation of the limb and, in many cases, life salvage. Acute limb ischemia develops when an abrupt interruption of blood flow to an extremity occurs, usually because of either embolic or thrombotic vascular occlusion. Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the clinical features of recurrent thrombosis in the venous and/or arterial circulation, often associated with loss of the fetus in a pregnant female (1). The Euro-Phospholipid project estimates that the manifestations of APS in the lower extremities include deep vein thrombosis in 38.9%, superficial thrombophlebitis in 11.7%, and arterial thrombosis in 4.3% (2). In the present report, we describe 2 cases of lower
Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Kiwako Suzuki, MD, Department of Plastic and Reconstructive Surgery, Saga University School of Medicine, Saga 849-8501, Japan. E-mail address: [email protected] (K. Suzuki).
extremity arterial thrombosis associated with APS in patients with systemic lupus erythematosus (SLE). Case Reports Patient 1 A 48-year-old Japanese male diagnosed with SLE-associated APS had had his left leg scratched 2 months earlier, and he was referred to the Saga University Hospital for treatment of a refractory leg ulceration with cellulitis that had been present from February 2008 to August 2008. Three days before his referral, he had experienced sudden pain in his left limb and a high fever. He presented with widespread cutaneous necrosis (Fig. 1), and leg angiography was obtained. The angiogram revealed obstructions in both the anterior and the posterior tibial arteries of his left leg (Fig. 2). We identified the condition as acute limb ischemia with infection, with no indication for revascularization. Instead, we recommended major amputation for control of the pain and infection. After 14 days of medical stabilization and supportive therapy, the patient underwent surgery. We confirmed the presence of emboli in the anterior and posterior tibial arteries and venae comitantes during the operation (Fig. 3). Histopathologic examination disclosed organized thrombi, hypertrophy of the intima with crystal-form cholesterol, invasion of foam cells, and
1067-2516/$ - see front matter Ó 2016 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2016.01.002
2
K. Suzuki et al. / The Journal of Foot & Ankle Surgery xxx (2016) 1–5
Fig. 1. View of patient 1 showing widespread cutaneous necrosis.
thromboarteritis (Fig. 4). After surgery, anticoagulant therapy was initiated with oral doses of warfarin 5 mg/day, aspirin 100 mg/day, and sarpogrelate 300 mg/day. Immunosuppressive therapy, under the
guidance of the rheumatologist, was initiated with oral doses of prednisolone 35 mg/day, which was gradually decreased, and intermittent pulse intravenous cyclophosphamide therapy (750 mg/day). He survived the surgery and has been walking with appliances for >5 years since the amputation. Case 2 A 65-year-old Japanese female presented with the sudden onset of gangrene of the right forefoot and heel in October 2012 (Fig. 5). She had 2 children and no history of a miscarriage. A contrast
Fig. 2. Angiogram showing obstruction.
Fig. 3. The presence of emboli in the anterior and posterior tibial arteries and venae comitantes was confirmed during surgery.
K. Suzuki et al. / The Journal of Foot & Ankle Surgery xxx (2016) 1–5
3
Fig. 4. Histopathologic view showing organized thrombi, hypertrophy of the intima with crystal form cholesterol, invasion of foam cells and thromboarteritis.
computed tomography image revealed occlusion of the anterior and posterior tibial arteries (Fig. 6). Because the gangrene progressed rapidly and her platelet count was 13,000 mL secondary consumption due to embolism, an urgent below-the-knee amputation was performed after 3 days of hospitalization. During the surgery, we confirmed the presence of emboli in the right popliteal artery and veins (Fig. 7). After surgery, collagen disease specialists were consulted, and serology revealed an elevated antinuclear antibody level of 1:640, positive double-stranded DNA-IgG antibody, lupus anticoagulant, and positive anticardiolipin b2-glycoprotein complex antibody. Pathologic examination revealed only 1 thrombus in the venous and arterial circulation, with no other findings of note (Fig. 8). She was diagnosed with SLE and secondary APS. We initiated her treatment with oral doses of prednisolone 35 mg, warfarin 2 mg/day, and aspirin 100 mg/day. At 1 year after the operation, she was able to walk with appliances and remained under treatment for her collagen disease.
Discussion
Fig. 5. (A and B) Sudden onset of gangrene of the right forefoot and heel. Posterior tibial and dorsalis pedis pulse was not palpable.
Fig. 6. Contrast computed tomography showing occlusion of anterior and posterior tibial arteries.
Acute limb ischemia is the result of a sudden deterioration in the arterial supply to the limb. There are 2 main reasons for acute ischemia: arterial embolism or thrombosis. The usual source of an embolus is the heart, and the material is mural thrombus that has detached after an accumulation in the wall of the vessel. The other main cause of acute ischemia is atherosclerotic debris from a diseased proximal artery, often the thoracic aorta, in individuals with a heavy burden of atherosclerotic disease. Thrombosis results from blood clotting within an artery, which can be caused by progressive
4
K. Suzuki et al. / The Journal of Foot & Ankle Surgery xxx (2016) 1–5
Fig. 7. The presence of emboli in the right popliteal artery and veins was confirmed during surgery.
atherosclerotic obstruction, hypercoagulability, Burger’s disease, dehydration, and trauma. Some localized problems could also be present in the vessel itself (1).
