Antiphospholipid syndrome natural history, complications and prognosis: Difference between revisions
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==Complications== | ==Complications== | ||
The complications of APS are described in a systemic order which are as follows: | 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 complications:''' | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Feham Tariq, MD [2]
Overview
If left untreated, 90% of patients with antiphospholipid syndrome (APS) progress to develop recurrent thrombotic or thromboembolic events such as pulmonary embolism, Stroke, Transient ischemic attack, Deep vein thrombosis.
Natural History
- If left untreated, 90% of patients with antiphospholipid syndrome (APS) progress to develop recurrent thrombotic or thromboembolic events which include:
- Other patients can develop thrombocytopenia, livedo reticularis, skin ulcers, microangiopathic hemolytic anemia (MAHA).
- Obstetrical complications include premature birth, early pregnancy loss and intrauterine growth restriction.
Complications
The complications of APS are described in a systemic order which are as follows:[1][2][3][4][5] [6][7]
Thrombotic complications:
Thrombotic events are the hallmark of APS. They are as follows:
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
- 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
Cutaneous complications:
Renal 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:
- 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%)
- Malignancy (14%)
- Hemorrhage (11%)
- Systemic lupus erythematosus involvement (8%)
- Catastrophic APS
References
- ↑ Rosove MH, Brewer PM (1992). "Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients". Ann Intern Med. 117 (4): 303–8. PMID 1637025.
- ↑ Finazzi G, Brancaccio V, Moia M, Ciaverella N, Mazzucconi MG, Schinco PC; et al. (1996). "Natural history and risk factors for thrombosis in 360 patients with antiphospholipid antibodies: a four-year prospective study from the Italian Registry". Am J Med. 100 (5): 530–6. PMID 8644765.
- ↑ Moroni G, Ventura D, Riva P, Panzeri P, Quaglini S, Banfi G; et al. (2004). "Antiphospholipid antibodies are associated with an increased risk for chronic renal insufficiency in patients with lupus nephritis". Am J Kidney Dis. 43 (1): 28–36. PMID 14712424.
- ↑ Arnson Y, Shoenfeld Y, Alon E, Amital H (2010). "The antiphospholipid syndrome as a neurological disease". Semin Arthritis Rheum. 40 (2): 97–108. doi:10.1016/j.semarthrit.2009.05.001. PMID 19596138.
- ↑ Zheng H, Chen Y, Ao W, Shen Y, Chen XW, Dai M; et al. (2009). "Antiphospholipid antibody profiles in lupus nephritis with glomerular microthrombosis: a prospective study of 124 cases". Arthritis Res Ther. 11 (3): R93. doi:10.1186/ar2736. PMC 2714149. PMID 19545416.
- ↑ Stratta P, Canavese C, Ferrero S, Grill A, Salomone M, Schinco PC; et al. (1999). "Catastrophic antiphospholipid syndromes in systemic lupus erythematosus". Ren Fail. 21 (1): 49–61. PMID 10048117.
- ↑ Hughson MD, Nadasdy T, McCarty GA, Sholer C, Min KW, Silva F (1992). "Renal thrombotic microangiopathy in patients with systemic lupus erythematosus and the antiphospholipid syndrome". Am J Kidney Dis. 20 (2): 150–8. PMID 1496968.