Background/Purpose: The relevance of non-criteria clinical manifestations of antiphospholipid syndrome (APS) has been less studied than its thrombotic and obstetric features. The aim of this study was to evaluate the relationship of APS non-criteria clinical manifestations with antiphospholipid antibodies and damage accrual.

Methods: We retrospectively included 176 patients with APS according to Sydney or Alarcon-Segovia’s criteria. We registered demographics, antiphospholipid antibody profile, use of prednisone and immunosuppresors, thrombotic and obstetric manifestations as well as the following so-called non-criteria clinical manifestations: cutaneous (livedo reticularis, skin ulceration,) hematological (thrombocytopenia, hemolytic anemia), renal (microangiopathy, proteinuria or renal impairment), heart valve disease, and neurological (chorea, migraine, seizures and myelitis). We scored a modified SLICC index where we also included the variables racemous livedo, adrenal insufficiency, placing of Greenfield filter and sclerosis multiple-like disease. We used descriptive statistics and logistic regression and reported OR with 95% CI.

Results: Seventy-eight percent of our patients were women with a mean age of 43.1 ± 14.7 years and median follow-up of 6.7 years. We observed thrombosis in 73%, obstetric features in 20% and 64% had non-criteria clinical manifestations (not exclusive groups). 73 patients (41.4%) had both thrombosis and a non-criteria clinical manifestation (10 concomitant, 42 post-thrombotic and 21 pre-thrombosis).The frequency of the non-criteria clinical manifestation were: hematological 64%, cutaneous 33%, neurological 27%, cardiological 8.2% and renal 6.4%. The univariate analysis showed that the prevalence of LA was higher in the non-criteria clinical manifestation group  (53.2% vs. 69.5%, p=0.03). This group also used more prednisone (35.8% vs.1%, <0.0001) and immunosupressors (48.6% vs. 0%, p <0.0001) and had a higher SLICC modified score SLICC (1.69±1.9 vs. 1.1±1.5, p=0.04). Most of the weight of the SLICC score was attributed to the neurologic, pulmonar and peripheral vascular domains. The variables associated with a modified SLICC ≥ 1 were thrombosis (OR 11.5; IC 95% 4.43-30.1; P≤ 0.0001), livedo reticularis (OR 5.45; IC 95% 1.49-19.8; P=0.01) and obstetric features (OR 0.30; IC 95% 0.12-0.7; P=0.01).

Conclusion: The APS non-criteria clinical manifestations are frequent, can precede or follow thrombosis and are associated with LA. Damage accrual was mainly associated with thrombotic events followed by lived reticularis.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/non-criteria-clinical-manifestations-in-antiphospholipid-syndrome-clinical-behavior-and-association-with-damage-accrual/