Antiphospholipid syndrome natural history, complications and prognosis
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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. 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 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:
- Venous thrombosis is more common than arterial thrombosis. The major sites of involvement are pulmonary, pelvic, renal, hepatic, portal, axillary, subclavian, ocular, and cerebral sinuses, as well as the inferior vena cava.
Neurological complications:
Following complications are seen on neurological exam:
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 (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:[8][9]
- 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.
- ↑ Cervera R, Serrano R, Pons-Estel GJ, Ceberio-Hualde L, Shoenfeld Y, de Ramón E; et al. (2015). "Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients". Ann Rheum Dis. 74 (6): 1011–8. doi:10.1136/annrheumdis-2013-204838. PMID 24464962.
- ↑ Cervera R, Khamashta MA, Shoenfeld Y, Camps MT, Jacobsen S, Kiss E; et al. (2009). "Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients". Ann Rheum Dis. 68 (9): 1428–32. doi:10.1136/ard.2008.093179. PMID 18801761.