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I agree with Hughes and Khamashta that the use of the term “seronegative antiphospholipid syndrome (APS)” is useful in clinical practice.1 However, the analogy with seronegative rheumatoid arthritis and antinuclear antibody (ANA) negative lupus is not correct. The current criteria for the classification of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) allow the diagnosis of RA or SLE to be made even if the rheumatoid factor or the ANA is negative, and therefore, seronegative RA and ANA negative SLE are embraced within the classification criteria and as such are not separate entities.2–4
In the case of APS (Hughes’ syndrome), the current preliminary classification criteria do not allow a diagnosis of APS to be made in the absence of at least two positive tests for either anticardiolipin antibodies or lupus anticoagulant at least 6 weeks apart.5 A revised international consensus statement on classification criteria for APS (Hughes’ syndrome) is required to accommodate the seronegative clinical entity.
We take Dr Jawad’s points and agree fully. We also believe that classification criteria are too often wrongly used in diagnosis. Our aim in writing the leader1 was to highlight what we believe to be a not uncommon diagnostic situation—the patient with many of the features of the syndrome in whom tests remain stubbornly negative.