eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Antiphospholipid Antibody Syndrome: Multimedia

Author: Barry L Myones, MD, Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital
Contributor Information and Disclosures

Updated: Aug 4, 2009

Multimedia

Palmar livedo reticularis associated with antipho...Media file 1: Palmar livedo reticularis associated with antiphospholipid antibody syndrome may range from a lacy, flat, reticulated pattern to a more confluent, nonblanching, slightly raised rash (secondary to extravasation of RBCs and plasma).
Palmar livedo reticularis associated with antipho...

Palmar livedo reticularis associated with antiphospholipid antibody syndrome may range from a lacy, flat, reticulated pattern to a more confluent, nonblanching, slightly raised rash (secondary to extravasation of RBCs and plasma).

Livedo reticularis of the upper and lower extremi...Media file 2: Livedo reticularis of the upper and lower extremities in a 15-year-old adolescent with primary antiphospholipid antibody syndrome. The pattern is lacy, flat, and nonblanching. The purplish hue is from stasis in the small vessel beds.
Livedo reticularis of the upper and lower extremi...

Livedo reticularis of the upper and lower extremities in a 15-year-old adolescent with primary antiphospholipid antibody syndrome. The pattern is lacy, flat, and nonblanching. The purplish hue is from stasis in the small vessel beds.

Muddy discoloration and mild diffuse swelling of ...Media file 3: Muddy discoloration and mild diffuse swelling of the fingers observed as part of the Raynaud phenomenon, which is associated with antiphospholipid antibody syndrome. At room temperature, this patient still has decreased capillary refill and cold fingers despite treatment with pentoxifylline. The discoloration extends proximally onto the palms and turns blue-purple when exposed to cold.
Muddy discoloration and mild diffuse swelling of ...

Muddy discoloration and mild diffuse swelling of the fingers observed as part of the Raynaud phenomenon, which is associated with antiphospholipid antibody syndrome. At room temperature, this patient still has decreased capillary refill and cold fingers despite treatment with pentoxifylline. The discoloration extends proximally onto the palms and turns blue-purple when exposed to cold.

Linear splinter hemorrhages are found under the n...Media file 4: Linear splinter hemorrhages are found under the nails of fingers and toes. These may be solitary or multiple and appear intermittently.
Linear splinter hemorrhages are found under the n...

Linear splinter hemorrhages are found under the nails of fingers and toes. These may be solitary or multiple and appear intermittently.

One set of suggested algorithms for the workup an...Media file 5: One set of suggested algorithms for the workup and treatment of patients with antiphospholipid antibody syndrome. This should not be considered dogmatic because laboratory evaluation is not standardized and treatment remains empiric and controversial. Laboratory testing is not recommended in healthy asymptomatic individuals with no risk factors and a negative family history.
One set of suggested algorithms for the workup an...

One set of suggested algorithms for the workup and treatment of patients with antiphospholipid antibody syndrome. This should not be considered dogmatic because laboratory evaluation is not standardized and treatment remains empiric and controversial. Laboratory testing is not recommended in healthy asymptomatic individuals with no risk factors and a negative family history.

Occlusion of the right middle cerebral artery in ...Media file 6: Occlusion of the right middle cerebral artery in a 3-year-old child with severe headache and hemiparesis associated with anticardiolipin antibodies.
Occlusion of the right middle cerebral artery in ...

Occlusion of the right middle cerebral artery in a 3-year-old child with severe headache and hemiparesis associated with anticardiolipin antibodies.

Organizing thrombus in an aortic valve in a patie...Media file 7: Organizing thrombus in an aortic valve in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE) and recurrent thrombotic events. The authors acknowledge the help of Hannes Vogel, MD, in preparing this image.
Organizing thrombus in an aortic valve in a patie...

Organizing thrombus in an aortic valve in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE) and recurrent thrombotic events. The authors acknowledge the help of Hannes Vogel, MD, in preparing this image.

High-power degenerating aortic valve in a patient...Media file 8: High-power degenerating aortic valve in a patient who has positive test results for antiphospholipid antibody and lupus anticoagulant and who has systemic lupus erythematosus (SLE) and recurrent thrombotic events. The authors acknowledge the help of Hannes Vogel, MD, in preparing this image.
High-power degenerating aortic valve in a patient...

High-power degenerating aortic valve in a patient who has positive test results for antiphospholipid antibody and lupus anticoagulant and who has systemic lupus erythematosus (SLE) and recurrent thrombotic events. The authors acknowledge the help of Hannes Vogel, MD, in preparing this image.

