title: Severe osteoporosis due to systemic mast cell disease: successful treatment with interferon alpha-2B.
abstract: We describe a 33-year old man suffering from severe vertebral osteoporosis and urticaria pigmentosa due to systemic mast cell disease (SMCD). Because i.v. clodronate therapy could not prevent further vertebral fractures, an additional treatment with interferon alpha-2b was initiated. During 24 months of treatment, our patient had no further pain episodes, no new vertebral fractures were discovered, trabecular bone mineral density (BMD) increased significantly and urticarial symptoms improved. Nevertheless, the extent of skin lesions remained unchanged. On histological examination, a remarkable decrease of mast cells was observed in the bone marrow, but not in the skin. Five months after discontinuation of interferon alpha-2b, trabecular BMD decreased and urticarial symptoms deteriorated. These findings illustrate a beneficial effect of interferon alpha-2b on SMCD-induced osteoporosis as well as urticarial symptoms, and raise the question whether this treatment may have a diverse impact on mast cell population in different tissues.
authors.affiliation: Division of Rheumatology, University Hospital, Berne, Switzerland.
authors.forename: T
authors.initials: T
authors.lastname: Lehmann
authors.forename: C
authors.initials: C
authors.lastname: Beyeler
authors.forename: B
authors.initials: B
authors.lastname: Lämmle
authors.forename: T
authors.initials: T
authors.lastname: Hunziker
authors.forename: P
authors.initials: P
authors.lastname: Vock
authors.forename: A J
authors.initials: AJ
authors.lastname: Olah
authors.forename: C
authors.initials: C
authors.lastname: Dahinden
authors.forename: N J
authors.initials: NJ
authors.lastname: Gerber
mesh_terms: D000328:Adult; D004002:Clodronic Acid / Q000627:therapeutic use; D005500:Follow-Up Studies; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008415:Mastocytosis / Q000209:etiology*; D010024:Osteoporosis / Q000209:etiology* / Q000628:therapy*; D010166:Palliative Care; D011994:Recombinant Proteins; D013122:Spinal Diseases / Q000209:etiology* / Q000628:therapy*; D014582:Urticaria Pigmentosa / Q000209:etiology
chemical_list: D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D004002:Clodronic Acid