Paget's Disease of Bone: Diagnosis, Alkaline Phosphatase, and Treatment | Clinique Omicron<\/title>\n<meta name=\"description\" content=\"La maladie de Paget osseux est un trouble du remodelage osseux causant une d\u00e9formation et des fractures. Phosphatase alcaline tr\u00e8s \u00e9lev\u00e9e, bisphosphonates, acide zol\u00e9dronique et prise en charge au Qu\u00e9bec.\">\n<meta name=\"keywords\" content=\"maladie de Paget osseux, Paget osseux traitement, phosphatase alcaline Paget, acide zol\u00e9dronique Paget, maladie Paget sympt\u00f4mes, Paget osseux diagnostic, bisphosphonates Paget, Paget d\u00e9formation osseuse, Paget scintigraphie osseuse, Paget Qu\u00e9bec\">\n<link rel=\"preconnect\" href=\"https:\/\/fonts.googleapis.com\">\n<link href=\"https:\/\/fonts.googleapis.com\/css2?family=Cinzel:wght@600&family=Poppins:wght@400;500;600;700&display=swap\" rel=\"stylesheet\">\n<style>\n@import url('https:\/\/fonts.googleapis.com\/css2?family=Cinzel:wght@600&family=Poppins:wght@400;500;600;700&display=swap');\n\n.co-wrap * {\n font-family: 'Poppins', sans-serif;\n box-sizing: border-box;\n}\n.co-wrap {\n max-width: 1100px;\n margin: 0 auto;\n padding: 30px 0 60px;\n margin-top: 10px;\n}\n.co-label {\n font-family: 'Cinzel', serif;\n font-size: 14px;\n font-weight: bold;\n letter-spacing: 1px;\n text-transform: uppercase;\n color: #4D6577;\n margin-bottom: 14px;\n display: block;\n}\n.co-wrap h1 {\n font-size: 32px;\n font-weight: 500;\n color: #323C52;\n margin: 0 0 22px;\n line-height: 1.2;\n letter-spacing: 0.5px;\n}\n.co-intro {\n font-size: 16px;\n font-weight: 400;\n line-height: 1.75;\n color: #4D6577;\n margin-bottom: 36px;\n padding-bottom: 32px;\n border-bottom: 1px solid rgba(77,101,119,.2);\n}\n.co-wrap h2 {\n font-size: 20px;\n font-weight: 600;\n color: #323C52;\n margin: 32px 0 12px;\n letter-spacing: 0.3px;\n}\n.co-wrap p {\n font-size: 15px;\n font-weight: 400;\n color: #4D6577;\n line-height: 1.7;\n margin-bottom: 14px;\n}\n.co-list {\n list-style: none;\n padding: 0;\n margin: 12px 0 24px;\n}\n.co-list li {\n font-size: 15px;\n font-weight: 400;\n color: #4D6577;\n padding: 10px 14px 10px 38px;\n margin-bottom: 8px;\n border-radius: 6px;\n position: relative;\n background: rgba(77,101,119,.06);\n border-left: 3px solid #4D6577;\n}\n.co-list li::before {\n content: \"\u2713\";\n position: absolute;\n left: 12px;\n font-weight: 700;\n color: #4D6577;\n}\n.co-table {\n width: 100%;\n border-collapse: collapse;\n margin: 14px 0 22px;\n font-size: 14px;\n border-radius: 8px;\n overflow: hidden;\n}\n.co-table thead tr {\n background: #323C52;\n color: #fff;\n}\n.co-table thead th {\n padding: 11px 16px;\n text-align: left;\n font-weight: 600;\n font-size: 13px;\n}\n.co-table tbody tr:nth-child(even) {\n background: rgba(77,101,119,.06);\n}\n.co-table tbody tr:nth-child(odd) {\n background: #fff;\n}\n.co-table td {\n padding: 10px 16px;\n color: #4D6577;\n border-bottom: 1px solid rgba(77,101,119,.12);\n font-size: 14px;\n vertical-align: top;\n}\n.co-table td:first-child {\n font-weight: 600;\n color: #323C52;\n}\n.co-infobox {\n display: flex;\n gap: 12px;\n background: rgba(77,101,119,.06);\n border-radius: 8px;\n border-left: 4px solid #4D6577;\n padding: 14px 18px;\n margin: 18px 0 28px;\n font-size: 14px;\n font-weight: 400;\n color: #4D6577;\n line-height: 1.65;\n}\n.co-infobox .ico {\n font-size: 18px;\n flex-shrink: 0;\n}\n.co-urgence {\n background: #fff8f8;\n border-left: 5px solid #c0392b;\n border-radius: 6px;\n padding: 20px 26px;\n margin: 24px 0 32px;\n}\n.co-urgence .co-urgence-titre {\n font-size: 13px;\n font-weight: 700;\n color: #c0392b;\n letter-spacing: 1.5px;\n text-transform: uppercase;\n margin-bottom: 10px;\n}\n.co-urgence p {\n color: #5a2020;\n font-size: 14px;\n margin: 0 0 10px;\n line-height: 1.7;\n}\n.co-urgence p:last-child {\n margin-bottom: 0;\n}\n.co-disclaimer {\n font-size: 13px;\n color: #8a9aaa;\n font-style: italic;\n border-top: 1px solid rgba(77,101,119,.15);\n padding-top: 24px;\n margin-top: 40px;\n line-height: 1.6;\n}\n<\/style>\n<\/head>\n<body>\n<div class=\"co-wrap\">\n\n <span class=\"co-label\">Rhumatologie & Endocrinologie & M\u00e9decine interne<\/span>\n <h1>Bone Paget's disease<\/h1>\n\n <div class=\"co-intro\">\n La maladie de Paget osseux \u2014 d\u00e9crite par le chirurgien britannique Sir James Paget en 1877 sous le terme d'ost\u00e9ite d\u00e9formante \u2014 est une maladie du remodelage osseux chronique focale et asym\u00e9trique, caract\u00e9ris\u00e9e par une hyperactivit\u00e9 primitive des ost\u00e9oclastes dans des foyers osseux limit\u00e9s qui entra\u00eene une r\u00e9sorption osseuse acc\u00e9l\u00e9r\u00e9e et anarchique, suivie d'une r\u00e9ponse ost\u00e9oblastique compensatrice excessive qui forme un nouvel os