Trichrome stain of a thrombus in the intestinal s...Media file 9: Trichrome stain of a thrombus in the intestinal serosa in a patient who has positive test results for antiphospholipid antibody and lupus anticoagulant and who has systemic lupus erythematosus (SLE) and catastrophic antiphospholipid antibody syndrome (CAPS). The authors acknowledge the help of Hannes Vogel, MD, in preparing this image.
Trichrome stain of a thrombus in the intestinal s...

Trichrome stain of a thrombus in the intestinal serosa in a patient who has positive test results for antiphospholipid antibody and lupus anticoagulant and who has systemic lupus erythematosus (SLE) and catastrophic antiphospholipid antibody syndrome (CAPS). The authors acknowledge the help of Hannes Vogel, MD, in preparing this image.

Antiphospholipid antibody syndrome in a patient w...Media file 10: Antiphospholipid antibody syndrome in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE), World Health Organization (WHO) class IV lupus nephritis, and acute renal failure. Top: Thrombosed kidney vessels (periodic acid-Schiff [PAS], original magnification X40). Bottom: Thrombosed kidney vessels (PAS, original magnification X20). Lumen is filled with eosinophilic fibrin with overlying injured endothelial cells. The authors acknowledge the help of Karen W. Eldin, MD, in preparing this image.
Antiphospholipid antibody syndrome in a patient w...

Antiphospholipid antibody syndrome in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE), World Health Organization (WHO) class IV lupus nephritis, and acute renal failure. Top: Thrombosed kidney vessels (periodic acid-Schiff [PAS], original magnification X40). Bottom: Thrombosed kidney vessels (PAS, original magnification X20). Lumen is filled with eosinophilic fibrin with overlying injured endothelial cells. The authors acknowledge the help of Karen W. Eldin, MD, in preparing this image.

Antiphospholipid antibody syndrome in a patient w...Media file 11: Antiphospholipid antibody syndrome in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE), World Health Organization (WHO) class IV lupus nephritis, and acute renal failure. Top: Thrombosed kidney vessel (hematoxylin and eosin [H&E] stain, original magnification X20). Lumen is occluded with fibrin. A perivascular stromal reaction with degenerating inflammatory cells is observed. Bottom: Thrombosed kidney vessel (H&E stain, original magnification X20). Lumen is occluded with fibrin. The authors acknowledge the help of Karen W. Eldin, MD, in preparing this image.
Antiphospholipid antibody syndrome in a patient w...

Antiphospholipid antibody syndrome in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE), World Health Organization (WHO) class IV lupus nephritis, and acute renal failure. Top: Thrombosed kidney vessel (hematoxylin and eosin [H&E] stain, original magnification X20). Lumen is occluded with fibrin. A perivascular stromal reaction with degenerating inflammatory cells is observed. Bottom: Thrombosed kidney vessel (H&E stain, original magnification X20). Lumen is occluded with fibrin. The authors acknowledge the help of Karen W. Eldin, MD, in preparing this image.

Antiphospholipid antibody syndrome in a patient w...Media file 12: Antiphospholipid antibody syndrome in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE), World Health Organization (WHO) class IV lupus nephritis, and acute renal failure. Thrombosed kidney vessel with recanalization (arrows) (Jones stain, original magnification X20). Architectural distortion in the surrounding stroma is observed. The authors acknowledge the help of Karen W. Eldin, MD, in preparing this image.
Antiphospholipid antibody syndrome in a patient w...

Antiphospholipid antibody syndrome in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE), World Health Organization (WHO) class IV lupus nephritis, and acute renal failure. Thrombosed kidney vessel with recanalization (arrows) (Jones stain, original magnification X20). Architectural distortion in the surrounding stroma is observed. The authors acknowledge the help of Karen W. Eldin, MD, in preparing this image.

Antiphospholipid antibody syndrome in a patient w...Media file 13: Antiphospholipid antibody syndrome in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE) and thrombocytopenia. Livedo reticularis of the upper extremities, which developed as petechiae in the classic lacy, reticular pattern, is observed.
Antiphospholipid antibody syndrome in a patient w...

Antiphospholipid antibody syndrome in a patient with positive test results for antiphospholipid antibody and lupus anticoagulant who has systemic lupus erythematosus (SLE) and thrombocytopenia. Livedo reticularis of the upper extremities, which developed as petechiae in the classic lacy, reticular pattern, is observed.

