title: Mycophenolate mofetil treatment in two children with severe polyarteritis nodosa refractory to immunosuppressant drugs.
abstract: Polyarteritis nodosa (PAN) is a necrotizing vasculitis of small- and medium-sized arteries with multiorgan involvement, rarely reported in childhood. Despite aggressive treatment with the combination of corticosteroids and cytotoxic agents, the overall prognosis is poor in most patients. We report on two siblings, now 15- and 14-year-old, affected with childhood onset PAN, refractory to multiple therapies, who showed rapid clinical and laboratory improvement when mycophenolate mofetil was introduced. The relationship between the administration of this immunosuppressant agent and the reduced disease activity is confirmed by the sustained absence of disease flares over 4 years of treatment.
authors.affiliation: Department of BioMedicine, Rheumatology Section, Transition Clinic, University of Florence, Viale Pieraccini 18, 50139 Florence, Italy. falcini@unifi.it
authors.forename: Falcini
authors.initials: F
authors.lastname: Fernanda
authors.forename: Capannini
authors.initials: C
authors.lastname: Serena
authors.forename: Russo
authors.initials: R
authors.lastname: Giustina
authors.forename: Battagliese
authors.initials: B
authors.lastname: Antonella
authors.forename: D'Amico
authors.initials: D
authors.lastname: Alessandra
authors.forename: Maria
authors.initials: M
authors.lastname: Alessio
authors.forename: Alessio
authors.initials: A
authors.lastname: Maria
mesh_terms: D000293:Adolescent; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D010488:Polyarteritis Nodosa / Q000188:drug therapy*; D011241:Prednisone / Q000627:therapeutic use; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid; D011241:Prednisone