structurellement d\u00e9sorganis\u00e9, m\u00e9caniquement fragile, hypervascularis\u00e9 et riche en collag\u00e8ne de mauvaise qualit\u00e9 \u2014 r\u00e9sultant en un os \u00e9paissi, d\u00e9form\u00e9 et pr\u00e9dispos\u00e9 aux fractures, aux douleurs et \u00e0 diverses complications locales. Elle est la deuxi\u00e8me maladie osseuse m\u00e9tabolique en fr\u00e9quence apr\u00e8s l'ost\u00e9oporose, avec une pr\u00e9valence estim\u00e9e \u00e0 2 \u00e0 5 % de la population de plus de 55 ans dans les pays anglosaxons (Grande-Bretagne, Australie, Nouvelle-Z\u00e9lande, Canada) et une incidence en diminution r\u00e9guli\u00e8re depuis les ann\u00e9es 1990 pour des raisons environnementales non encore totalement \u00e9lucid\u00e9es. La maladie est asymptomatique dans 70 \u00e0 80 % des cas et souvent d\u00e9couverte fortuitement devant une phosphatase alcaline (PAL) tr\u00e8s \u00e9lev\u00e9e sur un bilan de routine ou une d\u00e9formation osseuse visuelle. Les os les plus fr\u00e9quemment touch\u00e9s sont le bassin (65 % des cas), le rachis lombaire (50 %), le f\u00e9mur (30 %), le cr\u00e2ne (25 %) et le tibia (20 %), avec une atteinte mono-ostotique dans 15 % des cas et polyostotique dans 85 %. L'\u00e9tiologie reste partiellement inconnue mais implique une interaction entre pr\u00e9disposition g\u00e9n\u00e9tique (mutations du g\u00e8ne SQSTM1\/p62 dans 5 \u00e0 10 % des cas sporadiques et 40 \u00e0 50 % des formes familiales) et un facteur environnemental possiblement viral (paramyxovirus \u2014 rougeole, maladie de Carr\u00e9 du chien). Le traitement repose sur les bisphosphonates intraveineux \u2014 principalement l'acide zol\u00e9dronique (Aclasta\u00ae) en perfusion unique qui normalise la phosphatase alcaline dans plus de 95 % des cas avec une dur\u00e9e d'effet sup\u00e9rieure \u00e0 6 ans dans les essais cliniques \u2014 transformant le pronostic de cette maladie autrefois difficile \u00e0 traiter.\n <\/div>\n\n <h2>Physiopathologie \u2014 remodelage osseux anarchique<\/h2>\n <ul class=\"co-list\">\n <li><strong>Phase ost\u00e9olytique initiale :<\/strong> activation and massive recruitment of multinucleated giant osteoclasts (containing up to 100 nuclei vs. 3\u20135 normally) at Paget's disease sites \u2192 accelerated bone resorption at a rate 10 to 50 times higher than normal \u2192 lytic lacunae progressing along the bones (osteolysis front visible on X-ray = circumscribed osteoporotic skull + flame-shaped long bones)<\/li>\n <li><strong>Phase mixte et ost\u00e9oscl\u00e9rotique :<\/strong> Massive osteoblastic response to hyperresorption \u2192 formation of new bone of poor quality \u2192 disorganized lamellar structure in a mosaic pattern (characteristic appearance on biopsy) + marked local hypervascularization + Pagetic bone is thickened but porous and mechanically inferior to normal bone \u2192 tendency towards deformities + fractures<\/li>\n <li><strong>R\u00f4le du g\u00e8ne SQSTM1\/p62 :<\/strong> mutations in the SQSTM1 gene encoding the p62 protein (sequestosome-1) \u2192 NF-\u03baB pathway dysfunction \u2192 osteoclast precursor hyperactivation in response to RANK-L \u2192 familial or sporadic Paget's disease + mutations are autosomal dominant with variable penetrance + genetic screening offered to first-degree relatives in documented familial forms<\/li>\n <\/ul>\n\n <h2>Pr\u00e9sentation clinique selon la localisation<\/h2>\n\n <table class=\"co-table\">\n <thead>\n <tr>\n <th>Location<\/th>\n <th>Clinical manifestations<\/th>\n <th>Complications sp\u00e9cifiques<\/th>\n <\/tr>\n <\/thead>\n <tbody>\n <tr>\n <td>Pelvis (iliac bones + sacrum)<\/td>\n <td>Souvent asymptomatique + douleurs pelviennes ou lombaires diffuses + \u00e9largissement du bassin + d\u00e9formation possible + hypertrophie des ailes iliaques visible \u00e0 la radiographie<\/td>\n <td>Coxarthrose secondaire (arthrose de hanche par d\u00e9formation de l'ac\u00e9tabulum pag\u00e9tique) + fracture du col f\u00e9moral ou du cotyle + compression vasculaire rare<\/td>\n <\/tr>\n <tr>\n <td>Rachis lombo-sacr\u00e9<\/td>\n <td>Lombalgie chronique + hypertrophie des corps vert\u00e9braux et des p\u00e9dicules + \u00e9paississement du mur post\u00e9rieur des vert\u00e8bres \u2192 st\u00e9nose canalaire lombaire \u2192 claudication neurog\u00e8ne + syndrome de la queue de cheval dans les formes s\u00e9v\u00e8res<\/td>\n <td>St\u00e9nose du canal vert\u00e9bral \u2192 d\u00e9ficit neurologique des membres inf\u00e9rieurs + troubles sphinct\u00e9riens + fracture vertebrale par insuffisance + compression radiculaire<\/td>\n <\/tr>\n <tr>\n <td>F\u00e9mur<\/td>\n <td>Douleurs de cuisse + incurvation ant\u00e9ro-lat\u00e9rale du f\u00e9mur (d\u00e9formation en crosse de hockey) + jambe arqu\u00e9e + in\u00e9galit\u00e9 de longueur