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Anticoagulation in Autoimmune Rheumatic Diseases

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Precision Anticoagulation Medicine

Abstract

Autoimmune rheumatic diseases as a group are characterized by common features such as multisystem involvement, polypharmacy, and the presence of comorbidities. Recent large epidemiological studies shed light on their role as risk factors for thromboembolic complications. The pathogenic factors are now better understood. The proposed mechanisms for this increased risk include increased hypercoagulability, lack of inhibitors, and other factors. The group of vasculitis which includes Behçet’s disease, giant cell vasculitis, Takayasu’s arteritis, and ANCA-associated vasculitis deserves special attention. However other diseases such as systemic lupus erythematosus, Sjögren syndrome, scleroderma, rheumatoid and psoriatic arthritis, myositis, and sarcoidosis are also associated with significantly increased risk of thrombosis.

The indications, choice, and monitoring of anticoagulation in the context of this group of disorders will need to be tailored precisely.

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Abbreviations

AAV:

ANCA-associated vasculitis

ANCA:

Antineutrophil cytoplasmic antibody

aPL:

Antiphospholipid

APS:

Antiphospholipid syndrome

AS:

Ankylosing spondylitis

AT III:

Antithrombin III

BD:

Behçet’s disease

CI:

Confidence interval

CVA:

Cerebrovascular accident

DM:

Dermatomyositis

DVT:

Deep vein thrombosis

ECP:

Eosinophil cationic protein

EGPA:

Eosinophilic granulomatosis with polyangiitis

EPO:

Eosinophil peroxidase

EULAR:

European league against rheumatism

GCA:

Giant cell arteritis

GPA:

Granulomatosis with polyangiitis

HDL-C:

High-density lipoprotein cholesterol

HR:

Hazard ratio

IBD:

Inflammatory bowel disease

IL:

Interleukin

IPH:

Idiopathic pulmonary hypertension

LDL-C:

Low-density lipoprotein cholesterol

LVV:

Large vessel vasculitis

MBP:

Major basic protein

MI:

Myocardial infarction

MPA:

Microscopic polyangiitis

NOACs:

New oral anticoagulants

PAI-1:

Plasminogen activator inhibitor-1

PAN:

Polyarteritis nodosa

PH:

Pulmonary hypertension

PM:

Polymyositis

PR3:

Proteinase 3

PsA:

Psoriatic arthritis

Pso:

Psoriasis

pSS:

Primary Sjögren syndrome

PTE:

Pulmonary thromboembolism

Ptn C:

Protein C

Ptn S:

Protein S

PVD:

Peripheral vascular disease

RA:

Rheumatoid arthritis

SLE:

Systemic lupus erythematosus

SS:

Sjögren syndrome

SSc:

Systemic sclerosis

TA:

Takayasu’s arteritis

TNF-α:

Tumor necrosis factor alpha

tsDMARDs:

Targeted synthetic disease-modifying antirheumatic drugs

VTE:

Venous thromboembolism

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Ragab, G., Hegazy, M.T., Codullo, V., Mattar, M., Avouac, J. (2020). Anticoagulation in Autoimmune Rheumatic Diseases. In: Goubran, H., Ragab, G., Hassouna, S. (eds) Precision Anticoagulation Medicine. Springer, Cham. https://doi.org/10.1007/978-3-030-25782-8_7

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