Title: Antiphospholipid syndrome: using anticoagulants properly:

clotting from APS can have a variety of serious health

consequences
Author(s):Jeannette Y. Wick
Source:Pharmacy Times. 79.2 (Feb. 2013): p70. From InfoTrac Custom 100 Titles.
Document Type:Article
Copyright:COPYRIGHT 2013 Pharmacy & Healthcare Communications, LLC
http://www.pharmacytimes.com/
Full Text:
[ILLUSTRATION OMITTED]
The autoimmune disorder antiphospholipid syndrome (APS) earns its name from its laboratory
protile, but in a complicated way: over time, we've learned
that antiphospholipid (aPL) antibodies are not the best or only markers for this syndrome.
When APS was first described in the 1980s, researchers linked aPL antibodies to APS, but
struggled to determine whether they were causal or coincidental to another problem. Among
healthy Americans who do not have APS, 1% to 5% have elevated aPL antibodies. This means
elevated aPL alone is not diagnostic--that is, aPL can be elevated with no clinical repercussions.
Further, among systemic lupus erythematosus (SLE) patients, 30% to 40% of patients have
aPL antibodies, but only approximately 10% have APS. In patients with APS, researchers later
found that antibodies directed against membrane anionic phospholipids--anticardiolipin
[aCL] antibody, lupus anticoagulant (aL), or associated plasma proteins (usually beta-2
glycoprotein 1)--are persistently elevated. But, the presence of aCL antibodies increases with
age, often with no clinical reper cussions. (1) A true APS diagnosis requires aberrant lab work on
2 occasions more than 12 weeks apart plus 1 or more episodes of vascular thrombosis or
obstetrical complication. (2)
What Is APS?
APS alters the homeostatic regulation of blood coagulation, and researchers have yet to
determine why. Its hallmark signs are recurrent venous or arterial thrombosis and/or fetal
miscarriage. Approximately half of patients simply develop APS alone, with the rest developing
APS secondary to SLE or another rheumatic or autoimmune disorder. Today's preferred jargon is
APS with or without associated rheumatic disease, (3) discarding the older "'primary" and
"secondary'" terms to avoid implying that one condition is different from or subordinate to the
other.
APS's clinical manifestations can be widespread, as hypercoagulability and recurrent thrombosis
have the potential to affect multiple organs. Most often APS patients develop deep venous
thrombosis (DVT) and pulmonary embolism. APS patients may also develop other serious health
consequences (Table 1). Patients who suffer ischemic stroke at an inordinately young age should
be tested for APS, as stroke is likely to occur without adequate prophylaxis. (1,4)
Obstetric patients with APS may deliver preterm or develop eclampsia. Spontaneous abortion
may be due to thrombosis in the placental bed, recurrent early fetal loss (before 10 weeks'
gestation), or fetal loss in the second or third trimesters. Clinicians should screen women who
have had more than 3 miscarriages before 10 weeks' gestation for APS. Pregnancy itself can
interfere with aPL testing; ideally, clinicians should test when the woman is not pregnant. (1)
Rarely, patients may present with catastrophic APS (CAPS), which is characterized by multiorgan
infarc tions over a period of days to weeks. Approximately one-third of patients with CAPS die.
(5)
Planning Treatment: Dose and Duration
Clinicians must immediately follow an APS diagnosis with individualized treatment planning
based on the patient's unique clinical status and thrombotic event history. Most interventions are
similar to those used for patients who have non-APS variants of hypercoagulability. The clinical
conundrums are, too. Most experts still debate whether high-dose, aggressive drug treatment is
better than low-dose treatment. Studies using traditional treatments--anticoagulants and
steroids--are under way now to find answers. Treatment duration is also largely empiric.
Prevention is key, and patients must avoid oral contraceptives and quit smoking. Treating
hypertension or hyperlipidemia is important, as these are risk factors for clotting. Although
clinicians often prescribe low-dose aspirin as prophylaxis, like most other treatments, its
effectiveness is under review.
All treatment should commence with a frank discussion of APS's risks and benefits, and the need
for adherence and monitoring, if these are necessary. If an acute thrombotic event occurs,
patients will need full anticoagulation with intravenous or subcutaneous heparin followed by
warfarin therapy. When warfarin is used, clinicians aim for an international normalized ratio
(INR) of 2.0 to 3.0 for venous thrombosis and 3.0 for arterial thrombosis. If thrombotic events
recur or are significant, the patient may need lifelong prophylaxis. (1,3,6) Corticosteroids are
not needed for patients with uncomplicated APS.
Complicated APS
Patients who experience recurrent DVT will definitely need lifelong anticoagulation and may need
surgery to place an inferior vena cava filter.
Treatment of the pregnant APS patient is controversial, because so many clinicians and pregnant
patients prefer to avoid all medication during pregnancy. The American College of Obstetricians
and Gynecologists guidelines recommend prophylaxis for pregnant women with no history of
thrombosis and full anticoagulation for those with a history of thrombosis. (7,8)
Low-dose aspirin may be sufficient to prevent miscarriage in women with uncomplicated
histories. (9) Warfarin is contraindicated in pregnancy, although it can be taken during breast-
feeding. Women who have had previous APS-related pregnancy losses should receive
prophylactic subcutaneous heparin (preferably low-molecular-weight heparin) and low-dose
aspirin. Around the time of delivery, all medications should be discontinued, then restarted after
delivery and continued for 6 to 12 weeks postpartum. Women who have had previous
thrombosis should receive therapeutic heparin doses throughout gestation and long term
anticoagulation postpartum. Corticosteroids may increase maternal morbidity and fetal
prematurity rates. Administering heparin or corticosteroids long-term may cause osteoporosis,
so the guidelines recommend calcium and vitamin D supplements. (6,9,10)
Patients who have comorbid SLE present challenges, especially if they have active or end-stage
disease. Lupus anticoagulants may react with reagents used to perform the INR, complicating
monitoring. (11) SLE patients who have a positive aPL may respond to hydroxychloroquine's
antithrombotic properties as prophylaxis.
Patients who develop CAPS need intensive hospital-based care. Aggressive anticoagulation,
plasma exchange, and corticosteroids are standard interventions, but no controlled trials
demonstrate clear benefit. Intravenous immunoglobulin may be of some benefit and
cyclophosphamide is sometimes administered, especially in SLE-associated CAPS.m Rituximab
has shown promise in the treatment of APS, and clinical trials are under way. (12,13)
Conclusion
Research continues to find better agents. Our current treatments address symptoms and
damages caused by thromboembolic events. APS is a "systemic" disease, and as we dismantle
and understand the molecular mechanisms of aPL-mediated damage, we may find an
immunomodulatory approach (Table 2). (14) Until then, we must use traditional agents
responsibly, and counsel patients to remain vigilant and aware of signs of developing clots.
Jeannette Y. Wick, RPh, MBA, FASCP
Ms. Wick is a visiting professor at the University of Connecticut School of Pharmacy and a
freelance clinical writer.
TABLE 1: POSSIBLE CONSEQUENCES OF AFS