des membres inf\u00e9rieurs<\/td>\n <td>Fractures en fissure ou compl\u00e8tes du cortex convexe (fractures bananes ou en craie) + arthrose du genou (gonarthrose secondaire) + transformation sarcomateuse (rare \u2014 < 1 %)<\/td>\n <\/tr>\n <tr>\n <td>Tibia<\/td>\n <td>D\u00e9formation ant\u00e9rieure du tibia (jambe en lame de sabre) + \u00e9largissement + douleurs \u00e0 la palpation + chaleur locale (hypervascularisation)<\/td>\n <td>Fractures en fissure corticale + gonarthrose + enth\u00e9sopathies locales<\/td>\n <\/tr>\n <tr>\n <td>Cr\u00e2ne<\/td>\n <td>\u00c9largissement de la bo\u00eete cr\u00e2nienne (chapeau devenant trop petit) + pro\u00e9minence des bosses frontales + c\u00e9phal\u00e9es diffuses + aspect caract\u00e9ristique \u00e0 la radiographie : \u00ab cr\u00e2ne en coton \u00bb (cotton wool skull) + ost\u00e9oporose circumscripta initiale<\/td>\n <td>Surdit\u00e9 de conduction ou mixte (compression ossiculaire ou du nerf VIII dans le canal osseux) + compression des nerfs cr\u00e2niens (V + VII + VIII++) + hydroc\u00e9phalie par compression des foramens + invagination basilaire (impression basilaire) + tr\u00e8s rarement sarcome<\/td>\n <\/tr>\n <tr>\n <td>Face et m\u00e2choires<\/td>\n <td>\u00c9largissement du maxillaire sup\u00e9rieur ou de la mandibule + malocclusion dentaire + difficult\u00e9s de mastication + aspect lion de la face (faci\u00e8s l\u00e9onin) dans les formes s\u00e9v\u00e8res<\/td>\n <td>Troubles de la mastication + malocclusion + probl\u00e8mes proth\u00e9tiques dentaires + compression nasale<\/td>\n <\/tr>\n <\/tbody>\n <\/table>\n\n <h2>Biological diagnosis and imaging<\/h2>\n <ul class=\"co-list\">\n <li><strong>Phosphatase alcaline totale (PAL) \u2014 marqueur cl\u00e9 :<\/strong> very high in active Paget's disease (often 5 to 20 times normal) + bone-specific ALP is even more sensitive and specific (excludes liver causes of elevated total ALP) + correlates with the extent and activity of the disease + decreases with treatment \u2192 marker of therapeutic monitoring + normal ALP in a suspicious clinical context should prompt measurement of bone-specific ALP and P1NP<\/li>\n <li><strong>P1NP and CTX (bone remodeling markers):<\/strong> very high in active Paget's disease + P1NP (formation) and CTX (resorption) can reach 10 to 30 times normal + more sensitive than total ALP for mono-ostotic or limited forms + useful for monitoring treatment response (normalization with bisphosphonates)<\/li>\n <li><strong>Calc\u00e9mie et phosphat\u00e9mie :<\/strong> normal in uncomplicated Paget's disease + hypercalcemia possible with prolonged immobilization (bone release of calcium without compensatory formation) + to be measured before any bisphosphonate treatment (contraindicated in case of hypocalcemia)<\/li>\n <li><strong>Standard radiography :<\/strong> Initial diagnostic confirmation test + characteristic appearance according to stage: circumscript osteoporotic bone (skull) + flame-like forehead (long bones) + cortical thickening + cotton-wool appearance of long bones and skull + deformities + fissured fractures + widening of bone diameter<\/li>\n <li><strong>Scintigraphie osseuse au techn\u00e9tium-99m :<\/strong> Intense and characteristic hyperfixation of Paget's foci + complete mapping of disease extent + near-total sensitivity (99 %) + moderate specificity (osseous metastases also show hyperfixation) + to be performed at diagnosis to establish the complete bone scan + very useful for deciding if treatment is indicated (disease extent)<\/li>\n <li><strong>Scanner and MRI:<\/strong> MRI indicated in case of neurological symptoms (vertebral canal stenosis + nerve compression) + CT scan useful for planning surgery + or if sarcomatous transformation is suspected (mass of soft tissues adjacent to a Paget's bone + aggressive bone lysis)<\/li>\n <\/ul>\n\n <div class=\"co-infobox\">\n <span class=\"ico\">\u2139\ufe0f<\/span>\n <span>La transformation sarcomateuse de l'os pag\u00e9tique est la complication la plus redout\u00e9e \u2014 elle survient dans moins de 1 % des cas de maladie de Paget mais repr\u00e9sente n\u00e9anmoins 20 \u00e0 30 % des ost\u00e9osarcomes de l'adulte de plus de 60 ans. Elle doit \u00eatre suspect\u00e9e devant : aggravation brutale des douleurs osseuses dans un territoire pag\u00e9tique stable + apparition d'une masse des parties molles ou d'un gonflement rapide + nouvelle l\u00e9sion lytique ou destruction corticale agressive \u00e0 l'imagerie + \u00e9l\u00e9vation tr\u00e8s marqu\u00e9e et rapide du LDH. Le pronostic est tr\u00e8s p\u00e9joratif (survie m\u00e9diane de 6 \u00e0 12 mois).