title: [Severe therapy refractive osteoporosis : A rare differential diagnosis].
abstract: Systemic mastocytosis is defined as a clonal increase of mast cells. We report on four patients with severe osteoporosis and histologically confirmed systemic mastocytosis. In spite of antiresorptive therapy the patients developed further vertebral fractures and suffered from ostealgia. Systemic mastocytosis is an important differential diagnosis in patients with therapy refractive and unexplained osteoporosis. Skin involvement (urticaria pigmentosa) is a rare occurrence and in most cases an isolated involvement of bone marrow is present. Determination of serum tryptase can provide indications for systemic mastocytosis but the diagnosis is only confirmed by bone marrow biopsy. There is a high risk of vertebral fractures and patients should be treated in specialized centers. Zoledronic acid can be a therapeutic option for indolent osteoporosis associated with systemic mastocytosis.
authors.affiliation: Fachklinik für Rheumatologie, Orthopädie und Osteologie, Klinik Der FÜRSTENHOF, Osteologisches Schwerpunkt- und Forschungszentrum DVO, Am Hylligen Born 7, 31812, Bad Pyrmont, Deutschland. gehlen@staatsbad-pyrmont.de.
authors.forename: M
authors.initials: M
authors.lastname: Gehlen
authors.affiliation: Fachklinik für Rheumatologie, Orthopädie und Osteologie, Klinik Der FÜRSTENHOF, Osteologisches Schwerpunkt- und Forschungszentrum DVO, Am Hylligen Born 7, 31812, Bad Pyrmont, Deutschland. lazarescu@staatsbad-pyrmont.de.
authors.forename: A D
authors.initials: AD
authors.lastname: Lazarescu
authors.affiliation: Fachklinik für Rheumatologie, Orthopädie und Osteologie, Klinik Der FÜRSTENHOF, Osteologisches Schwerpunkt- und Forschungszentrum DVO, Am Hylligen Born 7, 31812, Bad Pyrmont, Deutschland.
authors.forename: C
authors.initials: C
authors.lastname: Hinz
authors.affiliation: Fachklinik für Rheumatologie, Orthopädie und Osteologie, Klinik Der FÜRSTENHOF, Osteologisches Schwerpunkt- und Forschungszentrum DVO, Am Hylligen Born 7, 31812, Bad Pyrmont, Deutschland.
authors.forename: N
authors.initials: N
authors.lastname: Schmidt
authors.affiliation: Fachklinik für Rheumatologie, Orthopädie und Osteologie, Klinik Der FÜRSTENHOF, Osteologisches Schwerpunkt- und Forschungszentrum DVO, Am Hylligen Born 7, 31812, Bad Pyrmont, Deutschland.
authors.forename: M
authors.initials: M
authors.lastname: Pfeifer
authors.affiliation: Helios Klinikum Emil von Behring, Berlin, Deutschland.
authors.forename: M
authors.initials: M
authors.lastname: Werner
authors.affiliation: Rheumatologische Facharztpraxis Joachimstraße, Hannover, Deutschland.
authors.forename: H F
authors.initials: HF
authors.lastname: Weidemann
authors.affiliation: Campus Medizin, Evangelisches Krankenhaus, Universität Oldenburg, Oldenburg, Deutschland.
authors.forename: M
authors.initials: M
authors.lastname: Schwarz-Eywill
authors.affiliation: Nordwestdeutsches Rheumazentrum, St.-Josef-Stift Sendenhorst, Sendenhorst, Deutschland.
authors.forename: A
authors.initials: A
authors.lastname: Maier
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D002908:Chronic Disease; D003937:Diagnosis, Differential; D004164:Diphosphonates / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007093:Imidazoles / Q000627:therapeutic use*; D008297:Male; D034721:Mastocytosis, Systemic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D008875:Middle Aged; D010024:Osteoporosis / Q000175:diagnosis* / Q000188:drug therapy*; D035583:Rare Diseases / Q000175:diagnosis; D017211:Treatment Failure; D016896:Treatment Outcome; D000077211:Zoledronic Acid
chemical_list: D000893:Anti-Inflammatory Agents; D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D007093:Imidazoles; D000077211:Zoledronic Acid
keywords: Systemic mastocytosis; Therapy refractory osteoporosis; Unexplained osteoporosis; Vertebral fractures; Zoledronic acid