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Toronto Notes 2019 Connective Tissue Disorders Table 16. Classification Criteria of APLS*
Rheumatology RH13
Manifestations of APLA
• Thromboembolic events
• Spontaneous abortions
• Thrombocytopenia
• Associated with livedo reticularis, migrane
headaches
Arterial and venous thrombosis are usually mutually exclusive
Criteria
CLINICAL
Vascular thrombosis Pregnancy morbidity
LABORATORY
Lupus anticoagulant Anti-cardiolipin Ab Anti-β2 glycoprotein-I Ab ANA
Description
Arterial: stroke/TIA, multi-infarct dementia, MI, valvular incompetence, limb ischemia Venous: DVT, PE, renal and retinal vein thrombosis
Must be confirmed by imaging or histopathology
Recurrent spontaneous abortions (<10 wk GA) , fetal death (>10 wk GA), or premature birth (<34 wk GA)
Labs must be positive on 2 occasions, at least 12 wk apart
Prolonged aPTT not corrected by the addition of normal plasma IgG and/or IgM
IgG and/or IgM
Most sensitive test (98%), not specific
* 1 clinical and 1 laboratory criteria must be present
Signs and Symptoms
• seeclinicalcriteriainTable16 • hematologic
J Thromb Haemost 2006;4:295-306
■ thrombocytopenia, hemolytic anemia, neutropenia • dermatologic
■ livedo reticularis, Raynaud’s phenomenon, purpura, leg ulcers, and gangrene
Treatment
• thrombosis
■ lifelong anti-coagulation with warfarin
■ target INR 2.0-3.0 for first venous event, >3.0 for recurrent and/or arterial event
• recurrentfetalloss
■ heparin/low molecular weight heparin ± ASA during pregnancy
• catastrophicALPS
■ high-dose steroids, anti-coagulation, cyclophosphamide, plasmapheresis
Scleroderma (i.e. Systemic Sclerosis)
Definition
• anon-inflammatoryautoimmunedisordercharacterizedbywidespreadsmallvesselvasculopathy, production of autoantibodies, and fibroblast dysfunction causing fibrosis
CREST Syndrome Calcinosis
Raynaud’s phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia
Scleroderma
(i.e. systemic sclerosis)
Localized
(no involvement of internal organs) • Mostly children and young adults
Generalized
Morphea
• Hard oval patches
on the skin
Linear
• Line of thickened skin
Limited systemic sclerosis • Skin sclerosis restricted to
hands, face, neck
• 3rd to 4th decade
• Pulmonary HTN common • CREST
Diffuse systemic sclerosis • Widespread skin disease (proximal to wrist, can involve trunk), tendons
• Early visceral involvement (renal, pulmonary fibrosis)
Figure 8. Forms of scleroderma
Table 17. The American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) Criteria for Scleroderma*
Item
1. Skin thickening of fingers of both hands extending proximal to the MCP (sufficient criterion) 2. Skin thickening of the fingers
3. Fingertip lesions
4. Telangiectasia
5. Abnormal nailfold capillaries
6. Pulmonary arterial HTN ± ILD (max score 2)
7. Raynaud’s phenomenon 8. Scleroderma related Ab
Sub-item Score
9
Puffy fingers 2 Sclerodactyly 4
Digital tip ulcers 2 Fingertip pitting scars 3
2
2
Pulmonary arterial HTN 2 ILD 2
3
Anti-centromere 3 Anti-topoisomerase I Anti-RNA polymerase III
Scleroderma is the most common cause of secondary Raynaud’s phenomenon
* Score of ≥9 is sufficient to classify a patient as having definite scleroderma (sensitivity 0.95, specificity 0.93) Arthritis & Rheum 2013;65(11):2737-2747