Capilaroscopia unghiala- importanta practica

Laura Groseanu

Centrul de Cercetare in Patogenia si Tratamentul Bolilor Reumatismale, Spitalul Clinic “Sf. Maria”, Clinica de Medicina Interna si Reumatologie Bucuresti

port

pilaroscopie

Raport de capilaroscopie- “Comment”

●Transparenta cutanata ●Plex venos subpapilar ●Densitate capilara ●Arhitecturala vasculara ●Anomalii morfologice ●Microhemoragii ●Neovascularizatie

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Evaluare capilaroscopica

Pattern recognition (calitativ)

Scoring semicantitativ

Scoring cantitativ ( computerizat)

Scleroderma pattern/ like pattern

Modificari nonspecifice

(microangiopatie nespecifica)

0=

1= < 33% modificari din nr capilare per mm 2= 33–66% modificari din nr capilare per mm

3= > 66% modificari din nr capilare per mm

fara modificari

2 imagini/deget

8 degete

Capilare dilatate

Capilare gigante

A

Hemoragii.depozite de hemosiderina

Corelat cu Forme severe d boala cu afecta vasculara severa pulmonara, cardiaca

B

Densitate capilara

Dezorganizare arhitecturala

Capilare ramificate

Scor de evolutie microangiopatica

Sindrom Raynaud ○dg diferential primar/sec ○monitorizare Scleroza sistemica ○dg precoce ○dg diferential sdr sclero-like ○dg corect ○ prognostic ○follow-up

Sdr Raynaud primar

(1) Raynaud clinic

(2) Capilaroscopie normala

(3) FARA elemente sugestive pt cauze sec: ulceratii,

gangrena, necroze, sclerodactilie, calcinoza , modificari

cutanate

(4)fara istoric de boala autoimuna

(5) ANA negativ/titru mic

Rol diagnostic-

reduce costul investigatiilor suplimentare costitoare

Rol prognostic –

monitorizarea tranzitiei catre Raynaud secundar (12,5%) 1

1 Hirschl M et al Transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon identified by diagnosis

of an associated disease: results of ten years of prospective surveillance Arthritis Rheum. 2006 Jun;54(6):1974-81

Sindrom Raynaud primar

Fara modificari specifice

-Anse alungite

-tortuozitati

23 ani, studenta UMF

Mama- LES

Raynaud 6 ani

Sindrom Raynaud secundar

-

-

-

-

-

2013

I.I., femeie, 23 ani Raynaud – 1 an

aug2016 – ulceratii digitale+++→gangrena

sclerodactilie

dispnee clasa III

ANA+++, antiU1RNP>200Ui/ml

BRD , sPAP=85mmHg

deces – nov2017

tardiv

Follow-up

tratament

Activ

Predictie

complicatii

precoce

Diagnostic

precoce

Diagnostic precoce VEDOSS

(very early disease onset of systemic sclerosis)

Diagnostic precoce - VEDOSS

G.M, 56 ani, ginecolog Puffy hands Raynaud -4 luni Artralgii art mici maini

ANA+,

antiSCL70>200U/l

Late scleroderma pattern

Diagnostic corect (1)

B.V., 54 ani 2011- Raynaud, ulceratii digitale 2014 - sclerodactilie, indurare tegumentara maini -ulceratii digitale ANA +, antiSCL70, antiU1RNP, ACA negativi

Scleroderma pattern active

Scleroza sistemica cu afectare cutanata limitata si ulceratii digitale active

Bosentan 9 luni

Diagnostic corect (2)

A.M., 43 ani, mediu rural Feb 2012- arttralgii art mici maini, Raynaud +/- Sdr inflamator, RF+++

Poliartrita reumatoida→Prednison 20mg/zi, MTX 10mg/sapt

May 2015 – persistenta artralgiilor - Raynaud ++ - discrete indurare tegumentara maini, fata,torace anterior

Diagnostic corect (2)

SSc pattern active-late

SCL 70 Ab >200 IU/ml

FVC=65%, FEV=62%, DLCO=55%

CT- pneumopatie interstitiala

Ciclofosfamide

Diagnostic diferential

65 ani, femeie Afectare cutanata (indurare) rapid progresiva Artralgii Nu relateaza Raynaud, ANA -