Livedo reticularis of the upper extremities, whic...Media file 14: Livedo reticularis of the upper extremities, which developed as petechiae in the classic lacy, reticular pattern and evolved as a confluent, nonblanching, slightly raised purpuric rash in the same reticular pattern. Same patient as in Media file 13.
Livedo reticularis of the upper extremities, whic...

Livedo reticularis of the upper extremities, which developed as petechiae in the classic lacy, reticular pattern and evolved as a confluent, nonblanching, slightly raised purpuric rash in the same reticular pattern. Same patient as in Media file 13.

Digital infarctions in a patient with systemic lu...Media file 15: Digital infarctions in a patient with systemic lupus erythematosus with antiphospholipid syndrome (APS) and long-standing Raynaud symptoms. Multiple and repeated digital infarctions are depicted, resulting in ulcerations and scarring. Scars and hyperpigmentation are also seen on the palmer aspect of hands and fingers.
Digital infarctions in a patient with systemic lu...

Digital infarctions in a patient with systemic lupus erythematosus with antiphospholipid syndrome (APS) and long-standing Raynaud symptoms. Multiple and repeated digital infarctions are depicted, resulting in ulcerations and scarring. Scars and hyperpigmentation are also seen on the palmer aspect of hands and fingers.

A patient (the same as in media files 17-19) with...Media file 16: A patient (the same as in media files 17-19) with multisystem small vessel coagulopathy (microangiopathy) but no known underlying disease process. Extensive involvement of all digits is noted, some with distal infarction and dry gangrene, others healing with residual eschar (and undermining epithelialization), and some with re-epithelialization and scarring. Healed superficial epidermal damage and desquamation is also present.
A patient (the same as in media files 17-19) with...

A patient (the same as in media files 17-19) with multisystem small vessel coagulopathy (microangiopathy) but no known underlying disease process. Extensive involvement of all digits is noted, some with distal infarction and dry gangrene, others healing with residual eschar (and undermining epithelialization), and some with re-epithelialization and scarring. Healed superficial epidermal damage and desquamation is also present.

A patient (the same as in Media files 16, 18, and...Media file 17: A patient (the same as in Media files 16, 18, and 19) with multisystem small vessel coagulopathy (microangiopathy) but no known underlying disease process. Eschar is still present on first digit bilaterally. More superficial lesions are shown here than are shown in Media file 16, with evolution and healing of lesions on all other toes.
A patient (the same as in Media files 16, 18, and...

A patient (the same as in Media files 16, 18, and 19) with multisystem small vessel coagulopathy (microangiopathy) but no known underlying disease process. Eschar is still present on first digit bilaterally. More superficial lesions are shown here than are shown in Media file 16, with evolution and healing of lesions on all other toes.

CAPS, Bone Infarction - MRI (High Resolution Prot...Media file 18: CAPS, Bone Infarction - MRI (High Resolution Proton Density and STIR images) and Nuclear Bone Scan - Patient (same as in images 16, 17, 19) with multisystem small vessel coagulopathy (microangiopathy) but no known underlying disease process. MRI shows multiple infarctions in the distal tibia, tarsal bones and metatarsal bones (extensive bone marrow edema and increased T1 with fat saturation signal in the calcaneus bones). Flow and early blood pool images of technetium 99m bone scan show increase in activity in both heel regions with focal areas of decreased activity in the center of each calcaneus.
CAPS, Bone Infarction - MRI (High Resolution Prot...

CAPS, Bone Infarction - MRI (High Resolution Proton Density and STIR images) and Nuclear Bone Scan - Patient (same as in images 16, 17, 19) with multisystem small vessel coagulopathy (microangiopathy) but no known underlying disease process. MRI shows multiple infarctions in the distal tibia, tarsal bones and metatarsal bones (extensive bone marrow edema and increased T1 with fat saturation signal in the calcaneus bones). Flow and early blood pool images of technetium 99m bone scan show increase in activity in both heel regions with focal areas of decreased activity in the center of each calcaneus.

