Acrosyndromes vasculaires phénomène de Raynaud prise en charge par le médecin vasculaire - Joël Constans Hôpital St André, CHU Bordeaux et ...

Acrosyndromes vasculaires
         phénomène de Raynaud
prise en charge par le médecin vasculaire
                       Joël Constans
            Hôpital St André, CHU Bordeaux et
                 Université de Bordeaux

Défini:on

Microcircula:on cutanée

                          J Constans, EMC ermato_cosmeto, 2007

Arcades palmaires

Classifica:on des acrosyndromes

Diagnos:c différen:el du phénomène de
Raynaud
• Ischémie digitale
• Hématome digital spontané
• Engelures
• acrocyanose

                              engelures   Hématome digital
                                          spontané

Recommenda)on 1:
Raynaud’s Phenomenon is the correct term for this disorder. It may take the form of
Primary Raynaud’s Phenomenon or Secondary Raynaud’s Phenomenon.

Recommenda)on 2
The terms Primary Raynaud’s Phenomenon and Secondary Raynaud’s Phenomenon should be
used and the terms ‘syndrome’ and ‘disease’ discarded.
Level C, Grade IIa

Diagnos:c de phénomène de Raynaud
• Souvent aisé
   • Ischémie des extrémités
   • ParoxysCque
   • Phases blanc, (bleu), (rouge)
• Peut être facilité par des photos
• Le principal problème
   • PrimiCf?
   • Secondaire?

Table 1 The condiCons which may be associated with secondary RP.
ConnecCve Tissue Disorders                                                    Endocrine
•         Systemic Sclerosis                                                  •Hypothyroidism
•         Systemic Lupus Erythematosus                                        •Pheochromocytoma
•         Mixed CTD
•         Sjögren’s Syndrome
•         DermatomyosiCs/PolymyosiCs                                          ParaneoplasCc (eg carcinoid)
•         Primary Biliary Cirrhosis (oYen with underlying SSc)                Miscellaneous
OccupaConal                                                                   • Buerger’s disease (ThromboangiiCs obliterans)
•         Hand Arm VibraCon Syndrome and Hypothenar Hammer Syndrome •Low BMI
•         Vinyl Chloride Monomer exposure
•         Silica and solvents (causing systemic sclerosis)
Drugs
•         AnC-migraine drugs eg ergot derivaCves
•         Non-selecCve β Blockers, including eye drops
•         Some Cytotoxic drugs
•         Cyclosporin (though may be obstrucCve especially in transplant paCents)
•         BromocripCne
•         Interferon α and β
•         Cocaine or amphetamine abuse, cannabis
•         Estrogen replacement therapy without progesterone
•         Ephedrine eg in Ear Nose and Throat preparaCons

Table 3: Condi)ons where microvascular occlusion may mimic Raynaud’s and
should be excluded
Occlusive vascular disease
•         Embolism (eg from thoracic outlet syndrome)
Haematological
•         Malignancies
•         Cryo diseases (cryoglobulinaemia, cryofibrinogenaemia and cold aggluCnin disease)
•         Hyperviscosity Syndromes
InfecCon
•         HepaCCs associated vasculiCs

                                 AggluCnines froides

Recommenda)on 3
Condi)ons associated with RP should be divided into true associated disorders with
e)ological links; those which worsen RP or precipitate its appearance; and those which do
not cause vasospasm but digital necrosis.
Level C, Grade III

Recommenda)on 4
A thorough history and examina)on should be taken from all pa)ents presen)ng in Primary
Care to ensure correct diagnosis of any underlying condi)on, as early diagnosis and organ
screening in CTD improves outcome.
Level C, Grade IIa

Critères évoquant un PR secondaire
• Absence de rémission esCvale complète
• Pouces touchés
• Survenue après 35 ans
• Phase bleue prolongée
• Caractère unilatéral persistant
• Absence de trouble trophique
• Sexe masculin

Recommenda)on 5
All pa)ents presen)ng with RP should undergo blood tests including full blood count, ESR or CRP, and
ANA tes)ng, and capillaroscopy when available.
Level C, Grade IIa
Recommenda)on 6
Capillaroscopy should only be carried out using equipment of good op)cal quality and by an
experienced operator, usually in Secondary or Ter)ary Care.
Level C, Grade IIa
Recommenda)on 7
Capillary microscopy is a useful diagnos)c tool. Abnormal capillary paTerns are strong predictors of
CTD, and should be employed by Secondary Care.
Level A, Grade IIa

