Doctor, Do I Have Lupus? Time to Reconsider the Lupus Anticoagulant Terminology

Hematologists deal with patients diagnosed with lupus anticoagulant (LA) on a regular basis, especially considering the common laboratory finding of LA. Broadly, such patients typically fall into two categories—first group comprises those diagnosed with LA from a prolonged clotting screen and the second group comprises those who had the laboratory tests performed to diagnose or exclude a clinical condition called antiphospholipid syndrome (APS). During the subsequent consultation, when the patient is informed of the positive LA result, the common response is “Doctor, do I have lupus?”

Lupus is more widely used to signify the rheumatologic condition, systemic lupus erythematosus (SLE), and was known from the time of Hippocrates. It is believed that the bishop of Tours, Herbernus, was the first to use the term “lupus” to describe the dermatological manifestations of this autoimmune condition.[1] Apparently, the word “lupus” (Latin for “wolf”) describes erosive facial lesions of a wolf's bite.[1] Although lupus continues to be the commonly accepted abbreviated term for SLE, it rarely describes the clinical manifestations associated with the identification of LA, except for the patients with SLE ([Fig. 1]).

Fig. 1 Proposed algorithm for lupus anticoagulant testing. The three main tests for APS are “lupus anticoagulant,” antibodies to cardiolipin (“anticardiolipin”), and antibodies to beta-2 glycoprotein I (“anti-beta-2 glycoprotein I”). APS, antiphospholipid syndrome; PT, prothrombin time; APTT, activated partial thromboplastin time.

Several publications have described the history of LA including this journal.[2] [3] [4] In short, the in vitro “anticoagulant” phenomenon was noted in some patients with SLE, but these cases were very rarely associated with bleeding.[2] [3] [4] On the contrary, the clinical manifestation was thrombosis in the arterial and venous circulation in patients with SLE who had LA positivity.[5] Furthermore, the same laboratory abnormality was noted in patients with no clinical or laboratory evidence of SLE. Thus, LA is neither lupus associated nor an anticoagulant. In this context, we have to consider whether it is right to continue to tell our patients that they have “lupus anticoagulant.” If we do tell them so, do we openly admit that “I am sorry to say that this term “lupus anticoagulant” is actually misnomer? You don't really have lupus (SLE) and you are not going to bleed in most situations.” Or should we reconsider the LA terminology, perhaps especially relevant for the post-COVID era?

Some of the easy and immediate solutions for the new era in this clinical context are as follows:

• Firstly, indiscriminate testing for LA must be discouraged. It should never be considered part of routine testing. In the author's institution, measures are being put in place to ensure that LA testing is requested only by rheumatologists or hematologists or senior obstetricians. In all other cases, a discussion is necessary with the hematologist prior to the test-ordering. This will ensure correct timing of testing and, importantly, that the test is repeated at the currently stipulated 12 weeks, according to the revised Sydney criteria.[6]

• LA testing should be performed only if the patient is suspected to have APS. One of the most recent suggestions is testing for LA in patients with noncriteria manifestations of APS as well.[7]

• It is important that the testing is undertaken after being made aware of the various problems with laboratory testing associated with LA, which can cause false positives and false negatives.[8] [9]

• One of the most common reasons for testing LA is following an abnormal coagulation screen including during preoperative screening to assess bleeding risk.[10] [11] Identification of LA positivity in this setting is fraught with issues, as there are no clear guidelines on what LA positivity in the absence of symptoms means. Many patients may start getting worried about lupus (as described earlier), which can create unnecessary psychological concerns. Such patients may even begin to experience nonobjective symptoms of SLE like arthralgia. In addition, some physicians may consider “mild” antithrombotics (low-dose aspirin and perioperative thromboprophylaxis) in these patients due to the fear of thrombosis from LA positivity which has no evidence base. Delay in surgery due to the prolongation of clotting screen is another unintended consequence of using LA-sensitive reagents.

• As with any other measures of improving healthcare, education and regular updates of physicians about the appropriateness of LA testing and its relevance should be undertaken.

Finally, coming to the issue of LA terminology, since it continues to create confusion among both patients and nonspecialist clinicians and, occasionally, the laboratory staff, it may be worthwhile reconsidering the LA moniker. Would antiphospholipid screen be a better term? Could this reduce the number of inappropriate requests by considering the test only in patients suspected to have APS? In the author's opinion, “LA” testing outside the setting of APS should be done only by specialists to exclude an inhibitor in patients suspected to have an acquired deficiency of coagulation factors, especially if LA-insensitive reagents are used for coagulation screens.

Publication History

Article published online:
01 May 2021

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