title: Bone mineral density, bone turnover markers and fractures in patients with indolent systemic mastocytosis.
abstract: OBJECTIVE
  We systematically assessed bone mineral density (BMD), bone turnover markers (BTM), and fractures in a large cohort of patients with Indolent Systemic Mastocytosis (ISM).
  
  
  METHODS
  Eighty-two patients (mean age 48 years, 37 women) with ISM were studied. BMD was measured by dual X-ray absorptiometry at the lumbar spine and proximal hip. The serum markers of bone turnover included bone-specific alkaline phosphatase, C-telopeptides of type I collagen, and serum osteocalcin. Previous clinical fractures were registered and spine X-ray was obtained from all patients.
  
  
  RESULTS
  Three women were excluded for concomitant diseases associated to osteoporosis. Osteoporosis according with the WHO classification (T-score<-2.5) was found in 16 patients (20.0%) (7 females and 9 men). Mastocytosis-related low BMD (Z-score at either the spine or the hip<-2) was found in 3 women (9%) and 13 men (28%). The BMD was generally lower at the spine than at the hip. No significant correlation was observed between serum tryptase levels and T or Z-score BMD. One or more moderate or severe vertebral fractures were found in 17 patients (12 men); in 11 of them Z-score values were>-2 or not valuable at the spine. No significant difference was found in the prevalence of mastocytosis-related low BMD and/or vertebral fractures between patients with or without skin involvement. Two patients had radiographic and densitometric osteosclerosis-like characteristics. In osteoporotic patients higher, normal or lower serum BTM were found, without correlations with serum tryptase levels, while in patients with osteosclerosis both BTM and serum tryptase values were particularly increased.
  
  
  CONCLUSIONS
  Vertebral osteoporosis and fractures are frequent in patients with ISM. Spine X-ray and densitometric examination are warranted in all patients, also without skin involvement and particularly in males; Z-score other than T-score BMD must be evaluated. Patients with idiopathic osteoporosis should be evaluated for mast cell disease. Both high than low BTM can be observed in patients with osteoporosis while osteosclerosis is characterized by high bone turnover and serum tryptase levels.
authors.affiliation: Rheumatology Section, Department of Medicine, University of Verona, Italy. maurizio.rossini@univr.it
authors.forename: Maurizio
authors.initials: M
authors.lastname: Rossini
authors.forename: Roberta
authors.initials: R
authors.lastname: Zanotti
authors.forename: Patrizia
authors.initials: P
authors.lastname: Bonadonna
authors.forename: Anna
authors.initials: A
authors.lastname: Artuso
authors.forename: Beatrice
authors.initials: B
authors.lastname: Caruso
authors.forename: Donatella
authors.initials: D
authors.lastname: Schena
authors.forename: Decio
authors.initials: D
authors.lastname: Vecchiato
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Bonifacio
authors.forename: Ombretta
authors.initials: O
authors.lastname: Viapiana
authors.forename: Davide
authors.initials: D
authors.lastname: Gatti
authors.forename: Gianenrico
authors.initials: G
authors.lastname: Senna
authors.forename: Annamaria
authors.initials: A
authors.lastname: Riccio
authors.forename: Giovanni
authors.initials: G
authors.lastname: Passalacqua
authors.forename: Giovanni
authors.initials: G
authors.lastname: Pizzolo
authors.forename: Silvano
authors.initials: S
authors.lastname: Adami
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000886:Anthropometry; D015415:Biomarkers / Q000378:metabolism*; D015519:Bone Density / Q000502:physiology*; D016723:Bone Remodeling / Q000502:physiology*; D003720:Densitometry; D005260:Female; D050723:Fractures, Bone / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D034721:Mastocytosis, Systemic / Q000097:blood / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008875:Middle Aged; D010388:Pelvis / Q000000981:diagnostic imaging; D011859:Radiography; D011877:Radionuclide Imaging; D012886:Skull / Q000000981:diagnostic imaging; D013131:Spine / Q000000981:diagnostic imaging; D053802:Tryptases / Q000097:blood; D055815:Young Adult
chemical_list: D015415:Biomarkers; D053802:Tryptases