Pulmonary hypertension associated with antiphospholipid antibody: Call for a screening tool?

Parthvi, Rukma; Sikachi, Rutuja R.; Adial, Ajay; Vulisha, Abhinav; Khanijo, Sameer; Talwar, Arunabh

doi:10.5582/irdr.2017.01044

Cited by 15 publications

(10 citation statements)

References 56 publications

(36 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Patients with progressive CTEPH should be evaluated by a multidisciplinary team of experts. Surgical pulmonary thromboendarterectomy should be performed to prevent irreversible damage, improve hemodynamics, exercise capacity and survival (58, 121, 122). In symptomatic patients with CTEPH who are ineligible for surgery, or when irreversible pulmonary resistance is already evident, off-label use of drugs approved for pulmonary arterial hypertension, such as prostacyclins and endothelin receptor antagonists, should be considered.…”

Section: Management Of Aps-related Cardiac Diseasementioning

confidence: 99%

“…A phase 3 randomized trial compared a group of 173 patients treated with riociguat (a soluble guanylate cyclase stimulator) to a group of 88 individuals who were administered a placebo. The results showed that riociguat significantly improved the clinical outcomes in patients with CTEPH who were ineligible for surgery or who had persistent/recurrent PHT after undergoing pulmonary endarterectomy (122). Since inflammatory mechanisms may contribute to APS-associated CTEPH pathogenesis, the addition of immunosuppressive therapy may be beneficial.…”

Section: Management Of Aps-related Cardiac Diseasementioning

confidence: 99%

See 1 more Smart Citation

Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form

et al. 2019

View full text Add to dashboard Cite

Antiphospholipid syndrome (APS) is a multisystem autoimmune disease most commonly associated with recurrent arterial and venous thromboembolism and recurrent fetal loss. Other possible antiphospholipid antibody (aPL)-related clinical manifestations include cardiac involvement. The heart can be involved through immune mediated and /or thrombotic mechanisms. Mortality due to cardiovascular problems is elevated in APS. However, the cardiovascular risk in patients with primary APS (PAPS) compared with lupus-related APS is yet to be established. Cardiac symptoms of APS include valve abnormalities (thickening and vegetations), coronary artery disease (CAD), myocardial dysfunction, pulmonary hypertension, and intracardiac thrombi. Heart valve lesions are the most common cardiac manifestation, observed in approximately one third of PAPS patients and usually do not cause hemodynamic significance. Deposits of immunoglobulins including anticardiolipin (aCL), and of complement components, are commonly observed in affected heart valves from these patients. This suggests that an inflammatory process is initiated by aPL deposition, eventually resulting in the formation of valvular lesion. aPL may have a direct role in the atherosclerotic process via induction of endothelial activation. Multiple traditional and autoimmune-inflammatory risk factors are involved in triggering an expedited atherosclerotic arterial disease evident in APS. It is imperative to increase the efforts in early diagnosis, control of risk factors and close follow-up, in the attempt to minimize cardiovascular risk in APS. Clinicians should bear in mind that a multidisciplinary therapeutic approach is of paramount importance in these patients. This article reviews the cardiac detriments of APS, including treatment recommendations for each cardiac complication.

show abstract

Section: Management Of Aps-related Cardiac Diseasementioning

confidence: 99%

Section: Management Of Aps-related Cardiac Diseasementioning

confidence: 99%

Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form

et al. 2019

View full text Add to dashboard Cite

show abstract

“…Among SLE patients, a meta-analysis found that aPL can identify patients at risk for pulmonary hypertension 80 . There are various pathogenic mechanisms proposed, including: (i) large vessel and small vessel thrombosis; (ii) pro-inflammatory effects of aPL; (iii) Libman-Sacks endocarditis and left-sided valvular disease; and (iv) chronic thromboemboli and associated endothelial remodeling 79,81,82 . The prevalence of this manifestation in the Euro-phospholipid cohort was 2.2% 13 , while another European study of 114 APS patients displayed a prevalence of 3.5% in primary APS and 1.8% in APS associated with other autoimmune diseases 83 .…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

