Validation of a radiographic scoring system for haemochromatosis arthropathy.
BACKGROUND
Arthropathy is one of the earliest and most common manifestations of hereditary haemochromatosis with a significant impact on quality of life. Although its radiographic features are well known, there is no assessment tool for their evaluation.


OBJECTIVE
To develop and validate a novel scoring system for the evaluation of radiographic features of haemochromatosis arthropathy.


METHODS
A dichotomous scoring system assessing four radiographic features of haemochromatosis arthropathy and a 4-grade scale reflecting severity of radiographic change have been developed. Standard radiographs (hand, wrist, knee and ankle) of 170 subjects (116 male, 54 female) with genetically confirmed haemochromatosis and laboratory signs of iron overload were assessed by three readers and construct validity, feasibility and cross-sectional reliability (intrareader and inter-reader) were assessed.


RESULTS
Intrareader and inter-reader reliability as assessed by percentage pairwise agreement and Cohen's weighed κ were good to excellent for most features and locations evaluated. Radiographic scores correlated well with clinical parameters (bony swollen joint count, hand function and physician's global health assessment; Pearson's correlation, r²=0.18-0.62, p<0.0001). A complete set of radiographs took 3.4 ± 1.2 (mean ± SD) min to be assessed. An atlas of characteristic radiographic features was compiled.


CONCLUSIONS
A feasible and reliable radiological assessment tool for the evaluation of haemochromatosis arthropathy has been validated and an atlas of characteristic radiographic features provided.
Dallos
Tomás
T
Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany. dallos@dfnsp.sk
Sahinbegovic
Enijad
E
Aigner
Elmar
E
Axmann
Roland
R
Schöniger-Hekele
Maximilian
M
Karonitsch
Thomas
T
Stamm
Tanja
T
Farkas
Martin
M
Karger
Thomas
T
Cavallaro
Alexander
A
Stölzel
Ulrich
U
Keysser
Gernot
G
Datz
Christian
C
Schett
Georg
G
Manger
Bernhard
B
Zwerina
Jochen
J
D000328:Adult; D000368:Aged; D000843:Ankle Joint / Q000000981:diagnostic imaging; D001271:Atlases as Topic; D002805:Chondrocalcinosis / Q000000981:diagnostic imaging / Q000209:etiology; D004812:Epidemiologic Methods; D005260:Female; D006432:Hemochromatosis / Q000150:complications*; D006801:Humans; D007592:Joint Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D007719:Knee Joint / Q000000981:diagnostic imaging; D008297:Male; D008662:Metacarpophalangeal Joint / Q000000981:diagnostic imaging; D008875:Middle Aged; D011859:Radiography; D012720:Severity of Illness Index; D014955:Wrist Joint / Q000000981:diagnostic imaging