2. Fenômeno de RaynaudFenômeno de Raynaud
Resposta fisiológica exagerada em resposta a exposição ao frio e ao
estresse
Isquemia Cianose Cianose
Reperfusão
Herrick, A. L. Nat. Rev. Rheumatol. 2012
3.
4. Fenômeno de RaynaudFenômeno de Raynaud
ion in Raynaud phenomenon
elaxes smooth muscle cells. In
othelial NO synthase (NOS) is
as in advanced disease, induc-
e roles of the different isoforms
ilatation are currently unclear10
on via neuronal NOS could also
16
demonstrating the interplay
eural’ abnormalities. Further
NO, in patients with SSc, the
enous inhibitor of endothelial
hylarginine—are increased.17
whether there is underproduc-
f NO in the digits of patients
disease-stage-dependent), NO
mple, via topical application of
n vasodilatation. This response
nts with SSc as in patients with
menon and healthy controls.18
on
onstrictors by endothelial cells
n-1 and angiotensin II) most
elated Raynaud phenomenon.
helin-1 is increased in sclero-
lance in the renin–angiotensin
otensin II, is thought to occur
studies implicated a role for
Nerve bres
(sympathetic
and sensory)
Smooth muscle cell
Endothelial cell
Endothelin-1
from endothelial cells
Oxidative stress
Endothelial damage
Reduced blood ow/
procoagulant tendency
NO
from endothelial cells
Platelet activation/
aggregation
Reactivity of smooth muscle
α2-adrenoceptors (via
Rho/Rho kinase activation)
Thrombin
Viscosity
Fibrinolysis
Red blood cell
deformability
Vasoconstriction Vasodilatation
Vasodilatatory neuropeptides
(e.g. CGRP) from sensor y
afferents
Figure 1 | Schematic representation of some of the key elements and
mechanisms contributing to the pathogenesis of Raynaud phenomenon. Some of
R E V I E W S
5. Fenômeno de RaynaudFenômeno de Raynaud
• Primário ou idiopático:
Mulher jovem
Ataques simétricos/ curta duração
Prevalência: 19-11%
Ausência de cicatriz, ulcera ou gangrena digital
Capilaroscopia normal
FAN negativo
PAIs normais
Exame físico normal
Ausência de sintomas sugestivos de DTC
Herrick, A. L. Nat. Rev. Rheumatol. 2012
6. Fenômeno de RaynaudFenômeno de Raynaud
•Secundário
• DTC:
• ESCLEROSE SISTÊMICAESCLEROSE SISTÊMICA
• Miopatias infamatórias
• Lúpus Eritematoso Sistêmico
• Síndrome de Sjogren
• Vasculite
12. Esclerodermia LocalizadaEsclerodermia Localizada
• Morféia
• mais comum
• placas de pele espessada com graus variados de pigmentação
• “gutata” e “subcutânea”
• Morféia generalizada
• Área extensa
• Esclerodermia linear
• Podem apresentar envolvimento profundo da pele até atingir os músculos.
• mais comuns nas pernas e nos braços e, quando acometem as articulações, podem acarretar
limitações da função articular.
• Esclerodermia “em golpe de sabre”
• face ou o couro cabeludo
• ocasionalmente pode causar atrofia da face (inclusive língua e a boca)
19. Síndrome de Parry- Romberg
• A síndrome de Parry-Romberg, também denominada atrofia
hemifacial progressiva, consiste em uma rara condição na
qual há degeneração progressiva e encolhimento dos tecidos
situados abaixo da pele da face, incluindo, em muitos casos,
os ossos.
29. Capilaroscopia padrão SDCapilaroscopia padrão SD
a
c d
b
Figure 3| Markers of scleroderma patterns. Images obtained by routine nailfold videocapillaroscopy analysis of patients
with systemic sclerosis (magnification ×200) reveal the morphological markers of microvascular disorganization that
characterize overt systemic sclerosis and related nailfold videocapillaroscopy patterns (‘early’,‘active’ and ‘late’ systemic
sclerosis) in the presence of secondary Raynaud phenomenon. a | Giant capillaries (arrows), the earliest and most striking
morphological feature of secondary Raynaud phenomenon, are homogeneously enlarged microvascular loops that serve as
potential markers of microangiopathy. b | Microhemorrhages (arrows) result from the microvascular extravasation of red
blood cells from damaged capillaries. c | Loss of capillaries (arrows) can lead to the presence of avascular areas in the
nailfold bed and subsequent local tissue hypoxia. d | Neoangiogenesis is characterized by the clustering of twisted and
R E V I E W S
Ectasia
Micro
hemorragia
Perda de capilar
Capilar em arbusto
