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  • Sclrodermie systmique Pronostic

    Ple de Mdecine Interne, Centre de rfrence pour les vascularites

    ncrosantes et la sclrodermie systmique, hpital Cochin, Assistance

    publique-Hpitaux de Paris, Paris

    Universit Paris Descartes, Inserm U1016, Institut Cochin, Paris

    Luc Mouthon

    http://www.inserm.fr/index.php

  • Skin score

    Visceral involvement

    SYSTEMIC SCLEROSIS : EVOLUTION

    Diffuse

    Limited cutaneous

    0 10 2 4 6 8

    Raynauds syndrome

    Kidney

    ILD + PAH

    Myositis

    Bowell

    ILD

    PAH

    Bowell

    0 10 2 4 6 8 Years

    Lung

  • Elements pronostiques (I)

    The 9 years survival rate was 38% with severe visceral involvement

    72% without visceral involvement (p < 0.001)

    Steen V, Arthritis Rheum 2000: 2437

  • lments pronostiques (II)

    Les sries rcentes montrent une diminution de la mortalit survie 10 ans de 80% 92% dans les formes

    limits

    survie 10 ans de 62 76% dans les formes diffuses

    Cela semble en grande partie lie lamlioration des traitements vise vasculaire

    Caractres pjoratifs dune atteinte viscrale en particulier pulmonaires, cardiaques et rnales.

  • Mortalit

  • Pronostic de la sclrodermie systmique

    tude Patients Survie 5 ans Survie 10

    ans

    Farmer 1960 236 51%

    Tuffanelli 1962 727 70% 59%

    Bennett 1971 67 73% 40%

    Medsger1971 309 60% 35%

    Medsger1973 358 44%

    Barnett 1978 113 70% 55%

    Eason 1981 47 60% 42%

    Wynn 1985 64 69% 51%

    Giordano 1986 90 72% 32%

    Altman 1991 264 63% 42%

    Bulpitt 1993 48 68%

    Wells 1994 68 85%

    Seibold 2000 68 85%

  • Surmortalit lie la ScS

    Ferri et al, Medicine, 2002

  • Changes in causes of systemic sclerosis

    related deaths between 1972 and 2001

    Steen VD, Medsger T, Ann Rheum Dis 2007, Feb 28 EPub

  • Principales causes de dcs

    rapportes dans la littrature

    Source N

    dcs

    Imput la

    ScS

    HTAP/Pulm Infection Cardiaque ou

    vasc

    Cancer Dig Rein

    Gouet (Gouet et al, 1986)

    29 6,9% /

    3,4%

    3,4% 48,3% 13,8

    %

    10,3

    %

    Scussell-Lonzetti

    (Scussel-

    Lonzetti et al,

    2002)

    309 53% 15,1% 19,7

    %

    Jacobsen

    (Jacobsen et al,

    1998)

    160 25,6% 6,3% /

    1,9%

    11,9% 30% 18,9

    %

    5,6% 10,6

    %

    Hesselstrand

    (Hesselstrand et

    al, 1998)

    49 30,6% 20,4% 20,4% 24,5

    %

    8,2% 2%

    Lee (Lee et al, 1992) 61 44,3% 32,8% 3,3% 23% 3,8% 6,6% 11,5

    %

    Kettaneh A, Sclrodermies, 2007

  • ScS diffuse et atteinte dorgane

    Steen , Arthr Rheum 2000; 43: 2437-2444 2000

  • Autoanticorps

    Survie 10 ans des patients atteints de

    ScS

    - 93 % avec anticorps anticentromre

    - 66 % avec Ac anti-Scl70

    - 30 % chez les patients ayant des anti-

    ARN polymrase.

    Kuwana M, Arthritis Rheum 1994; 37: 75-83

  • Demographic and clinical factors associated

    with in-hospital death among patients with

    systemic sclerosis.

    Data were obtained on all hospitalizations in South Carolina for patients who were ever hospitalized between 1996 and 2000 with a diagnosis of SSc.

    Proportions of in-hospital deaths among blacks (23.0%) and others (27.7%) were higher than among whites (15.6%), a finding that remained after adjustment for other sociodemographic and clinical factors (black/white odds ratio: 1.70).

    Black and other non-white patients with SSc appear to experience an elevated risk of death during their hospital stays.

    Nietert PJ, J Rheumatol 2005

  • Race and association with disease manifestations and

    mortality in scleroderma: a 20-year experience at the Johns

    Hopkins Scleroderma Center and review of the literature (I).

    Between 1990 and 2009, 409 African American and 1808 white patients with SSc were evaluated at a single university medical center.

    African American patients presented to the center at a younger mean age than white patients (47 vs. 53 yr; p < 0.001).

    Two-thirds of white patients manifested the limited cutaneous subset of disease, whereas the majority of African American patients manifested the diffuse cutaneous subset (p < 0.001).

    The proportion seropositive for anticentromere antibody was nearly 3-fold greater among white patients, at 34%, compared to African American patients (12%; p < 0.001).

