History

Fact Explanation
Body paralysis [1] Antiphospholipid syndrome is an autoimmune thrombophilia characterized by venous or artery thrombosis [8] caused by antiphospholipid antibodies which provokes thrombosis [2] Ischemic stroke due to thrombus formation can cause body paralysis. [1]
Pregnancy related complications [10] High thrombophilic risk leads to pregnancy related complications such as miscarriages, premature birth [1]
Purpura, skin ulceration [3] Thrombotic events on cutaneous vessels [3]
Chest pain [3] Ischemia due to blocked coronary vessels [3]
Seizures [9] Non thrombotic neurological features [4]
Visual defects [6] Due to thrombosis in retinal artery and vein. [6]
hypertension, diabetes mellitus, obesity, dyslipidemia, smoking, and sedentary lifestyle [7] These comorbidities may act on inflammatory mechanisms and lipid metabolism contributing to the process of vascular injury, which therefore will trigger and propagate the formation of atherosclerotic plaques. It is suspected that this process may be implicated in vascular involvement in patients with antiphospholipid syndrome [7]
References
  1. ETEMADIFAR M, DEHGHANI L, TAHANI S, TOGHIANIFAR N, RAHAIMI M, ESKANDARI N. Neurological manifestations in patients with antiphospholipid syndrome Iran J Neurol [online] 2013, 12(4):172-175 [viewed 20 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3829301
  2. DE GROOT PG. Platelets as pivot in the antiphospholipid syndrome. Blood [online] 2014 Jul 24, 124(4):475-6 [viewed 11 September 2014] Available from: doi:10.1182/blood-2014-06-576983
  3. MERONI P L.,Antiphospholipid Syndrome.American college of rheumatology.2013 [viewed on 18 May 2014].Available from:https://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Antiphospholipid_Syndrome/
  4. DE CARVALHO JF, PASOTO SG, APPENZELLER S. Seizures in Primary Antiphospholipid Syndrome: The Relevance of Smoking to Stroke Clin Dev Immunol [online] 2012:981519 [viewed 20 May 2014] Available from: doi:10.1155/2012/981519
  5. TSIRONI E, GATSELIS N, KOTOULA MG, ZACHOU K, PEFKIANAKI M, ZACHARAKI F, CHATZOULIS DZ, DALEKOS GN. Ocular disorders as the prevailing manifestations of antiphospholipid syndrome: a case series Cases J [online] :159 [viewed 20 May 2014] Available from: www.ncbi.nlm.nih.gov/uniquesig0/pmc/articles/PMC2783115/
  6. CERVERA RICARD, PIETTE JEAN-CHARLES, FONT JOSEP, KHAMASHTA MUNTHER A., SHOENFELD YEHUDA, CAMPS MAR�A TERESA, JACOBSEN SOREN, LAKOS GABRIELLA, TINCANI ANGELA, KONTOPOULOU-GRIVA IRENE, GALEAZZI MAURO, MERONI PIER LUIGI, DERKSEN RONALD H. W. M., DE GROOT PHILIP G., GROMNICA-IHLE ERIKA, BALEVA MARTA, MOSCA MARTA, BOMBARDIERI STEFANO, HOUSSIAU FR�D�RIC, GRIS JEAN-CHRISTOPHE, QU�R� ISABELLE, HACHULLA ERIC, VASCONCELOS CARLOS, ROCH BEATE, FERN�NDEZ-NEBRO ANTONIO, BOFFA MARIE-CLAIRE, HUGHES GRAHAM R. V., INGELMO MIGUEL. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis & Rheumatism [online] December, 46(4):1019-1027 [viewed 11 September 2014] Available from: doi:10.1002/art.10187
  7. DA SILVA FF, LEVY RA, DE CARVALHO JF. Cardiovascular Risk Factors in the Antiphospholipid Syndrome. J Immunol Res [online] 2014:621270 [viewed 11 September 2014] Available from: doi:10.1155/2014/621270
  8. DE CARVALHO JF, LEVY RA, SHOENFELD Y. Antiphospholipid syndrome and antibodies. J Immunol Res [online] 2014:942869 [viewed 20 September 2014] Available from: doi:10.1155/2014/942869
  9. PEIXOTO MV, DE CARVALHO JF, RODRIGUES CE. Clinical, Laboratory, and Therapeutic Analyses of 21 Patients with Neonatal Thrombosis and Antiphospholipid Antibodies: A Literature Review. J Immunol Res [online] 2014:672603 [viewed 20 September 2014] Available from: doi:10.1155/2014/672603
  10. DUSSE LM, DIAS E SILVA F, FREITAS LG, RIOS DR, ARMOND SC, MARCOLINO MS. Antiphospholipid syndrome: a clinical and laboratorial challenge. Rev Assoc Med Bras [online] 2014 Mar-Apr, 60(2):181-6 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24919006

