title: Assessing joint involvement in haemophilia by clinical rheumatologic scores. A pilot study on similarities with subjects with psoriatic arthritis.
abstract: Because of joint haemorrhages, severe haemophilia subjects often have limitations in their daily activities. Current orthopaedic scores (OJS) in haemophilia miss mild joint impairments and only pick up severe alterations. Twelve young severe haemophiliacs (20.25 ± 1.9 years of age), were evaluated for OJS as well as for indices employed in rheumatoid arthritis [28-joint Disease Activity Score (DAS-28), Ritchie index, Health Assessment Questionnaire (HAQ), visual analogue scale (VAS)], spondyloarthritis [Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), HAQ, VAS] and osteoarthritis [Knee injury and Osteoarthritis Outcome Score (KOOS), VAS]. Twenty-four matched apparently healthy subjects and 29 subjects with psoriatic arthritis (PsA) with oligoarticular involvement (one to three swollen joints) served as controls. In addition to the impairment of target joints (elbow, in five of five in those on-demand treatment; three, elbows; four, knee in those on secondary prophylaxis), HAQ (mean 0.71 ± 0.95) and VAS (3.12 ± 2.36) documented a quality of life and a perception of pain in haemophiliacs similar to that of PsA subjects (p = 0.061 and p = 0.063, respectively). Their Ritchie index did not differ from that of subjects with psoriatic arthritis (5.75 ± 8.1 vs 7.73 ± 9.22; p = 0.408), nor did the BASDAI score with respect to psoriatic arthritis patients (p = 0.105). Six of 12 haemophiliacs (50%) had KOOS values from 70 to 50 (significant function joint impairment); 3 of 12 (25%) showed DAS-28 values >3.2 (moderate disease activity), 2 of 12 (16.6%) severe disease activity (>5.1). All these indices significantly correlated with VAS and HAQ in haemophilia subjects. A rheumatologic assessment may help identify early polyarticular disease and subclinical abnormalities involving joints not usually studied (not target joint) in haemophiliacs. These pilot data provide the rationale for testing a systemic involvement in haemophiliacs by means of high-tech imaging and to start early-onset prophylaxis/treatment in this setting.
authors.affiliation: Department of Clinical and Experimental Medicine, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy.
authors.forename: Matteo Nicola Dario
authors.initials: MN
authors.lastname: Di Minno
authors.forename: Salvatore
authors.initials: S
authors.lastname: Iervolino
authors.forename: Rosario
authors.initials: R
authors.lastname: Peluso
authors.forename: Antonio
authors.initials: A
authors.lastname: del Puente
authors.forename: Anna
authors.initials: A
authors.lastname: Russolillo
authors.forename: Antonio
authors.initials: A
authors.lastname: Coppola
authors.forename: Carlo
authors.initials: C
authors.lastname: Ruosi
authors.forename: Anna
authors.initials: A
authors.lastname: Guida
authors.forename: Raffaele
authors.initials: R
authors.lastname: Scarpa
authors.forename: Giovanni
authors.initials: G
authors.lastname: Di Minno
mesh_terms: D015535:Arthritis, Psoriatic / Q000175:diagnosis* / Q000503:physiopathology; D006304:Health Status; D006467:Hemophilia A / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D006470:Hemorrhage / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007596:Joints / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D011788:Quality of Life; D012720:Severity of Illness Index; D055815:Young Adult