<\/span>\n <\/div>\n\n <h2>Treatment<\/h2>\n <ul class=\"co-list\">\n <li><strong>Treatment indications<\/strong> High alkaline phosphatase (active disease) + Paget's disease-related bone pain + bone involvement in areas at risk of complications (skull + spine + weight-bearing bones + adjacent to a major joint) + preparation for orthopedic surgery on Pagetic bone (reduction of intraoperative bleeding) + immobilization hypercalcemia<\/li>\n <li><strong>Acide zol\u00e9dronique (Aclasta\u00ae) 5 mg IV \u2014 traitement de r\u00e9f\u00e9rence :<\/strong> perfusion IV unique de 5 mg sur 15 minutes + normalisation de la PAL dans 95 % des patients + dur\u00e9e d'effet sup\u00e9rieure \u00e0 6 ans (essai PRISM \u2014 Reid 2011 NEJM) + sup\u00e9rieur au ris\u00e9dronate oral (normalisation PAL 96 % vs 74 %) + administration unique \u2192 excellente observance + pr\u00e9m\u00e9dication syst\u00e9matique : parac\u00e9tamol 1 g + hydratation IV ou orale abondante (pr\u00e9vention de la n\u00e9phrotoxicit\u00e9) + syndrome pseudo-grippal dans les 24\u201348 h post-perfusion (fr\u00e9quent \u00e0 la premi\u00e8re administration) + contre-indiqu\u00e9 si DFG < 35 mL\/min + hypocalc\u00e9mie non corrig\u00e9e<\/li>\n <li><strong>Ris\u00e9dronate (Actonel\u00ae) oral \u2014 alternative :<\/strong> 30 mg\/day \u00d7 2 months orally + fasting with a large glass of water + remain standing for 30 minutes after taking + less effective than IV zoledronic acid but a valid option if there is a contraindication or refusal of IV infusion + requires strict daily adherence for 2 months<\/li>\n <li><strong>Pr\u00e9vention de l'hypocalc\u00e9mie post-traitement :<\/strong> Systematic supplementation with calcium (1,000\u20131,500 mg\/day) and vitamin D (800\u20132,000 IU\/day) at least 2 weeks before and during treatment + rapid remineralization after blocking pagetic resorption can induce severe hypocalcemia if calcium and vitamin D reserves are insufficient.<\/li>\n <li><strong>Post-treatment follow-up:<\/strong> PAL dosage at 3 and 6 months post-treatment \u2192 normalization check + if PAL is normal: annual monitoring without new treatment + if PAL does not normalize or relapses after normalization \u2192 consider a second treatment + CTX and P1NP decrease faster than PAL (from day 30) and can guide early response monitoring<\/li>\n <\/ul>\n\n <div class=\"co-urgence\">\n <div class=\"co-urgence-titre\">Situations requiring prompt medical attention<\/div>\n <p>Consulter un m\u00e9decin ou un rhumatologue rapidement si un patient avec maladie de Paget connue pr\u00e9sente une aggravation brutale et inexpliqu\u00e9e des douleurs dans un foyer osseux pag\u00e9tique stable \u2014 cette pr\u00e9sentation doit faire \u00e9voquer une fracture pathologique ou une transformation sarcomateuse. De m\u00eame, si une surdit\u00e9 progressive ou des sympt\u00f4mes neurologiques (faiblesse des membres + troubles mictionnels) apparaissent dans une maladie de Paget cr\u00e2nienne ou rachidienne, une consultation neurologique ou ORL urgente est indiqu\u00e9e.<\/p>\n <p>Pour le bilan d'une phosphatase alcaline tr\u00e8s \u00e9lev\u00e9e, la prescription de la scintigraphie osseuse et des radiographies des zones suspectes, le dosage de la PAL osseuse et du P1NP, et l'initiation du traitement par acide zol\u00e9dronique, Clinique Omicron offre des consultations m\u00e9dicales dans ses points de service au Qu\u00e9bec et en t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, visitez <a href=\"https:\/\/cliniqueomicron.ca\" style=\"color:#c0392b;font-weight:600;text-decoration:none;\">cliniqueomicron.ca<\/a>.<\/p>\n <\/div>\n\n <h2>Consult at Clinique Omicron<\/h2>\n <p>Les m\u00e9decins et infirmiers praticiens sp\u00e9cialis\u00e9s (IPS) de Clinique Omicron \u00e9valuent les patients avec une phosphatase alcaline isol\u00e9ment tr\u00e8s \u00e9lev\u00e9e \u2014 premier signal d'une maladie de Paget asymptomatique \u2014 prescrivent la scintigraphie osseuse et les radiographies cibl\u00e9es, initient la suppl\u00e9mentation en calcium et vitamine D pr\u00e9alable au traitement, et orientent vers la rhumatologie ou l'endocrinologie pour l'administration de l'acide zol\u00e9dronique intraveineux et le suivi sp\u00e9cialis\u00e9. Des consultations sont disponibles dans plusieurs points de service au Qu\u00e9bec et en t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, visitez <a href=\"https:\/\/cliniqueomicron.ca\" style=\"color:#4D6577;font-weight:600;text-decoration:none;\">cliniqueomicron.ca<\/a>.<\/p>\n\n <p class=\"co-disclaimer\">Le contenu de cette page est fourni \u00e0 titre informatif uniquement et ne remplace pas l'avis d'un m\u00e9decin ou d'un rhumatologue. La maladie de Paget osseux requiert un suivi sp\u00e9cialis\u00e9 pour \u00e9valuer l'\u00e9tendue des l\u00e9sions par scintigraphie, choisir les indications de traitement et surveiller la r\u00e9ponse \u00e0 long terme.