{"id":460,"date":"2014-11-25T11:00:28","date_gmt":"2014-11-25T11:00:28","guid":{"rendered":"http:\/\/stg-blogs.bmj.com\/bmjebmspotlight\/?p=460"},"modified":"2017-08-21T12:06:31","modified_gmt":"2017-08-21T12:06:31","slug":"primary-care-corner-with-geoffrey-modest-md-paget-disease-clinical-guidelines","status":"publish","type":"post","link":"https:\/\/stg-blogs.bmj.com\/bmjebmspotlight\/2014\/11\/25\/primary-care-corner-with-geoffrey-modest-md-paget-disease-clinical-guidelines\/","title":{"rendered":"Primary Care Corner with Geoffrey Modest MD: Paget disease clinical guidelines"},"content":{"rendered":"

By: Dr. Geoffrey Modest<\/strong><\/p>\n

The Endocrine Society just released clinical practice guidelines for the diagnosis and management of Paget disease of the bone.<\/p>\n

\"Untitled\"<\/a><\/p>\n

Some background: there is a genetic component with family history in 10-20% and autosomal dominant transmission pattern, clinically rare prior to age 40, men and women affected.<\/p>\n

Basic recommendations:<\/p>\n

Diagnosis<\/p>\n

–In patient with suspected Paget, Get plain xray of suspected area. If diagnosed with Paget, then get radionuclide bone scan to assess extent of disease<\/p>\n

–If diagnosed with Paget by xray, get biochemical evaluation: alkaline phosphatase (ALP)\u00a0or more specific marker of bone formation (especially if there is hepatobiliary disease which could affect the ALP) \u2013 can check amino-terminal propeptide of type 1 collagen (P1NP, the best option but expensive) or bone-specific ALP (though there is about 20% cross-reactivity between antibodies to liver\u00a0and bone ALP)\u00a0and\u00a0bC-terminal propeptide of type 1 collagen (bCTx)\u00a0or urinary\u00a0N-terminal propeptide of type 1 collagen (NTx).<\/p>\n

\u00a0Treatment<\/p>\n

–Bisphosponates if at risk of future complications<\/p>\n

–“Suggest” a single 5-mg dose of IV zolendronate as treatment of choice, since it usually lasts\u00a0>6 years and requires less costly followup. other options include alendrontate 40mg\/d for 6 months (may need to retreat in 2-6 years) or risedronate 30 mg for 2 months\u00a0(may need to retreat in 1-5\u00a0years)<\/p>\n

–If symptoms need to be controlled urgently (see below), assess markers of short-term response to assess if there is adequate response to therapy (here it is best to use\u00a0b\u200bCTx as marker of bone resorption, since it responds more rapidly to treatment than ALP). Assess before and shortly after treatment initiation<\/p>\n

–If osteolytic lesions present, repeat xray in 1 year<\/p>\n

–Maintaining remission: check ALP or other baseline disease markers at 6-12 weeks after therapy, though maximal suppression may take 6 months.\u00a0follow bone turnover markers (should be below midpoint of assay reference range) as marker of need for retreatment. for zolendronate, check every 1-2 years. for other regimens, check every 6-12 months.<\/p>\n

–Monostotic Paget: \u00a0all of\u00a0the biochemical markers of bone turnover\u00a0may be normal even in symptomatic patients<\/p>\n

Complication Management<\/p>\n

–Hearing loss: treat with potent bisphosphonate (eg IV zolendronate) to prevent worsening<\/p>\n

–Osteoarthritis: if severe, bisphosphonates (even before joint replacement surgery). \u00a0when mild, use analgesics. often hard to know if it is the paget’s or underlying osteoarthritis causing the pain. can consider bisphophonates as well as analgesics, though data sparse.<\/p>\n

–Bowing of lower extremity: potent\u00a0bisphosphonate (eg IV zolendronate)\u200b\u00a0prior to surgery<\/p>\n

–Paralysis (with disease of the spine):\u00a0bisphosphonate with neurosurgical evaluation. usually medical therapy is sufficient (unless severe structural damage)<\/p>\n

–Neoplasm: if osteosarcoma or giant cell tumor, pretreat with \u00a0potent\u00a0bisphosphonate prior to surgery. interesting that neoplasms seem to be less frequent in this era of bisphosphonates.<\/p>\n

In reviewing financial disclosures, of the 7 members of the taskforce, 2 without conflict, 1 with declared conflicts, and 4 with financial interests in several drug companies but stated that there were no conflicts.<\/p>\n

 <\/p>\n","protected":false},"excerpt":{"rendered":"

Paget disease clinical guidelines […]<\/p>\n

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