Examination

Fact Explanation
Heart murmurs [1] Patients with anti phospholipid syndrome have a higher chance of valvular lesions [1]
Livedo reticularis [2] Thrombotic events in cutaneous vessels (arterial thrombosis) [3]
Tachypnea [3] Pulmonary embolism- high risk due to thrombophilic state [2]
References
  1. DJOKOVIC A, STOJANOVICH L, KONTIC M, STANISAVLJEVIC N, RADOVANOVIC S, MARISAVLJEVIC D. Association between cardiac manifestations and antiphospholipid antibody type and level in a cohort of Serbian patients with primary and secondary antiphospholipid syndrome. Isr Med Assoc J [online] 2014 Mar, 16(3):162-7 [viewed 20 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24761704
  2. CERVERA RICARD, PIETTE JEAN-CHARLES, FONT JOSEP, KHAMASHTA MUNTHER A., SHOENFELD YEHUDA, CAMPS MAR�A TERESA, JACOBSEN SOREN, LAKOS GABRIELLA, TINCANI ANGELA, KONTOPOULOU-GRIVA IRENE, GALEAZZI MAURO, MERONI PIER LUIGI, DERKSEN RONALD H. W. M., DE GROOT PHILIP G., GROMNICA-IHLE ERIKA, BALEVA MARTA, MOSCA MARTA, BOMBARDIERI STEFANO, HOUSSIAU FR�D�RIC, GRIS JEAN-CHRISTOPHE, QU�R� ISABELLE, HACHULLA ERIC, VASCONCELOS CARLOS, ROCH BEATE, FERN�NDEZ-NEBRO ANTONIO, BOFFA MARIE-CLAIRE, HUGHES GRAHAM R. V., INGELMO MIGUEL. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis & Rheumatism [online] December, 46(4):1019-1027 [viewed 11 September 2014] Available from: doi:10.1002/art.10187
  3. MERONI P L.,Antiphospholipid Syndrome.American college of rheumatology.2013 [viewed on 18 May 2014].Available from:https://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Antiphospholipid_Syndrome/

Differential Diagnoses

Fact Explanation
Syphilis [1] Can be erroneously diagnosed as having APS based on elevated aCL Ab levels [1]
Lyme disease [1] Can be erroneously diagnosed as having APS based on elevated aCL Ab levels [1]
HIV [1] Can be erroneously diagnosed as having APS based on elevated aCL Ab levels [1]
Multiple sclerosis [2] Due to elevated IgM anticardiolipin antibody [2]
References
  1. JACOB H R., WOLGAST L R., Dos and don'ts in diagnosing antiphospholipid syndrome.,American society of hematology education program., 2012 December [viewed 20 May 2014] Available from: http://http://asheducationbook.hematologylibrary.org/content/2012/1/455.long#sec-5
  2. IJDO JW, CONTI-KELLY AM, GRECO P, ABEDI M, AMOS M, PROVENZALE JM, GRECO TP. Anti-phospholipid antibodies in patients with multiple sclerosis and MS-like illnesses: MS or APS? Lupus [online] 1999, 8(2):109-15 [viewed 20 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/10192504

Investigations - for Diagnosis

Fact Explanation
Lupus anticoagulant antibody [1] Together with the history,diagnosed if present or if medium to high levels,in plasma [1]
Anticardiolipin antibodies [2] Together with the history,presence of moderate to high levels of anticardiolipin antibiodies in serum or plasma [1]
References
  1. Diagnosis and Treatment of Antiphospholipid Syndrome.American Family Physician,July 2006 [viewed 18 May 2014].Available from: http://www.aafp.org/afp/2006/0701/p184.html
  2. CERVERA RICARD, PIETTE JEAN-CHARLES, FONT JOSEP, KHAMASHTA MUNTHER A., SHOENFELD YEHUDA, CAMPS MAR�A TERESA, JACOBSEN SOREN, LAKOS GABRIELLA, TINCANI ANGELA, KONTOPOULOU-GRIVA IRENE, GALEAZZI MAURO, MERONI PIER LUIGI, DERKSEN RONALD H. W. M., DE GROOT PHILIP G., GROMNICA-IHLE ERIKA, BALEVA MARTA, MOSCA MARTA, BOMBARDIERI STEFANO, HOUSSIAU FR�D�RIC, GRIS JEAN-CHRISTOPHE, QU�R� ISABELLE, HACHULLA ERIC, VASCONCELOS CARLOS, ROCH BEATE, FERN�NDEZ-NEBRO ANTONIO, BOFFA MARIE-CLAIRE, HUGHES GRAHAM R. V., INGELMO MIGUEL. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis & Rheumatism [online] December, 46(4):1019-1027 [viewed 11 September 2014] Available from: doi:10.1002/art.10187

