Differential Diagnoses

Workup

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Asherson RA, Cervera R, Piette JC. Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore). 2001 Nov. 80(6):355-77. [QxMD MEDLINE Link].
Cervera R, Asherson RA, Font J. Catastrophic antiphospholipid syndrome. Rheum Dis Clin North Am. 2006 Aug. 32(3):575-90. [QxMD MEDLINE Link].
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Amigo MC. Kidney disease in antiphospholipid syndrome. Rheum Dis Clin North Am. 2006 Aug. 32(3):509-22. [QxMD MEDLINE Link].
Daugas E, Nochy D, Huong du LT. Antiphospholipid syndrome nephropathy in systemic lupus erythematosus. J Am Soc Nephrol. 2002 Jan. 13(1):42-52. [QxMD MEDLINE Link].
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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. 2004 Aug. 50(8):2569-79. [QxMD MEDLINE Link].
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Cervera R. Recent advances in antiphospholipid antibody-related valvulopathies. J Autoimmun. 2000 Sep. 15(2):123-5. [QxMD MEDLINE Link].
Tenedios F, Erkan D, Lockshin MD. Cardiac manifestations in the antiphospholipid syndrome. Rheum Dis Clin North Am. 2006 Aug. 32(3):491-507. [QxMD MEDLINE Link].
Soltész P, Szekanecz Z, Kiss E, Shoenfeld Y. Cardiac manifestations in antiphospholipid syndrome. Autoimmun Rev. 2007 Jun. 6(6):379-86. [QxMD MEDLINE Link].
Yadav D, Chandra J, Sharma S, Singh V. Essential thrombocytosis and antiphospholipid antibody syndrome causing chronic budd-Chiari syndrome. Indian J Pediatr. 2012 Apr. 79(4):538-40. [QxMD MEDLINE Link].
Espinosa G, Cervera R, Font J, Asherson RA. Adrenal involvement in the antiphospholipid syndrome. Lupus. 2003. 12(7):569-72. [QxMD MEDLINE Link].
Brey RL. Differential diagnosis of central nervous system manifestations of the antiphospholipid antibody syndrome. J Autoimmun. 2000 Sep. 15(2):133-8. [QxMD MEDLINE Link].
Frances C, Piette JC. The mystery of Sneddon syndrome: relationship with antiphospholipid syndrome and systemic lupus erythematosus. J Autoimmun. 2000 Sep. 15(2):139-43. [QxMD MEDLINE Link].
Muscal E, Brey RL. Neurologic manifestations of the antiphospholipid syndrome: integrating molecular and clinical lessons. Curr Rheumatol Rep. 2008 Jan. 10(1):67-73. [QxMD MEDLINE Link].
Muscal E, Brey RL. Neurological manifestations of the antiphospholipid syndrome: risk assessments and evidence-based medicine. Int J Clin Pract. 2007 Sep. 61(9):1561-8. [QxMD MEDLINE Link].
Bertolaccini ML, Hughes GR. Antiphospholipid antibody testing: which are most useful for diagnosis?. Rheum Dis Clin North Am. 2006 Aug. 32(3):455-63. [QxMD MEDLINE Link].
Carreras LO, Forastiero RR, Martinuzzo ME. Which are the best biological markers of the antiphospholipid syndrome?. J Autoimmun. 2000 Sep. 15(2):163-72. [QxMD MEDLINE Link].
Derksen RH, de Groot PG. Tests for lupus anticoagulant revisited. Thromb Res. 2004. 114(5-6):521-6. [QxMD MEDLINE Link].
Exner T. Conceptions and misconceptions in testing for lupus anticoagulants. J Autoimmun. 2000 Sep. 15(2):179-83. [QxMD MEDLINE Link].
Galli M. Should we include anti-prothrombin antibodies in the screening for the antiphospholipid syndrome?. J Autoimmun. 2000 Sep. 15(2):101-5. [QxMD MEDLINE Link].
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Mcintyre JA, Wagenknecht DR. Anti-phosphatidylethanolamine (aPE) antibodies: a survey. J Autoimmun. 2000 Sep. 15(2):185-93. [QxMD MEDLINE Link].
Merrill JT. Which antiphospholipid antibody tests are most useful?. Rheum Dis Clin North Am. 2001 Aug. 27(3):525-49. [QxMD MEDLINE Link].
Petri M. Diagnosis of antiphospholipid antibodies. Rheum Dis Clin North Am. 1994 May. 20(2):443-69. [QxMD MEDLINE Link].
Pierangeli SS, Harris EN. Advances in antiphospholipid antibody testing. Clin Appl Immunol Rev. 2000. 1:59-72.
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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. [QxMD MEDLINE Link].
Tincani A, Balestrieri G, Allegri F, et al. Overview on anticardiolipin ELISA standardization. J Autoimmun. 2000 Sep. 15(2):195-7. [QxMD MEDLINE Link].
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Triplett DA. Use of the dilute Russell viper venom time (dRVVT): its importance and pitfalls. J Autoimmun. 2000 Sep. 15(2):173-8. [QxMD MEDLINE Link].
Wong RC. Consensus guidelines for anticardiolipin antibody testing. Thromb Res. 2004. 114(5-6):559-71. [QxMD MEDLINE Link].
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Khamashta MA. Primary prevention of thrombosis in subjects with positive antiphospholipid antibodies. J Autoimmun. 2000 Sep. 15(2):249-53. [QxMD MEDLINE Link].
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Petri M. Management of thrombosis in antiphospholipid antibody syndrome. Rheum Dis Clin North Am. 2001 Aug. 27(3):633-42, viii. [QxMD MEDLINE Link].
Rai R. Obstetric management of antiphospholipid syndrome. J Autoimmun. 2000 Sep. 15(2):203-7. [QxMD MEDLINE Link].
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Derksen RH, de Groot PG. Do we Know which Patients with the Antiphospholipid Syndrome Should Receive Long-term High Dose Anti-coagulation?. J Autoimmun. 2000 Sep. 15(2):255-259. [QxMD MEDLINE Link].
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Media Gallery

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 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 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 nails of fingers and toes. These may be solitary or multiple and appear intermittently.
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 a 3-year-old child with severe headache and hemiparesis associated with anticardiolipin antibodies.
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 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 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 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 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 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 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, 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.
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 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 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, with evolution and healing of lesions on all other toes.
CAPS, Bone Infarction - MRI (High Resolution Proton Density and STIR images) and Nuclear Bone Scan - Patient 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 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.

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Sections Pediatric Antiphospholipid Antibody Syndrome
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Pediatric Antiphospholipid Antibody Syndrome Differential Diagnoses

Differential Diagnoses

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