    Nearly a third of African American (31%) patients had autoantibodies to topoisomerase, compared to 19% of white patients (p = 0.001).

    Notably, African American patients experienced an increase in prevalence of cardiac (adjusted odds ratio [OR], 1.6; 95% confidence interval [CI], 1.3-2.2), renal (OR, 1.6; 95% CI, 1.2-2.1), digital ischemia (OR, 1.5; 95% CI, 1.4-2.2), muscle (OR, 1.7; 95% CI, 1.3-2.3), and restrictive lung (OR, 6.9; 95% CI, 5.1-9.4) disease.

    Gelber AC et al, Medicine 2013

  • Race and association with disease manifestations and

    mortality in scleroderma: a 20-year experience at the Johns

    Hopkins Scleroderma Center and review of the literature (I).

    Overall, 700 (32%) patients died (159 African American; 541 white).

    Cumulative incidence of mortality at 10 years: 43% among African American patients vs 35% among white patients (log-rank p = 0.0011).

    Compared to white patients, African American patients experienced an 80% increase in risk of mortality (relative risk [RR], 1.8; 95% CI, 1.4-2.2), after adjustment for age at disease onset and disease duration.

    Adjustment by sex, disease subtype, SSc-specific autoantibody status, socioeconomic measures of educational attainment and health insurance status, diminished these risk estimates (RR, 1.3; 95% CI, 1.0-1.6).

    The heightened risk of mortality persisted in strata defined by age at disease onset, diffuse SSc, anticentromere seropositivity, decade of care at the center, and among women.

    These findings support the notion that race is related to a distinct phenotypic profile in scleroderma, and a more unfavorable prognosis among African Americans, warranting heightened diagnostic evaluation and vigilant care of these patients.

    Gelber AC et al, Medicine 2013

  • Disease-related nutritional risk and mortality in systemic sclerosis

    Single-centre prospective cohort study: 160 SSc patients (median age, 62 yr).

    Nutritional risk assessed by the Malnutrition Universal Screening Tool (MUST), a screening tool that combines anthropometric parameters of nutritional status (body mass index [BMI] and percentage of unintentional weight loss [WL]) with the presence of an "acute disease" (disease activity score 3 according to Valentini's criteria).

    Prevalence of high nutritional risk (MUST score 2): 24.4% [95%CI, 17.4-31.3].

    Hazard analysis (median follow-up duration = 46 months [25th-75th percentile, 31-54]), high nutritional risk was significantly associated with mortality (HR = 8.3 [95%CI, 2.1-32.1]).

    Performance of the model based on nutritional risk including disease activity (Harrell's c = 0.74 [95%CI, 0.59-0.89]) was superior to that based on active disease alone (HR = 6.3 [95%CI, 1.8-21.7]; Harrell's c = 0.68 [95%CI, 0.53-0.84]).

    In SSc outpatients MUST significantly predicts mortality.

    The combined assessment of nutritional parameters and disease activity significantly improves the evaluation of mortality risk.

    Cereda E et al. Clin Nutr 2013

  • Pronostic en fonction du

    type datteinte viscrale

  • When major

    visceral

    involvement occurs

    in scleroderma, the

    outcome is poor

    Lung fibrosis

    Cardiac

    involvement

    Renal insufficiency

    Pulmonary

    hypertension

    SYSTEMIC SCLEROSIS

    Ferri et al, Medicine, 2002

  • Severe organ involvement in SSc with diffuse scleroderma

    Steen VD, Medsger T, Arthr Rheum 2000; 43: 2437-2444

  • Pronostic: latteinte cutane proximale et la

    rapidit dinstallation sont les lments

    pronostiques importants

    Ferri et al, Medicine, 2002

  • Shand L, et al. Arthritis Rheum 2007; 56:2422-31.

    Disease duration and skin score in

    dcSSc

    Change in skin score over 3 years in the subgroups Survival in the subgroups

    High baseline/improvers

    Disease duration

    12 24 36 10

    20

    30

    40

    50

    Low

    baseline/improvers

    High baseline/non-improvers

    mR

    SS

    High baseline/ improvers, n = 40

    Low baseline/

    improvers, n = 67

    252 180 108 36 0

    20

    40

    60

    80

    100

    p=0.003

    High baseline/

    non-improvers, n = 24 C

    um

    ula

    tive s

    urv

    ival

    (%)

    Disease duration

  • Koh et al. Br J Rheumatol 1996;35:989-93.

    0

    10

    20

    30

    40

    50

    60

    70

    80

    90

    100

    0 1 2 3 4 5 6 7 8 9 10 11 12 13

    Dlai depuis le diagnostic (ans)

    Surv

    ie (

    %)

    HTAP

    Poumon

    (sans HTAP)

    Aucun

    SURVIE DES PATIENTS ATTEINTS DE SCLRODERMIE

  • Steen V, Arthritis Rheum 1994. 37:1283.

    PRONOSTIC DE LA FIBROSE PULMONAIRE

    100

    80

    60

    40

    20

    0 0 2 4 6 8 10

    Dure en annes

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