<\/p>\n\n<\/div>\n<\/body>\n<\/html>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>","protected":false},"excerpt":{"rendered":"<p>Maladie de Paget osseux : diagnostic, phosphatase alcaline et traitement | Clinique Omicron Rhumatologie & Endocrinologie & M\u00e9decine interne Maladie de Paget osseux La maladie de Paget osseux \u2014 d\u00e9crite par le chirurgien britannique Sir James Paget en 1877 sous le terme d’ost\u00e9ite d\u00e9formante \u2014 est une maladie du remodelage osseux chronique focale et asym\u00e9trique,… <a href=\"https:\/\/cliniqueomicron.ca\/en\/maladie-paget\/\" rel=\"bookmark\">Read More \"<span class=\"screen-reader-text\">Paget's Disease of Bone: Diagnosis, Alkaline Phosphatase, and Treatment | Clinique Omicron<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"om_disable_all_campaigns":false,"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"neve_meta_sidebar":"","neve_meta_container":"","neve_meta_enable_content_width":"off","neve_meta_content_width":100,"neve_meta_title_alignment":"","neve_meta_author_avatar":"","neve_post_elements_order":"","neve_meta_disable_header":"","neve_meta_disable_footer":"","neve_meta_disable_title":"","_themeisle_gutenberg_block_has_review":false,"_metasync_otto_title":"Paget (maladie osseuse de) | Brossard | Clinique Omicron","_metasync_otto_description":"La maladie de Paget osseux est un trouble du remodelage osseux avec PAL tr\u00e8s \u00e9lev\u00e9e. Cr\u00e2ne en coton, d\u00e9formation tibiale, acide zol\u00e9dronique Aclasta perfusio...","_metasync_otto_keywords":"","_metasync_otto_og_title":"Maladie de Paget osseux : | Brossard | Clinique Omicron","_metasync_otto_og_description":"Paget (maladie osseuse de) Paget (maladie osseuse de) est une condition m\u00e9dicale qui peut n\u00e9cessiter une \u00e9valuation et un suivi m\u00e9dical appropri\u00e9s. Nos profe...","_metasync_otto_twitter_title":"Maladie de Paget osseux : | Brossard | Clinique Omicron","_metasync_otto_twitter_description":"Paget (maladie osseuse de) Paget (maladie osseuse de) est une condition m\u00e9dicale qui peut n\u00e9cessiter une \u00e9valuation et un suivi m\u00e9dical appropri\u00e9s. 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\n\t\t\t\t\t\n\n\n\n\nPaget's Disease of Bone: Diagnosis, Alkaline Phosphatase, and Treatment | Clinique Omicron<\/title>\n<meta name=\"description\" content=\"La maladie de Paget osseux est un trouble du remodelage osseux causant une d\u00e9formation et des fractures. 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Elle est la deuxi\u00e8me maladie osseuse m\u00e9tabolique en fr\u00e9quence apr\u00e8s l'ost\u00e9oporose, avec une pr\u00e9valence estim\u00e9e \u00e0 2 \u00e0 5 % de la population de plus de 55 ans dans les pays anglosaxons (Grande-Bretagne, Australie, Nouvelle-Z\u00e9lande, Canada) et une incidence en diminution r\u00e9guli\u00e8re depuis les ann\u00e9es 1990 pour des raisons environnementales non encore totalement \u00e9lucid\u00e9es. La maladie est asymptomatique dans 70 \u00e0 80 % des cas et souvent d\u00e9couverte fortuitement devant une phosphatase alcaline (PAL) tr\u00e8s \u00e9lev\u00e9e sur un bilan de routine ou une d\u00e9formation osseuse visuelle. Les os les plus fr\u00e9quemment touch\u00e9s sont le bassin (65 % des cas), le rachis lombaire (50 %), le f\u00e9mur (30 %), le cr\u00e2ne (25 %) et le tibia (20 %), avec une atteinte mono-ostotique dans 15 % des cas et polyostotique dans 85 %. L'\u00e9tiologie reste partiellement inconnue mais implique une interaction entre pr\u00e9disposition g\u00e9n\u00e9tique (mutations du g\u00e8ne SQSTM1\/p62 dans 5 \u00e0 10 % des cas sporadiques et 40 \u00e0 50 % des formes familiales) et un facteur environnemental possiblement viral (paramyxovirus \u2014 rougeole, maladie de Carr\u00e9 du chien). Le traitement repose sur les bisphosphonates intraveineux \u2014 principalement l'acide zol\u00e9dronique (Aclasta\u00ae) en perfusion unique qui normalise la phosphatase alcaline dans plus de 95 % des cas avec une dur\u00e9e d'effet sup\u00e9rieure \u00e0 6 ans dans les essais cliniques \u2014 transformant le pronostic de cette maladie autrefois difficile \u00e0 traiter.