A patient (the same as in Media files 16-18) with...Media file 19: A patient (the same as in Media files 16-18) with multisystem small vessel coagulopathy (microangiopathy) but no known underlying disease process. The technetium 99m bone scan reveals irregular multifocal areas of tracer accumulation within the left ventricle of the heart suggestive of myocardial infarction and altered calcium deposition. Irregular cutaneous and subcutaneous uptake is noted in multiple areas of the torso and upper arms (as well as in the upper thighs). High-resolution CT scanning of the chest reveals extensive calcification involving the myocardium, the mitral and tricuspid valve annuli, the aortic valve annulus, the proximal right coronary artery, and the left main coronary artery.
A patient (the same as in Media files 16-18) with...

A patient (the same as in Media files 16-18) with multisystem small vessel coagulopathy (microangiopathy) but no known underlying disease process. The technetium 99m bone scan reveals irregular multifocal areas of tracer accumulation within the left ventricle of the heart suggestive of myocardial infarction and altered calcium deposition. Irregular cutaneous and subcutaneous uptake is noted in multiple areas of the torso and upper arms (as well as in the upper thighs). High-resolution CT scanning of the chest reveals extensive calcification involving the myocardium, the mitral and tricuspid valve annuli, the aortic valve annulus, the proximal right coronary artery, and the left main coronary artery.

More on Antiphospholipid Antibody Syndrome

Overview: Antiphospholipid Antibody Syndrome
Differential Diagnoses & Workup: Antiphospholipid Antibody Syndrome
Treatment & Medication: Antiphospholipid Antibody Syndrome
Follow-up: Antiphospholipid Antibody Syndrome
Multimedia: Antiphospholipid Antibody Syndrome
References

References

  1. Myones BL, McCurdy D. The antiphospholipid syndrome: immunologic and clinical aspects. Clinical spectrum and treatment. J Rheumatol. Apr 2000;27 Suppl 58:20-8. [Medline].

  2. Cuadrado MJ, Hughes GR. Hughes (antiphospholipid) syndrome. Clinical features. Rheum Dis Clin North Am. Aug 2001;27(3):507-24, v. [Medline].

  3. Roubey RA. Antiphospholipid antibody syndrome. In: Koopman's Textbook of Arthritis and Allied Health Conditions. 14th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2001:1546-61.

  4. Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med. Mar 7 2002;346(10):752-63. [Medline].

  5. Lockshin MD. Antiphospholipid antibody syndrome. Rheum Dis Clin North Am. Feb 1994;20(1):45-59. [Medline].

  6. Cimaz R, Descloux E. Pediatric antiphospholipid syndrome. Rheum Dis Clin North Am. Aug 2006;32(3):553-73. [Medline].

  7. Petri M. Epidemiology of the antiphospholipid antibody syndrome. J Autoimmun. Sep 2000;15(2):145-51. [Medline].

  8. Welsch S, Branch DW. Antiphospholipid syndrome in pregnancy. Obstetric concerns and treatment. Rheum Dis Clin North Am. Feb 1997;23(1):71-84. [Medline].

  9. Hansen KE, Kong DF, Moore KD, Ortel TL. Risk factors associated with thrombosis in patients with antiphospholipid antibodies. J Rheumatol. Sep 2001;28(9):2018-24. [Medline].

  10. Harris EN, Pierangeli SS. 'Equivocal' antiphospholipid syndrome. J Autoimmun. Sep 2000;15(2):81-5. [Medline].

  11. Avcin T, Cimaz R, Silverman ED, et al. Pediatric antiphospholipid syndrome: clinical and immunologic features of 121 patients in an international registry. Pediatrics. Nov 2008;122(5):e1100-7. [Medline].

  12. Amigo MC, Khamashta MA. Antiphospholipid (Hughes) syndrome in systemic lupus erythematosus. Rheum Dis Clin North Am. May 2000;26(2):331-48. [Medline].

  13. Wilson WA, Gharavi AE, Koike T, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum. Jul 1999;42(7):1309-11. [Medline].

  14. Wilson WA, Gharavi AE, Piette JC. International classification criteria for antiphospholipid syndrome: synopsis of a post-conference workshop held at the Ninth International (Tours) aPL Symposium. Lupus. 2001;10(7):457-60. [Medline].

  15. Lockshin MD, Sammaritano LR, Schwartzman S. Validation of the Sapporo criteria for antiphospholipid syndrome. Arthritis Rheum. Feb 2000;43(2):440-3. [Medline].

  16. Wilson WA. Classification criteria for antiphospholipid syndrome. Rheum Dis Clin North Am. Aug 2001;27(3):499-505, v. [Medline].

  17. Miyakis S, Lockshin MD, Atsumi T. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. Feb 2006;4(2):295-306. [Medline].