Investigations - Fitness for Management

Fact Explanation
Full blood count [1] To assess the general fitness and for the correcion of other blood abnormalities (eg: hemoglobin levels) [1]
Clotting profile [1] To detect thrombotic abnormalities [1]
Fasting blood sugar,lipid profile [1] For the correction of conventional risk factors for thrombosis (diabetes, high blood pressure, high cholesterol, obesity, and smoking) [2]
References
  1. MERONI P L.,Antiphospholipid Syndrome.American college of rheumatology.2013 [viewed on 18 May 2014].Available from:https://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Antiphospholipid_Syndrome/
  2. DA SILVA FF, LEVY RA, DE CARVALHO JF. Cardiovascular Risk Factors in the Antiphospholipid Syndrome. J Immunol Res [online] 2014:621270 [viewed 11 September 2014] Available from: doi:10.1155/2014/621270

Investigations - Followup

Fact Explanation
transthoracic echocardiogram [1] To identify heart valve lesions as patients with Antiphospholipid syndrome are more prone to have valve lesions [1]
References
  1. CERVERA RICARD, PIETTE JEAN-CHARLES, FONT JOSEP, KHAMASHTA MUNTHER A., SHOENFELD YEHUDA, CAMPS MAR�A TERESA, JACOBSEN SOREN, LAKOS GABRIELLA, TINCANI ANGELA, KONTOPOULOU-GRIVA IRENE, GALEAZZI MAURO, MERONI PIER LUIGI, DERKSEN RONALD H. W. M., DE GROOT PHILIP G., GROMNICA-IHLE ERIKA, BALEVA MARTA, MOSCA MARTA, BOMBARDIERI STEFANO, HOUSSIAU FR�D�RIC, GRIS JEAN-CHRISTOPHE, QU�R� ISABELLE, HACHULLA ERIC, VASCONCELOS CARLOS, ROCH BEATE, FERN�NDEZ-NEBRO ANTONIO, BOFFA MARIE-CLAIRE, HUGHES GRAHAM R. V., INGELMO MIGUEL. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis & Rheumatism [online] December, 46(4):1019-1027 [viewed 11 September 2014] Available from: doi:10.1002/art.10187

Investigations - Screening/Staging

Fact Explanation
Anti- beta2GPI antibodies [1] High prevalence of IgA-β2GPI antibodies in patients with antiphospholipid syndrome [2]
aPTT [3] Detection of thrombotic risk [3] frequently used as the initial screening test [4]
Anti cardiolipin antibody [3] Correlation between higher antibody titers and antiphospholipid syndrome [3]
Doppler studies [5] To identify deep venous thrombosis, as these patients have a higher risk due to thrombophilic state [5]
Noncontrast head computed tomography (CT) [6] To identify ischemic changes caused by thrombi [6]
References
  1. ANIS S, AHMED E, MUZAFFAR R. Prevalence of anti- beta2GPI antibodies and their isotypes in patients with renal diseases and clinical suspicion of antiphospholipid syndrome J Nephropathol [online] 2013 Jul, 2(3):181-189 [viewed 20 May 2014] Available from: www.ncbi.nlm.nih.gov/uniquesig0/pmc/articles/PMC3891139/
  2. NAKASHIMA MO, ROGERS HJ. Hypercoagulable states: an algorithmic approach to laboratory testing and update on monitoring of direct oral anticoagulants. Blood Res [online] 2014 Jun, 49(2):85-94 [viewed 11 September 2014] Available from: doi:10.5045/br.2014.49.2.85
  3. JACOB H R., WOLGAST L R., Dos and don'ts in diagnosing antiphospholipid syndrome.,American society of hematology education program., 2012 December [viewed 20 May 2014] Available from: http://asheducationbook.hematologylibrary.org/content/2012/1/455.long#sec-5
  4. GREAVES M., COHEN H., MACHIN S. J., MACKIE I.. Guidelines On The Investigation and Management Of The Antiphospholipid Syndrome. Br J Haematol [online] 2000 June, 109(4):704-715 [viewed 11 September 2014] Available from: doi:10.1046/j.1365-2141.2000.02069.x
  5. CERVERA RICARD, PIETTE JEAN-CHARLES, FONT JOSEP, KHAMASHTA MUNTHER A., SHOENFELD YEHUDA, CAMPS MAR�A TERESA, JACOBSEN SOREN, LAKOS GABRIELLA, TINCANI ANGELA, KONTOPOULOU-GRIVA IRENE, GALEAZZI MAURO, MERONI PIER LUIGI, DERKSEN RONALD H. W. M., DE GROOT PHILIP G., GROMNICA-IHLE ERIKA, BALEVA MARTA, MOSCA MARTA, BOMBARDIERI STEFANO, HOUSSIAU FR�D�RIC, GRIS JEAN-CHRISTOPHE, QU�R� ISABELLE, HACHULLA ERIC, VASCONCELOS CARLOS, ROCH BEATE, FERN�NDEZ-NEBRO ANTONIO, BOFFA MARIE-CLAIRE, HUGHES GRAHAM R. V., INGELMO MIGUEL. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis & Rheumatism [online] December, 46(4):1019-1027 [viewed 11 September 2014] Available from: doi:10.1002/art.10187
  6. SAAD AF, NICKELL LT, HEITHAUS RE, SHAMIM SA, OPATOWSKY MJ, LAYTON KF. Basilar artery thrombosis in the setting of antiphospholipid syndrome. Proc (Bayl Univ Med Cent) [online] 2014 Jul, 27(3):210-2 [viewed 11 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24982561

