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.
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
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.