APS, 1 of the acquired thrombotic diseases in our 2 patients, can also cause acute limb ischemia. APS is a systemic autoimmune disorder characterized by a combination of arterial and/or venous thrombosis and recurrent fetal loss, often accompanied by mild-to-moderate thrombocytopenia and elevated titers of antiphospholipid antibodies (i.e., the lupus anticoagulant and/or anticardiolipin antibodies). First recognized in patients with SLE and later, although less frequently, in other autoimmune disorders, it is now well-known that development of this syndrome can also be independent of any underlying disease. In such cases, it is called primary APS. More recently, a subset of APS cases with outstanding features has been described, including multiple occlusive events that usually affect the small vessels supplying organs and that manifest themselves within a short period. This subset has been termed catastrophic APS (2). Arterial thrombosis associated with APS first appeared in the published data in 1983 in the description of a stroke case. Involvement of other noncerebral arteries (both large and small) has subsequently been documented (3–5). Small vessel occlusions (e.g., glomerular, retinal, and nail fold) have been included in the latest consensus statements regarding APS (3,5). Although a great variety of clinical features have been described in patients with APS, gangrene leading to the amputation of a limb is 1 of the most devastating complications. Dubois and Arterberry (6) and Asherson et al (3) estimated the incidence of limb gangrene in SLE to be 1.3%. Yang et al (7), in their report on gangrene and SLE in 1996, reviewed 14 such cases from published studies (3). The Euro-Phospholipid project noted a 4.3% incidence of arterial thrombosis in the lower limbs (2). As a disease causing gangrene of the limbs, APS is very important clinically.
Fig. 8. Pathologic examination revealed only 1 thrombus in the venous and arterial circulation.
K. Suzuki et al. / The Journal of Foot & Ankle Surgery xxx (2016) 1–5 Table Patient characteristics Variable
Patient 1
Patient 2
Sex Age (y) Disease Duration (y) Precipitating factors Amputation Pathologic finding ANA positive dsDNA positive Complement level (mg/dL) C3 C4 CH50 aPL Obstruction
Male 48 SLE 20 DM, HL, smoking Below the knee Thromboarteritis Yes Yes
Female 65 SLE 0 None Below the knee Thrombus Yes Yes
41 7.9 14 b2-Glycoprotein I antibodies ATA, PTA
68 11 35 Anticardiolipin antibodies ATA
Abbreviations: ANA, antinuclear antibody; ATA, anterior tibial artery; aPL, antiphospholipid antibodies; DM, diabetes mellitus; HL, hyperlipidemia; PTA, posterior tibial artery; SLE, systemic lupus erythematosus.
5
In conclusion, we have described 2 cases of limb loss in patients with acute limb ischemia associated with SLE and APS. From our experience with these patients and our understanding of the published data, we have concluded that the following points are important to consider when treating a patient with acute limb ischemia due to APS: 1. Acute limb ischemia should be diagnosed carefully and without error. Once the diagnosis is firm, a therapeutic strategy should be established in consultation with a cardiovascular surgeon. If the limb is deemed nonsalvageable, its amputation must be a part of the strategy. 2. If APS is suspected, the patient should be treated in cooperation with a collagen disease specialist, such as a rheumatologist. 3. Vascular insufficiency can develop in both males and females, irrespective of their history of disease. The presence of antiphospholipid antibody positivity should be regarded as a major risk factor for the future development of vascular occlusions. References
The clinical and serologic features of our 2 patients are summarized in the Table. Originally diagnosed with SLE, both of our patients developed secondary APS. Patient 1 had been receiving treatment for SLE for 20 years before the onset of APS. In contrast, APS in patient 2 was incipient in the episode. The great majority of SLE cases are in females, as attested by the ratio of males to females of 1:9. However, males are afflicted, although rarely, with SLE just as in patient 1. However, nothing in his medical history of the disease showed an indication that might have led to gangrene. In both cases, the location of obstruction was distal to the popliteal artery; therefore, below-theknee amputation was applicable. Acute limb ischemia due to secondary APS can appear with no relation to the patient’s history of disease, and both males and females can experience its sudden onset. APS might not be a common cause of acute ischemia of the limb. However, surgeons treating a case of acute limb ischemia should remember the possibility of APS. Surgeons often have very limited choice regarding when an amputation is necessary (6,8,9). The rare indication of a devastating major amputation is recognized only when it is a documented situation in which obstruction of large- and medium-size arteries will surely result in lower extremity ischemia.
1. Earnshaw JJ. Evaluation and decision making. In: Rutherford’s Vascular Surgery, ed 7., pp. 2389–2398, edited by JL Cronenwett, KW Johnston, WB Saunders, Philadelphia, 2010. 2. Cervera R, Boffa M-C, Khamashta MA, Hughes GRV. The Euro-Phospholipid project: epidemiology of the antiphospholipid syndrome in Europe. Lupus 18:889–893, 2009. 3. Asherson RA, Cervera R, Stojanovic L, Bucciarelli S, Levy R. Amputation of digits or limbs in patients with antiphospholipid syndrome. Semin Arthristis Rheum 38:124–131, 2008. 4. Hughes GRV. Thrombosis, abortion, cerebral disease and the “lupus anticoagulant.”. BMJ 287:47–51, 1983. 5. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, Derksen RH, de Groot PG, Koike T, Meroni PL, Reber G, Shoenfeld Y, Tincani A, Viachoyiannopoulos PG, Krilis SA. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome. J Thromb Hemost 4:295–306, 2006. 6. Dubois EL, Arterberry JD. Gangrene as a manifestation of systemic lupus erythematosus. JAMA 181:366–374, 1962. 7. Yang SG, Kim KH, Park KC, Youn SW, Joh GY. A case of systemic lupus erythematosus showing acute gangrenous changes of fingertips. Br J Dermatol 134:178– 192, 1996. 8. Jou IM, Liu MF, Chao SC. Widespread cutaneous necrosis associated with antiphospholopid syndrome. Clin Rheumatol 15:394–398, 1996. 9. Dubois EL. Gangrene of the extremities. In: Lupus Erythematosus, ed 2, pp. 296– 305, edited by EL Dubois, University of Southern California Press, Los Angeles, 1976.