Management - General Measures

Fact Explanation
Avoid smoking,take regular physical exercise, maintain a healthy diet and avoid overweight/obesity,avoid excessive alcohol intake. [1] Healthy lifestyle in line with prevention of cardiovascular disease [1]
Adequate management of cardiovascular risk factors, including diabetes, hypertension and hyperlipidaemia [1] To control the other co morbidities [1]
Avoid other factors which contribute to increase the thrombophilic risk [1] Eg:oral contraceptives increase the thrombophilic risk [1]
References
  1. GóMEZ-PUERTA JA, MARTíN H, AMIGO MC, AGUIRRE MA, CAMPS MT, CUADRADO MJ, HUGHES GR, KHAMASHTA MA. Long-term follow-up in 128 patients with primary antiphospholipid syndrome: do they develop lupus? Medicine (Baltimore) [online] 2005 Jul, 84(4):225-30 [viewed 20 May 2014] Available from: sp-3.12.0b/ovidweb.cgi?T=JS&PAGE=fulltext&D=ovft&AN=00005792-200507000-00004&NEWS=N&CSC=Y&CHANNEL=PubMed

Management - Specific Treatments

Fact Explanation
Medical therapy for vascular events [1] Acute thrombotic events are treated with anticoagulants (blood thinners), initially with intravenous heparin and then followed by oral warfarin [1] oral anticoagulants (producing an international normalized ratio of >3) with or without low-dose aspirin are an effective prophylaxis against both venous and arterial thrombosis in most patients. cessation of warfarin therapy in patients with antiphospholipid-antibody–associated thromboses carries a high risk of recurrent thrombosis. Aspirin is widely used as an antiplatelet agent. [2]
Medical therapy for obstetrical manifestations [1] Subcutaneous injections of heparin and low-dose aspirin are the standard therapy for preventing miscarriages. The therapy is started at the beginning of the pregnancy and continued in the period immediately after the delivery. [1] Treatment of antiphospholipid syndrome during pregnancy reduces the risks of pregnancy loss, preeclampsia, placental insufficiency, preterm birth, and thrombosis. [3] Low molecular weight heparin can be used as early as six weeks’ gestation until 34–36 weeks’ gestation [4]
References
  1. MERONI P L.,Antiphospholipid Syndrome.American college of rheumatology.2013 [viewed on 18 May 2014].Available from:https://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Antiphospholipid_Syndrome/
  2. KHAMASHTA MUNTHER A., CUADRADO MARIA JOSE, MUJIC FEDZA, TAUB NICK A., HUNT BEVERLEY J., HUGHES GRAHAM R.V.. The Management of Thrombosis in the Antiphospholipid-Antibody Syndrome. N Engl J Med [online] 1995 April, 332(15):993-997 [viewed 11 September 2014] Available from: doi:10.1056/NEJM199504133321504
  3. Diagnosis and Treatment of Antiphospholipid Syndrome.American Family Physician,July 2006 [viewed 18 May 2014].Available from: http://www.aafp.org/afp/2006/0701/p184.html
  4. ABU-HEIJA A. Thrombophilia and Recurrent Pregnancy Loss: Is heparin still the drug of choice? Sultan Qaboos Univ Med J [online] 2014 Feb, 14(1):e26-36 [viewed 11 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24516750