Morbidity and mortality in antiphospholipid syndrome based on cluster analysis: a 10-year longitudinal cohort study

Ogata, Yuka; Fujieda, Yuichiro; Sugawara, Masayoshi; Sato, Taiki; Ohnishi, Naoki; Kono, Michihito; Kato, Masaaki; Oku, Kenji; Amengual, Olga; Atsumi, Tatsuya

doi:10.1093/rheumatology/keaa542

Cited by 21 publications

(30 citation statements)

References 22 publications

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“…In another study, a cluster of patients with arterial thrombosis and cardiovascular risk factors, a cluster with SLE+APS and a cluster with venous thrombosis and triple aPL positivity were identified. 4 Frequency of thrombotic events and mortality rate were significantly higher in the first cluster. Cluster analysis of the APS ACTION Registry revealed three different disease phenotypes: female PAPS patients with venous thromboembolism and triple aPL positivity; female SLE/APS patients with venous thromboembolism and extra-criteria manifestations; older male patients with arterial thrombosis and cardiovascular risk factors.…”

Section: Discussionmentioning

confidence: 90%

“…Recently cluster analysis has been used in different APS cohorts to show different disease phenotypes and to compare the frequencies of thrombosis and mortality frequencies between subgroups. [3][4][5] Organ damage is defined as "permanent loss of the normal function of an organ system because of a clinical manifestation of the disease." 6 Disease damage has been shown as an essential predictor of survival and a major 1 Division of Rheumatology Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey determinant of the quality of life in many studies.…”

Section: Introductionmentioning

confidence: 99%

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Description of damage in different clusters of patients with antiphospholipid syndrome

Uludag

Çene

Gurel

et al. 2022

Lupus

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Objective To identify the different clinical phenotypes of antiphospholipid syndrome (APS) by using cluster analysis and describe cumulative damage of disease clusters. Methods This retrospective study includes patients with APS (±systemic lupus erythematosus (SLE)). Two-step cluster analysis was applied by considering clinical data. Damage was calculated for all patients by applying damage index for APS (DIAPS). Results A total of 237 patients (198 females; median age of 43 years; median follow-up of 9.5 years) were classified into four clusters. Cluster 1 ( n = 74) consisted of older patients with arterial-predominant thrombosis, livedo reticularis, and increased cardiovascular risk; cluster 2 ( n = 70) of SLE+APS patients with thrombocytopenia and heart valve disease; cluster 3 ( n = 59) of patients with venous-predominant thrombosis, less extra-criteria manifestations; and cluster 4 ( n = 34) of patients with only pregnancy morbidity with lower frequency of extra-criteria features and cardiovascular risk. Patients with SLE+APS ( n = 123) had the highest mean DIAPS. Regarding clusters, 1 and 2 had high cumulative damage. While cumulative survival rates of clusters did not differ, cluster 2 and 3 had lower survival rates at further years. There was no correlation between DIAPS and mortality. Conclusion SLE+APS patients with extra-criteria manifestations and older APS patients with arterial thrombosis and increased cardiovascular risk have higher cumulative damage. Effective treatment of SLE disease activity and control of cardiovascular risk may help to reduce cumulative damage in these patients.

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Section: Discussionmentioning

confidence: 90%

Section: Introductionmentioning

confidence: 99%

Description of damage in different clusters of patients with antiphospholipid syndrome

Uludag

Çene

Gurel

et al. 2022

Lupus

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“…However, a significant proportion -one third -of affected patients have CAPS. The incidence in this subgroup is thus relatively high, between 10 to 16% [56]. A provoking moment usually initiates aPL-induced AH.…”

Section: Adrenal Hemorrhagementioning

confidence: 97%

“…A microscopic pathoanatomical picture typically reveals capillaritis with interstitial neutrophilic infiltrate, thrombi in small muscular pulmonary arteries, myointimal thickening, and the remodeling of the muscular pulmonary arteries and arterioles [53,54]. The condition is rare and appears in less than 1% of all aPL-positive patients, though it is considerably more frequent and clinically relevant in those with CAPS, affecting 5-10% [54][55][56][57]. Both genders are affected, but males constitute approximately 2/3 of cases with primary APS, whereas women dominate the group with APS secondary to SLE [54].…”