  18. [Guideline] Asherson RA, Cervera R, de Groot PG, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530-4. [Medline].

  19. Cook MC. B cell biology, apoptosis, and autoantibodies to phospholipids. Thromb Res. 2004;114(5-6):307-19. [Medline].

  20. Esmon NL, Safa O, Smirnov MD, Esmon CT. Antiphospholipid antibodies and the protein C pathway. J Autoimmun. Sep 2000;15(2):221-5. [Medline].

  21. Meroni PL, Raschi E, Camera M, et al. Endothelial activation by aPL: a potential pathogenetic mechanism for the clinical manifestations of the syndrome. J Autoimmun. Sep 2000;15(2):237-40. [Medline].

  22. Meroni PL, Raschi E, Testoni C, et al. Antiphospholipid antibodies and the endothelium. Rheum Dis Clin North Am. Aug 2001;27(3):587-602. [Medline].

  23. Rauch J, Subang R, D'Agnillo P, Koh JS, Levine JS. Apoptosis and the antiphospholipid syndrome. J Autoimmun. Sep 2000;15(2):231-5. [Medline].

  24. Angles-Cano E, Guillin MC. Antiphospholipid antibodies and the coagulation cascade. Rheum Dis Clin North Am. Aug 2001;27(3):573-86. [Medline].

  25. Petri M. Pathogenesis and treatment of the antiphospholipid antibody syndrome. Med Clin North Am. Jan 1997;81(1):151-77. [Medline].

  26. Matsuura E, Kobayashi K, Tabuchi M. Accelerated atheroma in the antiphospholipid syndrome. Rheum Dis Clin North Am. Aug 2006;32(3):537-51. [Medline].

  27. Horita T, Atsumi T, Yoshida N, Nakagawa H, Kataoka H, Yasuda S. STAT4 single nucleotide polymorphism, rs7574865 G/T, as a risk for antiphospholipid syndrome. Ann Rheum Dis. Aug 2009;68(8):1366-7. [Medline].

  28. Pierangeli SS, Vega-Ostertag M, Harris EN. Intracellular signaling triggered by antiphospholipid antibodies in platelets and endothelial cells: a pathway to targeted therapies. Thromb Res. 2004;114(5-6):467-76. [Medline].

  29. Meroni PL, Raschi E, Testoni C, Tincani A, Balestrieri G, Molteni R, et al. Statins prevent endothelial cell activation induced by antiphospholipid (anti-beta2-glycoprotein I) antibodies: effect on the proadhesive and proinflammatory phenotype. Arthritis Rheum. Dec 2001;44(12):2870-8. [Medline].

  30. Ferrara DE, Swerlick R, Casper K, Meroni PL, Vega-Ostertag ME, Harris EN, et al. Fluvastatin inhibits up-regulation of tissue factor expression by antiphospholipid antibodies on endothelial cells. J Thromb Haemost. Sep 2004;2(9):1558-63. [Medline].

  31. Ferrara DE, Liu X, Espinola RG, Meroni PL, Abukhalaf I, Harris EN, et al. Inhibition of the thrombogenic and inflammatory properties of antiphospholipid antibodies by fluvastatin in an in vivo animal model. Arthritis Rheum. Nov 2003;48(11):3272-9. [Medline].

  32. López-Pedrera Ch, Buendía P, Aguirre MA, Velasco F, Cuadrado MJ. Antiphospholipid syndrome and tissue factor: a thrombotic couple. Lupus. 2006;15(3):161-6. [Medline].

  33. Redecha P, Franzke CW, Ruf W, Mackman N, Girardi G. Neutrophil activation by the tissue factor/Factor VIIa/PAR2 axis mediates fetal death in a mouse model of antiphospholipid syndrome. J Clin Invest. Oct 2008;118(10):3453-61. [Medline].

  34. Hughson MD, McCarty GA, Brumback RA. Spectrum of vascular pathology affecting patients with the antiphospholipid syndrome. Hum Pathol. Jul 1995;26(7):716-24. [Medline].

  35. Asherson RA, Cervera R, Piette JC. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore). May 1998;77(3):195-207. [Medline].

  36. Asherson RA, Cervera R, Piette JC. Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore). Nov 2001;80(6):355-77. [Medline].

  37. Cervera R, Asherson RA, Font J. Catastrophic antiphospholipid syndrome. Rheum Dis Clin North Am. Aug 2006;32(3):575-90. [Medline].