\n <\/div>\n\n <h2>Physiopathologie \u2014 remodelage osseux anarchique<\/h2>\n <ul class=\"co-list\">\n <li><strong>Phase ost\u00e9olytique initiale :<\/strong> activation and massive recruitment of multinucleated giant osteoclasts (containing up to 100 nuclei vs. 3\u20135 normally) at Paget's disease sites \u2192 accelerated bone resorption at a rate 10 to 50 times higher than normal \u2192 lytic lacunae progressing along the bones (osteolysis front visible on X-ray = circumscribed osteoporotic skull + flame-shaped long bones)<\/li>\n <li><strong>Phase mixte et ost\u00e9oscl\u00e9rotique :<\/strong> Massive osteoblastic response to hyperresorption \u2192 formation of new bone of poor quality \u2192 disorganized lamellar structure in a mosaic pattern (characteristic appearance on biopsy) + marked local hypervascularization + Pagetic bone is thickened but porous and mechanically inferior to normal bone \u2192 tendency towards deformities + fractures<\/li>\n <li><strong>R\u00f4le du g\u00e8ne SQSTM1\/p62 :<\/strong> mutations in the SQSTM1 gene encoding the p62 protein (sequestosome-1) \u2192 NF-\u03baB pathway dysfunction \u2192 osteoclast precursor hyperactivation in response to RANK-L \u2192 familial or sporadic Paget's disease + mutations are autosomal dominant with variable penetrance + genetic screening offered to first-degree relatives in documented familial forms<\/li>\n <\/ul>\n\n <h2>Pr\u00e9sentation clinique selon la localisation<\/h2>\n\n <table class=\"co-table\">\n <thead>\n <tr>\n <th>Location<\/th>\n <th>Clinical manifestations<\/th>\n <th>Complications sp\u00e9cifiques<\/th>\n <\/tr>\n <\/thead>\n <tbody>\n <tr>\n <td>Pelvis (iliac bones + sacrum)<\/td>\n <td>Souvent asymptomatique + douleurs pelviennes ou lombaires diffuses + \u00e9largissement du bassin + d\u00e9formation possible + hypertrophie des ailes iliaques visible \u00e0 la radiographie<\/td>\n <td>Coxarthrose secondaire (arthrose de hanche par d\u00e9formation de l'ac\u00e9tabulum pag\u00e9tique) + fracture du col f\u00e9moral ou du cotyle + compression vasculaire rare<\/td>\n <\/tr>\n <tr>\n <td>Rachis lombo-sacr\u00e9<\/td>\n <td>Lombalgie chronique + hypertrophie des corps vert\u00e9braux et des p\u00e9dicules + \u00e9paississement du mur post\u00e9rieur des vert\u00e8bres \u2192 st\u00e9nose canalaire lombaire \u2192 claudication neurog\u00e8ne + syndrome de la queue de cheval dans les formes s\u00e9v\u00e8res<\/td>\n <td>St\u00e9nose du canal vert\u00e9bral \u2192 d\u00e9ficit neurologique des membres inf\u00e9rieurs + troubles sphinct\u00e9riens + fracture vertebrale par insuffisance + compression radiculaire<\/td>\n <\/tr>\n <tr>\n <td>F\u00e9mur<\/td>\n <td>Douleurs de cuisse + incurvation ant\u00e9ro-lat\u00e9rale du f\u00e9mur (d\u00e9formation en crosse de hockey) + jambe arqu\u00e9e + in\u00e9galit\u00e9 de longueur des membres inf\u00e9rieurs<\/td>\n <td>Fractures en fissure ou compl\u00e8tes du cortex convexe (fractures bananes ou en craie) + arthrose du genou (gonarthrose secondaire) + transformation sarcomateuse (rare \u2014 < 1 %)<\/td>\n <\/tr>\n <tr>\n <td>Tibia<\/td>\n <td>D\u00e9formation ant\u00e9rieure du tibia (jambe en lame de sabre) + \u00e9largissement + douleurs \u00e0 la palpation + chaleur locale (hypervascularisation)<\/td>\n <td>Fractures en fissure corticale + gonarthrose + enth\u00e9sopathies locales<\/td>\n <\/tr>\n <tr>\n <td>Cr\u00e2ne<\/td>\n <td>\u00c9largissement de la bo\u00eete cr\u00e2nienne (chapeau devenant trop petit) + pro\u00e9minence des bosses frontales + c\u00e9phal\u00e9es diffuses + aspect caract\u00e9ristique \u00e0 la radiographie : \u00ab cr\u00e2ne en coton \u00bb (cotton wool skull) + ost\u00e9oporose circumscripta initiale<\/td>\n <td>Surdit\u00e9 de conduction ou mixte (compression ossiculaire ou du nerf VIII dans le canal osseux) + compression des nerfs cr\u00e2niens (V + VII + VIII++) + hydroc\u00e9phalie par compression des foramens + invagination basilaire (impression basilaire) + tr\u00e8s rarement sarcome<\/td>\n <\/tr>\n <tr>\n <td>Face et m\u00e2choires<\/td>\n <td>\u00c9largissement du maxillaire sup\u00e9rieur ou de la mandibule + malocclusion dentaire + difficult\u00e9s de mastication + aspect lion de la face (faci\u00e8s l\u00e9onin) dans les formes s\u00e9v\u00e8res<\/td>\n <td>Troubles de la mastication + malocclusion + probl\u00e8mes proth\u00e9tiques dentaires + compression nasale<\/td>\n <\/tr>\n <\/tbody>\n <\/table>\n\n <h2>Biological diagnosis and imaging<\/h2>\n <ul class=\"co-list\">\n <li><strong>Phosphatase alcaline totale (PAL) \u2014 marqueur cl\u00e9 :<\/strong> very high in active Paget's disease (often 5 to 20 times normal) + bone-specific ALP is even more sensitive and specific (excludes liver causes of elevated total ALP) + correlates with the extent and activity of the disease + decreases with treatment \u2192 marker of therapeutic monitoring + normal ALP in a suspicious clinical context should prompt measurement of bone-specific ALP and P1NP<\/li>\n <li><strong>P1NP and CTX (bone remodeling markers):<\/strong> very high in active Paget's disease + P1NP (formation) and CTX (resorption) can reach 10 to 30 times normal + more sensitive than total ALP for mono-ostotic or limited forms + useful for monitoring treatment response (normalization with bisphosphonates)<\/li>\n <li><strong>Calc\u00e9mie et phosphat\u00e9mie :<\/strong> normal in