Camurati-Engelmann disease: failure of response to bisphosphonates: report of two cases

Camurati-Engelmann disease is a rare bone disorder characterized by cortical thickening of the diaphysis of tubular bones, with sparing of the epiphysis. It has variable degrees of penetrance and expression, but may be very disabling for the affected individuals who manifest the painful symptoms. Th...

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Bibliographic Details
Published in:Clinical rheumatology 2005-08, Vol.24 (4), p.398-401
Main Authors: Castro, Glaucio R W, Appenzeller, Simone, Marques-Neto, João Francisco, Bértolo, Manoel B, Samara, Adil M, Coimbra, Ibsen
Format: Article
Language:English
Subjects:
Adult
Bone Density - physiology
Camurati-Engelmann Syndrome - diagnosis
Camurati-Engelmann Syndrome - drug therapy
Densitometry
Diphosphonates - administration & dosage
Dose-Response Relationship, Drug
Drug Administration Schedule
Female
Follow-Up Studies
Humans
Rare Diseases
Severity of Illness Index
Treatment Failure
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Summary:Camurati-Engelmann disease is a rare bone disorder characterized by cortical thickening of the diaphysis of tubular bones, with sparing of the epiphysis. It has variable degrees of penetrance and expression, but may be very disabling for the affected individuals who manifest the painful symptoms. The authors report on two women with typical presentation of severe Camurati-Engelmann disease whose treatment with bisphosphonates failed to add any improvement beyond that elicited by corticosteroids alone.
ISSN:0770-3198
1434-9949
DOI:10.1007/s10067-004-1056-7