Cardiovascular Cerebrovascular accident, sinus
thrombosis, cardiac valvulopathy,
myocardial infarction

Dermatologic Livedo reticularis, purpura,
infarcts/ulceration

Hematologic Thrombocytopenia, hemolytic anemia,
arterial or venous thrombo embolic
disease

Musculoskeletal Avascular necrosis of bone and
other infarctions or hemorrhage

Neurologic Stroke, transient ischemic attacks,
cognitive dysfunction

Obstetric Spontaneous abortion, preterm
delivery (before 34 weeks), pre
eclampsia, eclampsia, or placental
insufficiency

Ophthalmic Ocular thrombosis

Pulmonary Embolism, pulmonary hypertension

APS = antisphospholipid syndrome.

Adapted from references 2, 4.

TABLE 2: INVESTIGATIONAL AGENTS FOR APS

Agent Proposed Mechanism

Hydroxycloroquine Prevent the aPL-induced
displacement of annexin A5 from
phos pholipid bilayers and inhibit
aPL-mediated thrombosis

Statins Reverse aPL-induced endothelial
cell activation and TF up
regulation

Rituximab Blocks B-cell activating factor

Clopidogrel Antiplatelet effect

Anticoagulation with predictable
anticoagulant response and little
potential for food or drug
interactions

aPL = antiphospholipid; APS = antisphospholipid syndrome;
TF = tissue factor.

Adapted from references 15-20.
Wick, Jeannette Y.
Source Citation (MLA 7
th
Edition)
Wick, Jeannette Y. "Antiphospholipid syndrome: using anticoagulants properly: clotting from APS can have a
variety of serious health consequences."Pharmacy Times Feb. 2013: 70+. InfoTrac Custom 100 Titles. Web. 19
June 2014.
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