  38. Cervera R, Bucciarelli S, Plasin MA, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the "CAPS Registry". J Autoimmun. May-Jun 2009;32(3-4):240-5. [Medline].

  39. Asherson RA, Piette JC. The catastrophic antiphospholipid syndrome 1996: acute multi-organ failure associated with antiphospholipid antibodies: a review of 31 patients. Lupus. Oct 1996;5(5):414-7. [Medline].

  40. Rosenzweig EB, Widlitz AC, Barst RJ. Pulmonary arterial hypertension in children. Pediatr Pulmonol. Jul 2004;38(1):2-22. [Medline].

  41. Moroni G, Ventura D, Riva P. Antiphospholipid antibodies are associated with an increased risk for chronic renal insufficiency in patients with lupus nephritis. Am J Kidney Dis. Jan 2004;43(1):28-36. [Medline].

  42. Nochy D, Daugas E, Huong DL, et al. Kidney involvement in the antiphospholipid syndrome. J Autoimmun. Sep 2000;15(2):127-32. [Medline].

  43. Amigo MC. Kidney disease in antiphospholipid syndrome. Rheum Dis Clin North Am. Aug 2006;32(3):509-22. [Medline].

  44. Daugas E, Nochy D, Huong du LT. Antiphospholipid syndrome nephropathy in systemic lupus erythematosus. J Am Soc Nephrol. Jan 2002;13(1):42-52. [Medline].

  45. Garcia-Martin F, De Arriba G, Carrascosa T. Anticardiolipin antibodies and lupus anticoagulant in end-stage renal disease. Nephrol Dial Transplant. 1991;6(8):543-7. [Medline].

  46. Tektonidou MG, Sotsiou F, Nakopoulou L. Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: prevalence, clinical associations, and long-term outcome. Arthritis Rheum. Aug 2004;50(8):2569-79. [Medline].

  47. Roldan CA, Shively BK, Lau CC, Gurule FT, Smith EA, Crawford MH. Systemic lupus erythematosus valve disease by transesophageal echocardiography and the role of antiphospholipid antibodies. J Am Coll Cardiol. Nov 1 1992;20(5):1127-34. [Medline].

  48. Turiel M, Sarzi-Puttini P, Peretti R, et al. Five-year follow-up by transesophageal echocardiographic studies in primary antiphospholipid syndrome. Am J Cardiol. Aug 15 2005;96(4):574-9. [Medline].

  49. Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome. Circulation. Apr 15 1996;93(8):1579-87. [Medline].

  50. Cervera R. Recent advances in antiphospholipid antibody-related valvulopathies. J Autoimmun. Sep 2000;15(2):123-5. [Medline].

  51. Tenedios F, Erkan D, Lockshin MD. Cardiac manifestations in the antiphospholipid syndrome. Rheum Dis Clin North Am. Aug 2006;32(3):491-507. [Medline].

  52. Soltész P, Szekanecz Z, Kiss E, Shoenfeld Y. Cardiac manifestations in antiphospholipid syndrome. Autoimmun Rev. Jun 2007;6(6):379-86. [Medline].

  53. Espinosa G, Cervera R, Font J, Asherson RA. Adrenal involvement in the antiphospholipid syndrome. Lupus. 2003;12(7):569-72. [Medline].

  54. Brey RL. Differential diagnosis of central nervous system manifestations of the antiphospholipid antibody syndrome. J Autoimmun. Sep 2000;15(2):133-8. [Medline].

  55. Frances C, Piette JC. The mystery of Sneddon syndrome: relationship with antiphospholipid syndrome and systemic lupus erythematosus. J Autoimmun. Sep 2000;15(2):139-43. [Medline].

  56. Muscal E, Brey RL. Neurologic manifestations of the antiphospholipid syndrome: integrating molecular and clinical lessons. Curr Rheumatol Rep. Jan 2008;10(1):67-73. [Medline].

  57. Muscal E, Brey RL. Neurological manifestations of the antiphospholipid syndrome: risk assessments and evidence-based medicine. Int J Clin Pract. Sep 2007;61(9):1561-8. [Medline].

  58. Bertolaccini ML, Hughes GR. Antiphospholipid antibody testing: which are most useful for diagnosis?. Rheum Dis Clin North Am. Aug 2006;32(3):455-63. [Medline].