Section: Bleeding In Thrombotic Microangiopathies Associated With Apl 41 Diffuse Alveolar Hemorrhagementioning

confidence: 99%

Bleeding in Patients with Antiphospholipid Antibodies

Kubisz¹,

Holly²,

Staško³

2022

Antiphospholipid Syndrome - Recent Advances in Clinical and Basic Aspects

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The antiphospholipid antibodies (aPL) are commonly associated with thrombotic events and obstetric complications. However, apart from the bleeding complications of antithrombotic therapy, the acquired coagulopathy caused by the aPL, particularly by lupus anticoagulant and anticardiolipin antibodies, might be occasionally manifested as a hemorrhagic syndrome with various clinical severity. Bleeding symptoms vary from mild (mucocutaneous) up to life-threatening (gastrointestinal, intracranial). The bleeding may be the first manifestation of aPL or appear concomitantly with thrombosis. The underlying hemostatic changes include thrombocytopenia, platelet function disorders, and coagulation factor inhibitors or deficiencies, namely prothrombin, FVII, FVIII, FX, and FXI. Thrombocytopenia is the most common finding, seen in up to 53% of patients with aPL, although it is usually mild to moderate and associated with significant bleeding only in a minority of cases. Of interest, patients with severe thrombocytopenia appear to be less likely to suffer from thrombotic events. The involved pathophysiological mechanisms are heterogeneous. Non-neutralizing antibodies against coagulation factors resulting in increased clearance, specific antibodies against platelet membrane glycoproteins, increasing platelet activation and aggregation with subsequent consumption, and immune-mediated platelet clearance are among those identified. Immunosuppression, preferably with corticosteroids, represents the first-choice therapeutic approach. Plasmapheresis is efficient in the case of catastrophic antiphospholipid syndrome. Antithrombotic therapy can be challenging, but its administration should continue as much as possible.

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“…As an exploratory method, cluster analysis was increasingly applied to APS [ 6 – 8 ]. Clusters corresponded to well-known phenotypes, including secondary APS, obstetric APS, asymptomatic aPLs carriers, and thrombotic APS with multiple cardiovascular risk factors, were identified [ 6 – 8 ].…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics and prognosis of patients with antiphospholipid antibodies based on cluster analysis: an 8-year cohort study

Huang

Jiang

et al. 2022

Arthritis Res Ther

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Background Antiphospholipid syndrome (APS) is an autoimmune disease characterized by persistent antiphospholipid antibodies (aPLs) positivity with a wide manifestation spectrum. A risk stratification is needed for management guidance and prognosis assessment. We aimed to identify phenotypes among aPL-positive patients and assess the prognosis of each phenotype. Methods This was a single-center, prospective cohort study of aPL-positive patients presented to Peking Union Medical College Hospital from 2012 to 2020. Demographic characteristics, aPL-related manifestations, cardiovascular risk factors, and antibodies profiles were recorded. The primary endpoint was defined as a combination of newly onset thrombosis, major bleeding events, non-criteria manifestations, and all-cause death. Hierarchical cluster analysis and Kaplan-Meier survival analysis were performed. Results Four clusters among 383 patients (70.2% female; mean age 37.7 years) were identified. Cluster 1 (n = 138): patients with systemic lupus erythematosus (SLE) and non-criteria manifestations; cluster 2 (n = 112): patients with multiple cardiovascular risk factors; cluster 3 (n = 83): female patients with obstetric morbidity; cluster 4 (n = 50): patients with isolated lupus anticoagulant (LA) positivity. Non-criteria manifestations were found aggregated with SLE from cluster analysis of variables. Cluster 3 showed the best outcome, while cluster 2 suffered highest frenquency of newly onset arterial thrombosis. Conclusions We identified 4 clinical phenotypes of aPL-positive patients. Non-criteria manifestations may indicate underlying SLE, for which immunosuppressive therapy besides anticoagulation may be necessary. Patients with isolated LA positivity suffered similar risks with secondary APS and patients with multiple cardiovascular risk factors. Attention should be paid to male patients, and the screening of cardiovascular risk factors should never be ignored.

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Morbidity and mortality in antiphospholipid syndrome based on cluster analysis: a 10-year longitudinal cohort study

Cited by 21 publications

References 22 publications

Description of damage in different clusters of patients with antiphospholipid syndrome

Description of damage in different clusters of patients with antiphospholipid syndrome

Bleeding in Patients with Antiphospholipid Antibodies

Clinical characteristics and prognosis of patients with antiphospholipid antibodies based on cluster analysis: an 8-year cohort study

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