uncomplicated Paget's disease + hypercalcemia possible with prolonged immobilization (bone release of calcium without compensatory formation) + to be measured before any bisphosphonate treatment (contraindicated in case of hypocalcemia)<\/li>\n <li><strong>Standard radiography :<\/strong> Initial diagnostic confirmation test + characteristic appearance according to stage: circumscript osteoporotic bone (skull) + flame-like forehead (long bones) + cortical thickening + cotton-wool appearance of long bones and skull + deformities + fissured fractures + widening of bone diameter<\/li>\n <li><strong>Scintigraphie osseuse au techn\u00e9tium-99m :<\/strong> Intense and characteristic hyperfixation of Paget's foci + complete mapping of disease extent + near-total sensitivity (99 %) + moderate specificity (osseous metastases also show hyperfixation) + to be performed at diagnosis to establish the complete bone scan + very useful for deciding if treatment is indicated (disease extent)<\/li>\n <li><strong>Scanner and MRI:<\/strong> MRI indicated in case of neurological symptoms (vertebral canal stenosis + nerve compression) + CT scan useful for planning surgery + or if sarcomatous transformation is suspected (mass of soft tissues adjacent to a Paget's bone + aggressive bone lysis)<\/li>\n <\/ul>\n\n <div class=\"co-infobox\">\n <span class=\"ico\">\u2139\ufe0f<\/span>\n <span>La transformation sarcomateuse de l'os pag\u00e9tique est la complication la plus redout\u00e9e \u2014 elle survient dans moins de 1 % des cas de maladie de Paget mais repr\u00e9sente n\u00e9anmoins 20 \u00e0 30 % des ost\u00e9osarcomes de l'adulte de plus de 60 ans. Elle doit \u00eatre suspect\u00e9e devant : aggravation brutale des douleurs osseuses dans un territoire pag\u00e9tique stable + apparition d'une masse des parties molles ou d'un gonflement rapide + nouvelle l\u00e9sion lytique ou destruction corticale agressive \u00e0 l'imagerie + \u00e9l\u00e9vation tr\u00e8s marqu\u00e9e et rapide du LDH. Le pronostic est tr\u00e8s p\u00e9joratif (survie m\u00e9diane de 6 \u00e0 12 mois).<\/span>\n <\/div>\n\n <h2>Treatment<\/h2>\n <ul class=\"co-list\">\n <li><strong>Treatment indications<\/strong> High alkaline phosphatase (active disease) + Paget's disease-related bone pain + bone involvement in areas at risk of complications (skull + spine + weight-bearing bones + adjacent to a major joint) + preparation for orthopedic surgery on Pagetic bone (reduction of intraoperative bleeding) + immobilization hypercalcemia<\/li>\n <li><strong>Acide zol\u00e9dronique (Aclasta\u00ae) 5 mg IV \u2014 traitement de r\u00e9f\u00e9rence :<\/strong> perfusion IV unique de 5 mg sur 15 minutes + normalisation de la PAL dans 95 % des patients + dur\u00e9e d'effet sup\u00e9rieure \u00e0 6 ans (essai PRISM \u2014 Reid 2011 NEJM) + sup\u00e9rieur au ris\u00e9dronate oral (normalisation PAL 96 % vs 74 %) + administration unique \u2192 excellente observance + pr\u00e9m\u00e9dication syst\u00e9matique : parac\u00e9tamol 1 g + hydratation IV ou orale abondante (pr\u00e9vention de la n\u00e9phrotoxicit\u00e9) + syndrome pseudo-grippal dans les 24\u201348 h post-perfusion (fr\u00e9quent \u00e0 la premi\u00e8re administration) + contre-indiqu\u00e9 si DFG < 35 mL\/min + hypocalc\u00e9mie non corrig\u00e9e<\/li>\n <li><strong>Ris\u00e9dronate (Actonel\u00ae) oral \u2014 alternative :<\/strong> 30 mg\/day \u00d7 2 months orally + fasting with a large glass of water + remain standing for 30 minutes after taking + less effective than IV zoledronic acid but a valid option if there is a contraindication or refusal of IV infusion + requires strict daily adherence for 2 months<\/li>\n <li><strong>Pr\u00e9vention de l'hypocalc\u00e9mie post-traitement :<\/strong> Systematic supplementation with calcium (1,000\u20131,500 mg\/day) and vitamin D (800\u20132,000 IU\/day) at least 2 weeks before and during treatment + rapid remineralization after blocking pagetic resorption can induce severe hypocalcemia if calcium and vitamin D reserves are insufficient.<\/li>\n <li><strong>Post-treatment follow-up:<\/strong> PAL dosage at 3 and 6 months post-treatment \u2192 normalization check + if PAL is normal: annual monitoring without new treatment + if PAL does not normalize or relapses after normalization \u2192 consider a second treatment + CTX and P1NP decrease faster than PAL (from day 30) and can guide early response monitoring<\/li>\n <\/ul>\n\n <div class=\"co-urgence\">\n <div class=\"co-urgence-titre\">Situations requiring prompt medical attention<\/div>\n <p>Consulter un m\u00e9decin ou un rhumatologue rapidement si un patient avec maladie de Paget connue pr\u00e9sente une aggravation brutale et inexpliqu\u00e9e des douleurs dans un foyer osseux pag\u00e9tique stable \u2014 cette pr\u00e9sentation doit faire \u00e9voquer une fracture pathologique ou une transformation sarcomateuse. De m\u00eame, si une surdit\u00e9 progressive ou des sympt\u00f4mes neurologiques (faiblesse des membres + troubles mictionnels) apparaissent dans une maladie de Paget cr\u00e2nienne ou rachidienne, une consultation neurologique ou ORL urgente est indiqu\u00e9e.