  59. Carreras LO, Forastiero RR, Martinuzzo ME. Which are the best biological markers of the antiphospholipid syndrome?. J Autoimmun. Sep 2000;15(2):163-72. [Medline].

  60. Derksen RH, de Groot PG. Tests for lupus anticoagulant revisited. Thromb Res. 2004;114(5-6):521-6. [Medline].

  61. Exner T. Conceptions and misconceptions in testing for lupus anticoagulants. J Autoimmun. Sep 2000;15(2):179-83. [Medline].

  62. Galli M. Should we include anti-prothrombin antibodies in the screening for the antiphospholipid syndrome?. J Autoimmun. Sep 2000;15(2):101-5. [Medline].

  63. Gilman-Sachs A, Lubinski J, Beer AE, et al. Patterns of anti-phospholipid antibody specificities. J Clin Lab Immunol. Jun 1991;35(2):83-8. [Medline].

  64. Mcintyre JA, Wagenknecht DR. Anti-phosphatidylethanolamine (aPE) antibodies: a survey. J Autoimmun. Sep 2000;15(2):185-93. [Medline].

  65. Merrill JT. Which antiphospholipid antibody tests are most useful?. Rheum Dis Clin North Am. Aug 2001;27(3):525-49. [Medline].

  66. Petri M. Diagnosis of antiphospholipid antibodies. Rheum Dis Clin North Am. May 1994;20(2):443-69. [Medline].

  67. Pierangeli SS, Harris EN. Advances in antiphospholipid antibody testing. Clin Appl Immunol Rev. 2000;1:59-72.

  68. Rapizzi E, Ruffatti A, Tonello M, et al. Correction for age of anticardiolipin antibodies cut-off points. J Clin Lab Anal. 2000;14(3):87-90. [Medline].

  69. Tincani A, Allegri F, Balestrieri G. Minimal requirements for antiphospholipid antibodies ELISAs proposed by the European Forum on antiphospholipid antibodies. Thromb Res. 2004;114(5-6):553-8. [Medline].

  70. Tincani A, Balestrieri G, Allegri F, et al. Overview on anticardiolipin ELISA standardization. J Autoimmun. Sep 2000;15(2):195-7. [Medline].

  71. Triplett DA. Antiphospholipid-protein antibodies: laboratory detection and clinical relevance. Thromb Res. Apr 1 1995;78(1):1-31. [Medline].

  72. Triplett DA. Use of the dilute Russell viper venom time (dRVVT): its importance and pitfalls. J Autoimmun. Sep 2000;15(2):173-8. [Medline].

  73. Wong RC. Consensus guidelines for anticardiolipin antibody testing. Thromb Res. 2004;114(5-6):559-71. [Medline].

  74. Khamashta MA. Primary prevention of thrombosis in subjects with positive antiphospholipid antibodies. J Autoimmun. Sep 2000;15(2):249-53. [Medline].

  75. Khamashta MA, Cuadrado MJ, Mujic F, et al. The management of thrombosis in the antiphospholipid-antibody syndrome. N Engl J Med. Apr 13 1995;332(15):993-7. [Medline].

  76. Petri M. Management of thrombosis in antiphospholipid antibody syndrome. Rheum Dis Clin North Am. Aug 2001;27(3):633-42, viii. [Medline].

  77. Rai R. Obstetric management of antiphospholipid syndrome. J Autoimmun. Sep 2000;15(2):203-7. [Medline].

  78. [Best Evidence] Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. Nov 17 2005;353(20):2148-57. [Medline].

  79. Humpl T, Reyes JT, Holtby H. Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation. Jun 21 2005;111(24):3274-80. [Medline].

  80. Rosenzweig EB, Ivy DD, Widlitz A. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol. Aug 16 2005;46(4):697-704. [Medline].

  81. Derksen RH, de Groot PG. Do we Know which Patients with the Antiphospholipid Syndrome Should Receive Long-term High Dose Anti-coagulation?. J Autoimmun. Sep 2000;15(2):255-259. [Medline].

  82. Wald DS, Bishop L, Wald NJ, et al. Randomized trial of folic acid supplementation and serum homocysteine levels. Arch Intern Med. Mar 12 2001;161(5):695-700. [Medline].

  83. Erkan D, Lockshin MD. New approaches for managing antiphospholipid syndrome. Nat Clin Pract Rheumatol. Mar 2009;5(3):160-70. [Medline].

