View source for Antiphospholipid syndrome natural history, complications and prognosis

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{{Antiphospholipid syndrome}}
{{CMG}}{{AE}}{{FT}}

==Overview==

If left untreated, 90% of patients with antiphospholipid syndrome (APS) progress to develop recurrent [[Thrombosis|thrombotic]] or [[Thrombosis|thromboembolic]] events such as pulmonary [[embolism]], [[Stroke]], [[Transient ischemic attack]], [[Deep vein thrombosis]]. The complications caused by APS are mainly thrombotic, neurological, obstetrical, pulmonary and ocular. APS is associated with increased morbidity and mortality. The mean age of death is 59 years.

==Natural History==
*If left untreated, 90% of patients with antiphospholipid syndrome (APS) progress to develop recurrent [[Thrombosis|thrombotic]] or [[Thrombosis|thromboembolic]] events which include:
** Pulmonary [[embolism]]
** [[Stroke]]
** [[Transient ischemic attack]]
** [[Deep vein thrombosis]]
* Other patients can develop [[thrombocytopenia]], [[Livedoid vasculitis|livedo reticularis]], skin [[Ulcer|ulcers]], [[microangiopathic hemolytic anemia]] (MAHA).
* Obstetrical complications include premature birth, early pregnancy loss and [[Intrauterine growth retardation|intrauterine growth restriction]].

==Complications==
The complications of APS are described in a systemic order which are as follows:<ref name="pmid1637025">{{cite journal| author=Rosove MH, Brewer PM| title=Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients. | journal=Ann Intern Med | year= 1992 | volume= 117 | issue= 4 | pages= 303-8 | pmid=1637025 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1637025  }} </ref><ref name="pmid8644765">{{cite journal| author=Finazzi G, Brancaccio V, Moia M, Ciaverella N, Mazzucconi MG, Schinco PC et al.| title=Natural history and risk factors for thrombosis in 360 patients with antiphospholipid antibodies: a four-year prospective study from the Italian Registry. | journal=Am J Med | year= 1996 | volume= 100 | issue= 5 | pages= 530-6 | pmid=8644765 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8644765  }} </ref><ref name="pmid14712424">{{cite journal| author=Moroni G, Ventura D, Riva P, Panzeri P, Quaglini S, Banfi G et al.| title=Antiphospholipid antibodies are associated with an increased risk for chronic renal insufficiency in patients with lupus nephritis. | journal=Am J Kidney Dis | year= 2004 | volume= 43 | issue= 1 | pages= 28-36 | pmid=14712424 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14712424  }} </ref><ref name="pmid19596138">{{cite journal| author=Arnson Y, Shoenfeld Y, Alon E, Amital H| title=The antiphospholipid syndrome as a neurological disease. | journal=Semin Arthritis Rheum | year= 2010 | volume= 40 | issue= 2 | pages= 97-108 | pmid=19596138 | doi=10.1016/j.semarthrit.2009.05.001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19596138  }} </ref><ref name="pmid19545416">{{cite journal| author=Zheng H, Chen Y, Ao W, Shen Y, Chen XW, Dai M et al.| title=Antiphospholipid antibody profiles in lupus nephritis with glomerular microthrombosis: a prospective study of 124 cases. | journal=Arthritis Res Ther | year= 2009 | volume= 11 | issue= 3 | pages= R93 | pmid=19545416 | doi=10.1186/ar2736 | pmc=2714149 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19545416  }} </ref><ref name="pmid10048117">{{cite journal| author=Stratta P, Canavese C, Ferrero S, Grill A, Salomone M, Schinco PC et al.| title=Catastrophic antiphospholipid syndromes in systemic lupus erythematosus. | journal=Ren Fail | year= 1999 | volume= 21 | issue= 1 | pages= 49-61 | pmid=10048117 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10048117  }} </ref><ref name="pmid1496968">{{cite journal| author=Hughson MD, Nadasdy T, McCarty GA, Sholer C, Min KW, Silva F| title=Renal thrombotic microangiopathy in patients with systemic lupus erythematosus and the antiphospholipid syndrome. | journal=Am J Kidney Dis | year= 1992 | volume= 20 | issue= 2 | pages= 150-8 | pmid=1496968 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1496968  }} </ref>

'''Thrombotic complications:'''

Thrombotic events are the hallmark of APS. They are as follows:
* Venous [[thrombosis]] is more common than arterial thrombosis. The major sites of involvement are [[Lung|pulmonary]], pelvic, renal, hepatic, portal, [[axillary]], [[subclavian]], [[ocular]], and [[Brain|cerebral]] sinuses, as well as the inferior vena cava.
'''Neurological complications:'''

Following complications are seen on neurological exam:
* [[Stroke]]
* [[Transient ischemic attack]]
'''Obstetrical complications:'''

The pregnancy related complications are as follows:
*Maternal [[thrombosis]]
*Fetal loss
*Pre-[[eclampsia]]
*Placental insufficiency
*Fetal growth restriction
*Iatrogenic preterm birth

'''Pulmonary complications:'''

Thromboembolic and non-thromboembolic complications include:
**Diffuse alveolar hemorrhage 
**Pulmonary arterial hypertension
**Acute respiratory distress syndrome ([[Acute respiratory distress syndrome|ARDS]])
**Pulmonary micrthrombosis

'''Hematological complications:'''
**[[Autoimmune hemolytic anemia]]
**[[Thrombocytopenia]]
**Bone marrow necrosis

'''Cardiac involvement:'''
**Valvular thickening and valve nodules also called as Libmann-Sacks endocarditis.
**Coronary artery disease

'''Adrenal involvement:'''
* Hemorrhagic infarction of the adrenals
'''Ocular manifestations:'''

The ocular manifestations are the following:
* [[Anterior ischemic optic neuropathy]]
* [[Amaurosis fugax]]
* Retinal venous occlusion 
'''Gastrointestinal complications:'''

Ischemia of the gastrointestinal tract leads to the following complications:
* Gastrointestinal bleeding
* Abdominal pain 
* Esophageal [[necrosis]] with perforation
* Duodenal ulcer

==Prognosis==
The prognosis of antiphospholipid syndrome is as follows:<ref name="pmid24464962">{{cite journal| author=Cervera R, Serrano R, Pons-Estel GJ, Ceberio-Hualde L, Shoenfeld Y, de Ramón E et al.| title=Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. | journal=Ann Rheum Dis | year= 2015 | volume= 74 | issue= 6 | pages= 1011-8 | pmid=24464962 | doi=10.1136/annrheumdis-2013-204838 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24464962  }} </ref><ref name="pmid18801761">{{cite journal| author=Cervera R, Khamashta MA, Shoenfeld Y, Camps MT, Jacobsen S, Kiss E et al.| title=Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. | journal=Ann Rheum Dis | year= 2009 | volume= 68 | issue= 9 | pages= 1428-32 | pmid=18801761 | doi=10.1136/ard.2008.093179 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18801761  }} </ref>
*It is associated with increased mortality and morbidity.
*The mean age of death is 59 years.
*Main causes of death in the order of percentage include the following:
**[[Thrombosis]] (31%)
**[[Sepsis]] (27%)
**[[Cancer|Malignancy]] (14%)
**[[Bleeding|Hemorrhage]] (11%)
**Systemic lupus erythematosus involvement (8%)
**Catastrophic APS

==References==
{{reflist|2}}

[[Category:Disease]]
[[Category:Rheumatology]]
[[Category:Hematology]]
[[Category:Autoimmune diseases]]
[[Category:Neurology]]
[[Category:Obstetrics]]
[[Category:Syndromes]]