Fasciita cu eozinofile

Valoare prognostica – Capilaroscopie= biomarker al activitatii bol

Smith V ¹ et al.,Cutolo M Do worsening scleroderma capillaroscopic patterns predict future severe organ involvement? a pilot study. Ann Rheum Dis. 2012

Oct;71(10):1636-9

“ NVC patterns were significantly associated with severe peripheral vascular (p<0.001), skin (p=0.002), joint (p=0.003), muscle (p<0.001), GI (p=0.038), lung (p<0.001) and heart (p=0.009) involvement…the odds ratio for severe peripheral involvement was 3.36/2.18 (95% CI 1.77- 6.96/1.06-4.88) for the “active” and 11.27/4.77 for the “late” NVC scleroderma pattern versus the “normal” and “early” pattern….the estimated odds ratio to present with ILD is stronger according to worsening NVC patterns

Valoare prognostica – Capilaroscopie= biomarker al activitatii bol

Ingegnoli F, Ardoino , Boracchi P Cutolo M EUSTAR co-authors

Nailfold capillaroscopy in systemic sclerosis: data from the EULAR scleroderma trials and research (EUSTAR) database. Microvasc Res. 2013 Sep;89:122-8

110centre EUSTAR, 2754 pacienti “…The "early" and "active" scleroderma patterns were generally observed in patients with mild/moderate skin involvement and a low number of disease manifestations, while the "late" scleroderma pattern was found more frequently in the more severe forms of the disease.”

6 months

2014- early

2015-early

F, 38 ani, SS

2013

– Raynaud -Ingrosare tegumentara difuza -antiSCL70 + Trat: MTX, vasodilatatoare

2015 – fara afectare viscerala majora

2012-active

Vadeanu

2015-active

F, 58 ani ●1995- SSc cu afectare tegumentara difuza, musculoarticulara, GI si pulmonara ●Tratament 1995-2015:

corticosteroizi, vasodilatatoare, Ciclofosfamide 3 luni→Tbc ●2015 – fara ulceratii/ PAH,

TLCO=73%

2010-late

coring =4

2013-late

Scoring=9

F, 57 ani 1996 – SS cu afectare cutanata limitata, calcificari sc si afectare GI 2013 – ulceratii digitale

2014

Late pattern

2015

B,74 ani 1995- SSc cu afectare tegumentara difu Tratament- corticosteroizi, vasodilatoar 2009 - active pattern 2014-2015

- Dispneea clasa III- fibroza pulmonara

(TLCO=38%)

-ulceratii digitale

Scleroderma like pattern

Pattern scleroderma

megacapilare rare/deloc !!!!

Poli/dermatomiozita

Boala mixta de tesut conjunctiv

Dermatomiozita

Sclero-like pattern

Scaderea densitatii

Capilare dilatate, tortuozitati

Capilare ramificate

Microhemoragii

BMTC

Normal→sclero-like pattern Dilatatii capilare Neovascularizatie Scaderea densitatii—zone avasculare

Afectare pulmonara (HTP, alveolita)

Microangiopatie nonspecifica

Modificari nespecifice Modificari specific izolate

Monitorizare evolutiva…

LUPUS ERITEMATOS SISTEMIC

Normal→modificari nespecifice (54%)

-tortuozitati, anse bizare, dilatate, ramificatii

-Scaderea densitatii capilare

-Plex vv subpapilar ↑

-F rar : sclero-like pattern

- Fara legatura cu sdr Raynaud

- Severitatea corelata cu

SLEDAI/ECLAM

- Reversibile in remisiune

Sindrom antifosfolipidic

Hemoragii/depozite de hemosiderina Anse alungite, tortuoase, dilatate

POLIARTRITA

REUMATOIDA

Normal→modificari nespecifice(80%)

-Vizibilitate crescuta plex vv subpapilar

-Capilare alungite/dilatate

SINDROM

SJOGREN

Normal→modificari nespecifice→sclero-like pattern

Trombangeita obliteranta

-microhemoragii

-tortuozitati

-edem papilar (aspect “foggy”)

-anse dilatate, megacapilare izolate

-oprire flux capilar

Fumator

Modificari nespecifice -Reducere diametru -Reducerea densitatii capilare -Tortuozitati -Capilare ramificate -Fragmentare flux sanguin