Antiphospholipid Syndrome (APS) - Epidemiology Forecast - 2034

Antiphospholipid Syndrome (APS) - Epidemiology Forecast - 2034

Key Highlights

Antiphospholipid syndrome (APS) is an autoimmune disorder where the immune system mistakenly produces abnormal antibodies called antiphospholipid antibodies, which target proteins attached to fat molecules, making blood more likely to clot in arteries and veins.

APS can lead to severe complications such as strokes, heart attacks, and obstetric/pregnancy-related issues like miscarriages and stillbirths.

aPLs are autoantibodies that target phospholipid-bound proteins, notably ß2-glycoprotein-I (ß2GPI).

It is commonly categorized into primary APS, known as Hughes syndrome, and secondary APS, linked to another systemic inflammatory rheumatic disease.

Signs and symptoms of APS include high blood pressure, deep vein thrombosis (DVT), strokes or transient ischemic attacks (TIAs), heart attacks, and pulmonary embolism (blockage in lung blood vessels).

APS is one of the few clinical conditions in which patients can present with both venous and arterial thrombosis, with deep vein thrombosis of the lower extremity the most common presentation and stroke the most common arterial manifestation. Recurrent pregnancy loss is the most common pregnancy-related complication.

The risk factors include autoimmune diseases like systemic lupus erythematosus, genetic predisposition, certain infections, female gender, age, pregnancy, atherosclerosis, heart disease, diabetes, etc. While specific triggers remain unclear, these factors are associated with an increased likelihood of developing APS.

The diagnosis of APS involves a combination of clinical evaluation and laboratory tests. Key criteria include the presence of antiphospholipid antibodies (aPL) and a history of characteristic clinical manifestations such as thrombosis or pregnancy complications. Diagnostic tests may include assays for aPL, lupus anticoagulant, and anticardiolipin antibodies. The complexity and variability of APS often require a multidisciplinary approach, considering both clinical and laboratory findings for accurate diagnosis and subsequent management.

APS mostly affects women between 30 and 40 years. In APS, abnormal proteins can cause the formation of clots in veins and arteries.

Total prevalent cases of antiphospholipid syndrome in the 7MM were approximately 300,000 in 2023, which is likely to increase at a CAGR of 0.4% during the study period (2020–2034).

Unfortunately, the clinical variability of APS, coupled with the absence of standardized diagnostic tests, contributes to its frequent underdiagnosis. The lack of a uniform approach to diagnosis may result in delayed identification and intervention, emphasizing the need for improved diagnostic tools and awareness to address this challenge in clinical practice.

DelveInsight’s “Antiphospholipid Syndrome (APS) – Epidemiology Forecast – 2034” report delivers an in-depth understanding of APS, historical and forecasted epidemiology in the United States, EU4 (Germany, France, Italy, and Spain) and the United Kingdom, and Japan.

APS Disease Understanding and Diagnostic Algorithm

APS Overview

APS is a rare, immune-mediated, acquired hypercoagulable disorder characterized by the presence of persistent antiphospholipid antibodies (aPL) in combination with clinical events of thrombosis in the venous, arterial or microvascular system and/or certain adverse pregnancy outcomes. aPLs are autoantibodies that target phospholipid-bound proteins, notably ß2-glycoprotein-I (ß2GPI). Although the presence of these antibodies is the defining feature of this syndrome, the mechanism by which aPLs result in a hypercoagulable state remains incompletely understood.

Other common non-criteria APS manifestations include thrombocytopenia, livedo reticularis, or cardiac valve thickening or dysfunction. The aPL that is included in the standard test panel are lupus anticoagulant, anticardiolipin antibody (aCL), and anti-ß2 glycoprotein-I antibodies (anti-ß2GPI).

APS was first described by Professor Graham Hughes in 1983. It is usually divided into two groups: primary APS, also named Hughes syndrome, and secondary APS, associated with another systemic inflammatory rheumatic disease. Approximately 50% of patients with APS have a secondary form in association with an inflammatory disease, most commonly systemic lupus erythematosus (SLE). It has been shown that up to 10% of patients with primary APS are diagnosed with SLE within 10 years and redefined as secondary APS.

