Différences entre versions de « Connectivites »
De médecine.top
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== ''Mixed connective tissue disease'' (MCTD) == | == ''Mixed connective tissue disease'' (MCTD) == | ||
+ | Mixed connective tissue disease (MCTD) is a specific overlap syndrome that includes clinical manifestations of at least two of the following: | ||
+ | * systemic lupus erythematosus (SLE) | ||
+ | * polymyositis | ||
+ | * systemic sclerosis. | ||
+ | * <u>Marqueur:</u> '''anticorps anti-U1-ribonucléoprotéine (RNP)''' | ||
+ | |||
== ''Undifferentiated connective tissue disease'' (UCTD) == | == ''Undifferentiated connective tissue disease'' (UCTD) == | ||
+ | |||
+ | == ''Overlap syndromes'' == | ||
== Références == | == Références == | ||
[[Category:Rhumatologie]] | [[Category:Rhumatologie]] | ||
[[Category:Allergo-immunologie]] | [[Category:Allergo-immunologie]] |
Version du 13 juin 2020 à 13:51
Lupus érythémateux systémique (LES)
- Classification:
- Acute cutaneous lupus erythematosus (ACLE): rash malaire
- Subacute cutaneous lupus erythematosus (SCLE): rash photosensible des bras, cou, visage. Association avec anti-Ro et anti-SSA (> 75%)
- Discoid lupus erythematosus (DLE): forme de manifestation cutanée chronique la plus commune
- Diagnosic:
- Clinique:
- Critères de l'ACR
- Chimie:
- If antinuclear antibody testing is positive (ANA), autoantibodies specific to systemic lupus erythematosus (anti–double-stranded DNA, anti-Smith, anti-U1-ribonucleoprotein, anti-Ro/SSA, and anti-La/SSB) should be obtained to further characterize the disease.
- Test de Coombs positif
- ↓ Complément (permet le suivi de l'activité de la maladie)
- Histologie:
- Biopsie cutanée suggestive
- Clinique:
- Complications: néphrite lupique (associée à ↑ anticorps anti-dsDNA), pneumonite lupique aiguë, hémorragie alvéolaire diffuse, shrinking lung syndrome, ostéonécrose aseptique de la hanche, syndrome des anti-phospholipides
- Traitement:
- En cas de suspicion d'atteinte rénale: la ponction-biopsie du rein est essentielle pour guider l'intensité du traitement du LES
Syndrome de Sjögren
Sclérodermie
Synonyme: sclérose systémique
- Caractéristiques: 2 formes
- Association avec le phénomène de Raynaud (blanc → bleu → rouge)
- Diagnostic: capillaroscopie
- Complications:
- Crise rénale sclérodermique → traitement par inhibiteurs de l'enzyme de conversion (IEC: captopril)
- Pneumopathie interstitielle → traitement par mycophénolate de mofétil
Atteinte cutanée limitée (lcSSc)
- Marqueur: anticorps anti-centromères
- Complications: hypertension artérielle pulmonaire
- Syndrome de CREST: calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia
Atteinte cutanée diffuse (dcSSc)
- Marqueurs: anticorps anti-Scl70, anticorps anti-RNA polymérase III
Polyarthrite rhumatoïde (PAR)
- Sujet traité sur la page Arthropathies
Myopathies inflammatoires
Dermatomyosite (DM)
- Clinique: papules de Gottron
- Complications: syndrome des anticorps anti-synthétase: interstitial lung disease, myositis, Raynaud phenomenon, nonerosive inflammatory arthritis, constitutional findings such as low-grade fever, and mechanic's hands; anti-aminoacyl-tRNA synthetases antibodies, such as anti–Jo-1, are highly suggestive of the diagnosis.
Polymyosite (PM)
- Clinique: mains de mécanicien
- Complications: syndrome des anticorps anti-synthétase
Myosite à inclusion (IBM)
Syndrome des anticorps anti-synthétases
Myosite nécrosante immuno-médiée
Mixed connective tissue disease (MCTD)
Mixed connective tissue disease (MCTD) is a specific overlap syndrome that includes clinical manifestations of at least two of the following:
- systemic lupus erythematosus (SLE)
- polymyositis
- systemic sclerosis.
- Marqueur: anticorps anti-U1-ribonucléoprotéine (RNP)