  84. Lockshin M, Tenedios F, Petri M, McCarty G, Forastiero R, Krilis S. Cardiac disease in the antiphospholipid syndrome: recommendations for treatment. Committee consensus report. Lupus. 2003;12(7):518-23. [Medline].

  85. Levine SR, Brey RL, Tilley BC, et al. Antiphospholipid antibodies and subsequent thrombo-occlusive events in patients with ischemic stroke. JAMA. Feb 4 2004;291(5):576-84. [Medline].

  86. Belizna CC, Richard V, Thuillez C, Levesque H, Shoenfeld Y. Insights into atherosclerosis therapy in antiphospholipid syndrome. Autoimmun Rev. Nov 2007;7(1):46-51. [Medline].

  87. Takemoto CM. Rituximab for ITP: a long-term fix?. Pediatr Blood Cancer. Feb 2009;52(2):155-6. [Medline].

  88. Semple JW. ITP three R's: regulation, routing, rituximab. Blood. Aug 15 2008;112(4):927-8. [Medline].

  89. Marks SD, Patey S, Brogan PA, et al. B lymphocyte depletion therapy in children with refractory systemic lupus erythematosus. Arthritis Rheum. Oct 2005;52(10):3168-74. [Medline].

  90. Asherson RA, Chan JK, Harris EN, et al. Anticardiolipin antibody, recurrent thrombosis, and warfarin withdrawal. Ann Rheum Dis. Dec 1985;44(12):823-5. [Medline].

  91. Lawrie AS, Purdy G, Mackie IJ, Machin SJ. Monitoring of oral anticoagulant therapy in lupus anticoagulant positive patients with the anti-phospholipid syndrome. Br J Haematol. Sep 1997;98(4):887-92. [Medline].

  92. Moll S, Ortel TL. Monitoring warfarin therapy in patients with lupus anticoagulants. Ann Intern Med. Aug 1 1997;127(3):177-85. [Medline].

  93. Pauzner R, Greinacher A, Selleng K, Althaus K, Shenkman B, Seligsohn U. False-positive tests for heparin-induced thrombocytopenia in patients with antiphospholipid syndrome and systemic lupus erythematosus. J Thromb Haemost. Jul 2009;7(7):1070-4. [Medline].

  94. Alarcon-Segovia D, Perez-Ruiz A, Villa AR. Long-term prognosis of antiphospholipid syndrome in patients with systemic lupus erythematosus. J Autoimmun. Sep 2000;15(2):157-61. [Medline].

  95. Amigo MC. Prognosis in antiphospholipid syndrome. Rheum Dis Clin North Am. Aug 2001;27(3):661-9. [Medline].

  96. Erkan D, Asherson RA, Espinosa G, et al. Long term outcome of catastrophic antiphospholipid syndrome survivors. Ann Rheum Dis. Jun 2003;62(6):530-3. [Medline].

Further Reading

Keywords

anti-phospholipid antibody syndrome, Hughes syndrome, Hughes' syndrome, antiphospholipid syndrome, anti-phospholipid syndrome, APS, APLS, Sneddon syndrome, Sneddon's syndrome, thrombosis, primary antiphospholipid syndrome, PAPS, secondary antiphospholipid syndrome, antiphospholipid, aPl, deep venous thrombosis, arterial occlusive events, migraine headache, Raynaud phenomenon, transient ischemic attack, TIA, systemic lupus erythematosus, SLE, lupus anticoagulant, LAC, migraine headache, peripheral vasospasm, thrombocytopenia, anticardiolipin antibody, aCL antibody, cerebrovascular accident, myocardial infarction, endocarditis, pulmonary emboli, treatment, diagnosis

Contributor Information and Disclosures

Author

Barry L Myones, MD, Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital
Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

James M Oleske, MD, MPH, François-Xavier Bagnoud Professor of Pediatrics, Director, Division of Pulmonary, Allergy, Immunology and Infectious Diseases, Department of Pediatrics, New Jersey Medical School
James M Oleske, MD, MPH is a member of the following medical societies: Academy of Medicine of New Jersey, American Academy of Pediatrics, American Public Health Association, American Society for Microbiology, Infectious Diseases Society of America, and Pediatric Infectious Diseases Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

David D Sherry, MD, Director, Clinical Rheumatology, Attending Physician, Pain Management, The Children's Hospital of Philadelphia; Professor of Pediatrics, University of Pennsylvania
David D Sherry, MD is a member of the following medical societies: American College of Rheumatology and American Pain Society
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center
Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.