<\/p>\n <p>Pour le bilan d'une phosphatase alcaline tr\u00e8s \u00e9lev\u00e9e, la prescription de la scintigraphie osseuse et des radiographies des zones suspectes, le dosage de la PAL osseuse et du P1NP, et l'initiation du traitement par acide zol\u00e9dronique, Clinique Omicron offre des consultations m\u00e9dicales dans ses points de service au Qu\u00e9bec et en t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, visitez <a href=\"https:\/\/cliniqueomicron.ca\" style=\"color:#c0392b;font-weight:600;text-decoration:none;\">cliniqueomicron.ca<\/a>.<\/p>\n <\/div>\n\n <h2>Consult at Clinique Omicron<\/h2>\n <p>Les m\u00e9decins et infirmiers praticiens sp\u00e9cialis\u00e9s (IPS) de Clinique Omicron \u00e9valuent les patients avec une phosphatase alcaline isol\u00e9ment tr\u00e8s \u00e9lev\u00e9e \u2014 premier signal d'une maladie de Paget asymptomatique \u2014 prescrivent la scintigraphie osseuse et les radiographies cibl\u00e9es, initient la suppl\u00e9mentation en calcium et vitamine D pr\u00e9alable au traitement, et orientent vers la rhumatologie ou l'endocrinologie pour l'administration de l'acide zol\u00e9dronique intraveineux et le suivi sp\u00e9cialis\u00e9. Des consultations sont disponibles dans plusieurs points de service au Qu\u00e9bec et en t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, visitez <a href=\"https:\/\/cliniqueomicron.ca\" style=\"color:#4D6577;font-weight:600;text-decoration:none;\">cliniqueomicron.ca<\/a>.<\/p>\n\n <p class=\"co-disclaimer\">Le contenu de cette page est fourni \u00e0 titre informatif uniquement et ne remplace pas l'avis d'un m\u00e9decin ou d'un rhumatologue. La maladie de Paget osseux requiert un suivi sp\u00e9cialis\u00e9 pour \u00e9valuer l'\u00e9tendue des l\u00e9sions par scintigraphie, choisir les indications de traitement et surveiller la r\u00e9ponse \u00e0 long terme.<\/p>\n\n<\/div>\n<\/body>\n<\/html>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>","protected":false},"excerpt":{"rendered":"<p>Maladie de Paget osseux : diagnostic, phosphatase alcaline et traitement | Clinique Omicron Rhumatologie & Endocrinologie & M\u00e9decine interne Maladie de Paget osseux La maladie de Paget osseux \u2014 d\u00e9crite par le chirurgien britannique Sir James Paget en 1877 sous le terme d’ost\u00e9ite d\u00e9formante \u2014 est une maladie du remodelage osseux chronique focale et asym\u00e9trique,… <a href=\"https:\/\/cliniqueomicron.ca\/en\/maladie-paget\/\" rel=\"bookmark\">Read More \"<span class=\"screen-reader-text\">Paget's Disease of Bone: Diagnosis, Alkaline Phosphatase, and Treatment | Clinique Omicron<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"om_disable_all_campaigns":false,"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"neve_meta_sidebar":"","neve_meta_container":"","neve_meta_enable_content_width":"off","neve_meta_content_width":100,"neve_meta_title_alignment":"","neve_meta_author_avatar":"","neve_post_elements_order":"","neve_meta_disable_header":"","neve_meta_disable_footer":"","neve_meta_disable_title":"","_themeisle_gutenberg_block_has_review":false,"_metasync_otto_title":"Paget (maladie osseuse de) | Brossard | Clinique Omicron","_metasync_otto_description":"La maladie de Paget osseux est un trouble du remodelage osseux avec PAL tr\u00e8s \u00e9lev\u00e9e. Cr\u00e2ne en coton, d\u00e9formation tibiale, acide zol\u00e9dronique Aclasta perfusio...","_metasync_otto_keywords":"","_metasync_otto_og_title":"Maladie de Paget osseux : | Brossard | Clinique Omicron","_metasync_otto_og_description":"Paget (maladie osseuse de) Paget (maladie osseuse de) est une condition m\u00e9dicale qui peut n\u00e9cessiter une \u00e9valuation et un suivi m\u00e9dical appropri\u00e9s. Nos profe...","_metasync_otto_twitter_title":"Maladie de Paget osseux : | Brossard | Clinique Omicron","_metasync_otto_twitter_description":"Paget (maladie osseuse de) Paget (maladie osseuse de) est une condition m\u00e9dicale qui peut n\u00e9cessiter une \u00e9valuation et un suivi m\u00e9dical appropri\u00e9s. Nos profe...","rank_math_title":"","rank_math_description":"","_yoast_wpseo_title":"","_yoast_wpseo_metadesc":"","_aioseo_title":"Maladie de Paget osseux : diagnostic, phosphatase alcaline et traitement | Clinique Omicron","_aioseo_description":"La maladie de Paget osseux est un trouble du remodelage osseux avec PAL tr\u00e8s \u00e9lev\u00e9e. 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