Paget's disease of bone: analysis of 134 cases from an island in Southern Brazil: another cluster of Paget's disease of bone in South America.
Paget's disease of bone (PDB) exhibits a marked geographic variation. In Brazil, the prevalence of PDB is unknown and only a few clinical data are available. The aim is to determine clinical, laboratory, imaging and response to treatment data in a large PDB case series in the city of Florianopolis, Brazil. We have performed a retrospective study based on charts reviews of all patients with PDB followed at the University Hospital of the Federal University of Santa Catarina and at five different private rheumatology outpatient offices in Florianopolis, between 1995 and 2009. One hundred and thirty-four patients with PDB were identified. Mean age at diagnosis was 63.2 ± 10.5 years, 67.2% were women, and 91.1% were Caucasian. Positive family history was reported in only 8.2%. Polyostotic disease was found in 75.0% of the cases, bone pain in 77.9%, and bone deformities in 15.9%. Higher levels of AP were significantly associated with polyostotic disease and skull involvement. Pelvic bones were the most frequently affected (53.7%). Complications included deafness in 8.2%, bone fractures in 3.0%, hydrocephalus in 2.2%, and cauda equina syndrome in 0.7% of the cases. Treatment with zoledronic acid achieved the best response with only 2.9% failing to respond adequately. According to literature data, PDB in South America seems to be characterized by an overall low prevalence, but with localized clusters with higher prevalence. The authors have described a cluster of PDB in Florianopolis, in Southern Brazil. Further properly designed studies are necessary to clarify the PDB epidemiology in South America.
Rheumatology Section at the University Hospital of the Federal University of Santa Catarina, Av. Eng. Max de Souza, 1588 ap 503, Florianópolis, SC 88080-000, Brazil. castrogrwc@gmail.com
Gláucio Ricardo
GR
Werner de Castro
Gláucia Itamaro
GI
Heiden
Adriana Fontes
AF
Zimmermann
Edelton Flávio
EF
Morato
Fabrício Souza
FS
Neves
Maria Amazile
MA
Toscano
Sonia Cristina
SC
de Magalhães Souza Fialho
Ivânio Alves
IA
Pereira
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000469:Alkaline Phosphatase / Q000097:blood; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D001938:Brazil / Q000453:epidemiology; D016000:Cluster Analysis; D015897:Comorbidity; D003638:Deafness / Q000453:epidemiology; D004164:Diphosphonates / Q000627:therapeutic use; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D050723:Fractures, Bone / Q000453:epidemiology; D006801:Humans; D006849:Hydrocephalus / Q000453:epidemiology; D007093:Imidazoles / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010001:Osteitis Deformans / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D011128:Polyradiculopathy / Q000453:epidemiology; D012189:Retrospective Studies; D016896:Treatment Outcome; D000077211:Zoledronic Acid
D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D007093:Imidazoles; D000077211:Zoledronic Acid; D000469:Alkaline Phosphatase