Capillaroscopie: quel matériel?
• Pour faire une capillaroscopie, il faut un capillaroscope
• Videocapillaroscope= opCmal
• Capillaroscope « tradiConnel »
   • Pas de mesure de densité ou de diamètre
   • Pas de possibilité de stockage d’images
• Autres techniques?
   Mais problème de grossissement, pas de mesures, pas de valida:on
          • Capteurs opCques reliés à un système numérique
          • dermatoscope

Quel grossissement u:liser?
• Etre habitué à son grossissement
• X100 le plus répandu
• X50: meilleure vue d’ensemble du lit capillaire mais repères
  différents
• X200-350:
    • réalisa)on plus difficile
    • Quelle zone d’intérêt?
    • Plus de variabilité? Perte d’informa)ons

Conduite de l’examen
capillaroscopique
• Pièce tempérée
• Malade au calme, pas de soins de manucurie récent,
  difficultés chez les travailleurs manuels
• Posi)on assise
• GouTe d’huile sur le rebord péri-unguéal
• Examiner tous les doigts sauf le pouce, les 2 mains
• Garder des images

Différentes étapes de la capillaroscopie
• Regarder l’ensemble du paysage
   • Œdème
   • Hémorragies
   • DésorganisaCon architecturale
• Mesurer la densité capillaire
   • N≥8/mm; détecter les plages avasculaires? (

Paysage
Paysage normal   sclérodermique

Sensibility of scleroderma paIern in SSc
                                             Prevalence of SSc landscape in
                                             confirmed SSC
           Maricq 1980                       82%
           (N=173)
           Vayssairat 1981                   94%
           (N= 74)
           Granier 1986                      93%
           (N=30)
           Blockmans 1996                    73-100%
           (N=42)
           Ohtsuka 1994                      90%
           (N=55)
           Cutolo 2004                       99%
           (N=241)

              86% (N=2754): cohorte EUSTAR (Ingegnoli 2013)

   Adapted from Atlas de Capillaroscopie, Collège des Enseignants de
   Médecine Vasculaire

Follow-up of Raynaud’s phenomenon
  Series            RP        Suspect RP                      Follow-up      Confirmed CTD
                                                              years          during follow-up

  Priollet 1987     240       26 (clinics or ANA)             6              50%

  Lugen 1995        64        64 (clinics or ANA)             8              50%

  Spencer 1998      639       262 (clinics or ANA)            4              30%

  Meli 2006         1024      168 (capillaroscopy)            6.5            82%

  Koenig 2008       784       133 (capillaroscopy or ANA)     15             50%

   Adapted from Atlas de Capillaroscopie, Collège des Enseignants de Médecine Vasculaire

Rheumatology (Oxford). 2017 Oct 1;56(10):1780-1783.

Recommenda)on 8
Children under the age of 12 should be referred to Secondary Care as PRP is less
common in these age groups
Level C, Grade IIa
Recommenda)on 9
Pa)ents with RP should be referred to Secondary Care when
•   There is evidence of an associated disorder or of occlusive vascular disease
•   Symptoms are severe or progressing despite first line lifestyle and drug
    treatment
    Level C, Grade IIa

Recommenda)on 10
Lifestyle change is an effec)ve means of controlling RP aTacks and should
include avoiding triggers such as cold, dressing warmly, ceasing smoking, and
an Occupa)onal Therapy assessment for aids if difficul)es are reported.
Level C, Grade IIa
Recommenda)on 11
Calcium channel blockers are the recommended first line drug treatment
for RP, if life-style modifica)on alone has failed.
Level A, Grade 1
Recommenda)on 12
Nifedipine in slow release form should be used to minimise debilita)ng
vasodilatory side effects and short dura)on of ac)on. Care should be taken
to increase dosage by increments to avoid side effects. If side effects are
not severe pa)ents should be encouraged to tolerate them for 2 – 3 weeks
as they may subside.
Level C, Grade IIa