Further details are provided in the report…

APS Diagnosis

Antiphospholipid Syndrome (APS) poses challenges in diagnosis due to its clinical variability and lack of standardized tests. Classification criteria, requiring clinical and laboratory criteria, aid in diagnosis but may exclude non-criteria manifestations.

Additional antibodies like IgA aCL, anti-b2GPI, and others, though recognized, are not included in the current criteria. Diagnostic tests include ELISA for cardiolipin and b2GPI antibodies and the Lupus Anticoagulant (LA) test, performed according to International Society on Thrombosis and Hemostasis guidelines. LA is associated with venous thrombosis and stroke, while aCL is linked to pregnancy morbidity. Complication risks increase with the number of positive antibodies, with triple positivity (LA, aCL, and anti-b2GPI) indicating the highest risk. Early recognition and treatment are crucial for reducing APS-related mortality and morbidity.

Further details related to country-based variations are provided in the report…

APS Epidemiology

The APS epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by Prevalent Cases of Antiphospholipid Syndrome, Diagnosed Cases of Antiphospholipid Syndrome, Age-specific Cases, Gender-specific Cases, Type-specific Cases, Clinical manifestation-specific Cases, Treated Cases in the United States, EU4 countries (Germany, France, Italy, Spain) and the United Kingdom, and Japan from 2020 to 2034.

In the US, there were nearly 80,000 and ~41,000 cases of primary and secondary antiphospholipid syndrome in 2023, respectively, which is anticipated to increase by 2034.

In EU4 and the UK, out of all age groups, the highest number of age-specific cases accounted for 18–44 years, followed by 55–64 years and =75 years in 2023. In contrast, the least age-specific cases were observed in 65–74 years age groups.

As per DelveInsight’s analysis, higher cases were observed for thrombotic than obstetric based on clinical manifestation-specific cases of symptomatic antiphospholipid syndrome throughout the 7MM countries.

In Japan, there were around 24,000 treated cases of antiphospholipid syndrome in 2023. These cases are likely to rise by 2034.

Scope of the Report

The report covers a segment of key events, an executive summary, and a descriptive overview of APS, explaining its causes, signs and symptoms, pathogenesis, and currently available therapies.

Comprehensive insight into the epidemiology segments and forecasts, the future growth potential of diagnosis rate, and disease progression have been provided.

A detailed review of current challenges in establishing diagnosis and diagnosis rate is provided.

APS Report Insights

Patient Population

Prevalent Cases of Antiphospholipid Syndrome, Diagnosed Cases of Antiphospholipid Syndrome, Age-specific Cases, Gender-specific Cases, Type-specific Cases, Clinical manifestation-specific Cases, Treated Cases of Antiphospholipid Syndrome

Country-wise Epidemiology Distribution

APS Report Key Strengths

Eleven-year Forecast

The 7MM Coverage

APS Epidemiology Segmentation

APS Report Assessment

Epidemiology Segmentation

Current Diagnostic Practices

Key Questions

Epidemiology Insights

What are the disease risks, burdens, and unmet needs of APS? What will be the growth opportunities across the 7MM with respect to the patient population pertaining to APS?

What is the historical and forecasted APS patient pool in the United States, EU4 (Germany, France, Italy, Spain) and the United Kingdom, and Japan?

What is the diagnostic pattern of APS?

Which clinical factors will affect APS?

Which factors will affect the increase in the diagnosis of APS?

Reasons to buy

Insights on disease burden, details regarding diagnosis, and factors contributing to the change in the epidemiology of the disease during the forecast years.

To understand the change in APS cases in varying geographies over the coming years.

Detailed overview on total hematopoietic stem-cell transplant cases, total allogeneic transplant cases, type of APS (acute and chronic), total incident cases of aAPS by grading and organ involvement, total 5-year prevalent cases of cAPS by grading and organ involvement, total treated patients of APS, mortality adjusted APS treated patients of APS is included.

To understand the perspective of key opinion leaders around the current challenges with establishing the diagnosis and insights on the treatment-eligible patient pool.

Detailed insights on various factors hampering disease diagnosis and other existing diagnostic challenges.