“…The prevalence of this manifestation in the Euro-phospholipid cohort was 2.2% 13 , while another European study of 114 APS patients displayed a prevalence of 3.5% in primary APS and 1.8% in APS associated with other autoimmune diseases 83 . The presence of aPL is suggested to be associated with pulmonary hypertension of across all the five WHO groups 81 . A review suggested that the outcome of pulmonary hypertension in aPL-positive patients seemed to be linked with the occurrence of new venous thromboembolic events or left-sided heart abnormalities 79 .…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

Non-criteria Manifestations of Antiphospholipid Syndrome: An Overview

Rosa¹,

Espinosa²,

Cervera³

2021

SJMED

View full text Add to dashboard Cite

While the classification criteria for definite antiphospholipid syndrome (APS) include specific thrombotic and obstetric manifestation, there are numerous clinical features also present in patients with antiphospholipid antibodies (aPL), which are not included in the classification criteria. The term "non-criteria" is currently used to refer to these manifestations. This review intends to provide a summarized, but far-reaching description, of the non-criteria manifestations present in the literature, with special focus on their association with APS and potential impact on the disease course. We analyze the following involvements: cardiac (cardiac microvascular disease and valvular heart disease), dermatological (livedo reticularis/racemosa, livedoid vasculopathy and skin ulcers), ear, nose, and throat (sensorineural hearing loss), endocrinological (adrenal insufficiency due to hemorrhagic infarction), hematological (hemolytic anemia and thrombocytopenia), musculoskeletal (ischemic bone necrosis), neurological (acute ischemic encephalopathy, chorea, cognitive dysfunction, epilepsy/seizures, migraine, and transverse myelitis), pulmonary (diffuse alveolar hemorrhage and pulmonary hypertension), ophthalmologic (amaurosis fugax), renal (APS nephropathy), vascular (superficial vein thrombosis), and obstetric (infertility, in vitro fertilization failure, and placenta-mediated complications) manifestations. Although gaining relevance in the current practice, the exact level of association of the different non-criteria manifestations with APS/aPL is still unclear or scarcely characterized in most cases.

show abstract

“…Other factors also may contribute to the pulmonary risk in these systemic autoimmune patients. SLE and anti-phospholipid syndrome APS are associated with hyper coagulability, and can cause chronic thrombo-emboli and therefore may develop CTEPH ( 3 ). SSc can induce pulmonary fibrosis leading to hypoxic vasoconstriction with long term vascular remodeling.…”

Section: Introductionmentioning

confidence: 99%

Vascular Remodeling in Pulmonary Arterial Hypertension: The Potential Involvement of Innate and Adaptive Immunity

et al. 2021

View full text Add to dashboard Cite

Pulmonary arterial hypertension (PAH) is a severe disease with high morbidity and mortality. Current therapies are mainly focused on vasodilative agents to improve prognosis. However, recent literature has shown the important interaction between immune cells and stromal vascular cells in the pathogenic modifications of the pulmonary vasculature. The immunological pathogenesis of PAH is known as a complex interplay between immune cells and vascular stromal cells, via direct contacts and/or their production of extra-cellular/diffusible factors such as cytokines, chemokines, and growth factors. These include, the B-cell—mast-cell axis, endothelium mediated fibroblast activation and subsequent M2 macrophage polarization, anti-endothelial cell antibodies and the versatile role of IL-6 on vascular cells. This review aims to outline the major pathophysiological changes in vascular cells caused by immunological mechanisms, leading to vascular remodeling, increased pulmonary vascular resistance and eventually PAH. Considering the underlying immunological mechanisms, these mechanisms may be key to halt progression of disease.

show abstract

Pulmonary hypertension associated with antiphospholipid antibody: Call for a screening tool?

Cited by 15 publications

References 56 publications

Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form

Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form

Non-criteria Manifestations of Antiphospholipid Syndrome: An Overview

Vascular Remodeling in Pulmonary Arterial Hypertension: The Potential Involvement of Innate and Adaptive Immunity

Contact Info

Product

Resources

About