SSc e nuovi anticorpi: quale rilevanza per la clinica Roberto - - PowerPoint PPT Presentation

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SSc e nuovi anticorpi: quale rilevanza per la clinica Roberto Manetti Dip. di Medicina Clinica e Sperimentale Firenze 10-11 novembre2017 Systemic sclerosis is an idiopathic chronic autoimmune disease characterized by microvascular

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SSc e nuovi anticorpi: quale rilevanza per la clinica

Roberto Manetti

Dip. di Medicina Clinica e Sperimentale

Firenze 10-11 novembre2017

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Systemic sclerosis is an idiopathic chronic autoimmune disease characterized by microvascular abnormalities, cutaneous and visceral fibrosis all accompanied by immune abnormalities

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> Raynaud’s phenomenon > Involvement of:

Skin
Gastrointestinal tract
Kidney
Cardiovascular system
Lung

SSc patients are classified as:

diffuse cutaneous SSc
limited cutaneous SSc
sine cutaneous disease

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Is SSc the correct diagnosis?
Is there a greater risk for involvement
f certain organs?
Can the disease course and the vital

prognosis be predicted?

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2013 ACR/EULAR Criteria for the classification of Systemic Sclerosis

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2013 ACR/EULAR Criteria for the classification of Systemic Sclerosis

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Autoantibodies are seen at first diagnosis:

in more than 95% of SSc patients
have been associated with distinct

disease subtypes

have been associated with differences

in disease severity (including extent

f skin involvement, internal organ

manifestations and prognosis)

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Indirect immunofluorescence technique using HEp-2 continues to be the preferred screening immunoassay to detect most clinically relevant autoantibodies in SSc.

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A negative IIF test does not necessarily exclude the presence of a wide spectrum of autoantibodies including: Ku Jo-1 SS-A/Ro60 Ro52 SS-B/La RNA polymerases PM/Scl In case of high clinical suspicion of SSc and a negative IIF test, the identification of autoantibodies should include other specific and sensitive assays

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Many of the autoantibodies in SSc target nuclear antigens

(ANA was reported to range 85% and 99%)

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NUCLEAR FLUORESCENCE “SPECKLED”

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“NUCLEOLAR” FLUORESCENCE

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“CENTROMERIC” FLUORESCENCE

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AutoAbs profile of 528 unselected Australian sera

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Anti-centromere antibodies (CENP)

Associated with a higher risk of:

pulmonary arterial hypertension

Negatively associated with:

cardiac and renal involvement

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Mehra S. Autoimmunity Review 2013

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Anti-topoisomerase I antibodies

Associated with a higher risk of:

severe pulmonary fibrosis
cardiac involvement
musculoskeletal involvement
digital ulcers

Anti-RNA polymerase I and III antibodies

Associated with a higher risk of:

renal crisis
joint involvement
synovitis
tendon friction rubs
myositis
developing malignancy

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Mehra S. Autoimmunity Review 2013

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Mehra S. Autoimmunity Review 2013

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Anti-fibrillarin (or anti-U3RNP)

Associated with a higher risk of:

dcSSc
visceral involvement
renal and cardiac involvement
severe pulmonary disease
pulmonary hypertension
severe small bowel involvement

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Anti-Th/To

Associated with a higher risk of:

lcSSc
pulmonary fibrosis
renal crisis

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Mehra S. Autoimmunity Review 2013

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SSc subsets stratified by autoantibodies

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SSc is a potentially life-threatening disease
Autoantibodies are important biomarkers for early

and accurate diagnosis of SSc

Autoantibodies are associated with distinctive

clinical subsets and prognostic features

Regular monitoring for major organ complications

needs to be considered in all patients

A more frequent coexistence of autoantibodies in

SSc patients than previously appreciated has been reported

Conclusions

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Grazie azie per l’atten ttenzi zione ne

Foto R. Biddau