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Small Percentage of aPL-Positive Patients Without Autoimmune Diseases Received Immunosuppression

January 12, 2023

Mid-January 2023

About 15% of patients with persistently positive antiphospholipid antibodies (aPLs) without other systemic autoimmune disease were found to have received immunosuppression other than corticosteroids or hydroxychloroquine for certain aPL manifestations, according to data from the APS ACTION Clinical Database and Repository. Researchers conducted a database analysis to better describe the indications for immunosuppression in aPL-positive patients and published their results in Lupus.

Patients with antiphospholipid syndrome (APS) and microvascular disease, such as diffuse alveolar hemorrhage, and with non-thrombotic disease, such as thrombocytopenia, “require a treatment strategy beyond anticoagulation,” explained study author Doruk Erkan, MD, attending rheumatologist at Hospital for Special Surgery and professor of medicine at Weill Cornell Medicine in New York. “Although immunosuppression is commonly used in the management of these patients, unfortunately, we do not have strong evidence-based recommendations.”

The APS ACTION Registry was created in 2010 to facilitate large-scale clinical studies and trials in persistently aPL-positive patients. To better describe the indications for immunosuppression in aPL-positive patients, Dr. Erkan and colleagues analyzed data from 537 patients without other systemic autoimmune disease. The majority were female (70%) and white (70%), and the mean age at entry was 45.

Researchers looked at follow-up data for immunosuppression manifestations as well as several aPL manifestations: diffuse alveolar hemorrhage (DAH), antiphospholipid nephropathy (aPL-N), livedoid vasculopathy (LV)-related skin ulcers, thrombocytopenia, autoimmune hemolytic anemia (AIHA), and cardiac valve disease (VD).

In all, 76 patients (14%) had used immunosuppression.

“The indication was at least one of the selected microvascular and/or non-thrombotic aPL-related manifestations in half of these patients,” Dr. Erkan said.

Specifically, 41 of the 76 patients who received immunosuppression (54%) received it for at least one selected aPL manifestation. Sixteen of these 41 patients had more than one aPL-related manifestation either simultaneously or at different time points.

Sixteen of the remaining 46% of patients had one or more of the selected aPL-related manifestations, but immunosuppression was not reported, the researchers noted.

The researchers also conducted a subgroup analysis of the 26% of patients (n=141) who had at least one of the selected microvascular and/or non-thrombotic manifestations and the type of immunosuppression used. Results of the subgroup analysis included the following:

  • eight patients had DAH; 75% received immunosuppression, most commonly with intravenous immunoglobulin (IVIG) or rituximab (RTX)
  • 19 patients had aPL-N; 32% received immunosuppression, most commonly mycophenolate mofetil or RTX
  • 28 patients had LV; 14% received immunosuppression with RTX
  • 88 patients (62%) had thrombocytopenia; 28% received immunosuppression, most commonly IVIG or RTX
  • 11 patients had AIHA; 55% received immunosuppression with IVIG or azathioprine
  • 43 patients had VD; only one received immunosuppression with IVIG

Dr. Erkan and colleagues discussed several limitations to their study. Specifically, the retrospective nature of the analysis “may not provide the most accurate information about each immunosuppressive indication,” they wrote, and it does not look at the effectiveness of the medications. In addition, the number of patients with some of the aPL-related manifestations was small.

Despite these limitations, first author Zeynep Belce Erton, MD, of Hospital for Special Surgery in New York noted, the “APS ACTION Registry has a heterogeneous group of aPL-positive patients from tertiary referral centers, representing a real-world experience.”

“While our results may guide physicians during the management of [aPL]-positive patients with microvascular disease and/or non-thrombotic disease,” Dr. Erkan said, “it is critical that future clinical studies address these immunosuppressive pathways and potentially other novel pathways in well-designed clinical trials to accumulate more evidence for their efficacy.”

Any conflicts of interest declared by the authors can be found in the original article.


Erton ZB, Leaf RK, de Andrade D, et al. Immunosuppression use in primary antiphospholipid antibody-positive patients: descriptive analysis of the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (“Registry”) [published online, 2022 Oct 7]. Lupus. doi: 10.1177/09612033221128742.


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