Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: To study inflammatory and structural ultrasound lesions in patients with hereditary hemochromatosis (HH) with and without arthropathy
Methods: Cross-sectional study of 50 patients with HH [mean age 57.5 (±SD 11.6) years years, 26.0% female, median disease duration 8.8 (range 0.8-27.6) years] recruited at medical centers in Graz, Oberndorf and Vienna. HH arthropathy (HH-A) was defined as the presence of hand pain (VAS>10mm and/or ≥1 tender joint) plus ≥1 radiographic finding compatible with HH-A. Thirty-eight patients with hand osteoarthritis (HOA, according to ACR criteria) were studied for comparison [mean age, 60.1 (±SD 9.4), 89.5% female). Clinical examination was performed at 68 joints, and we retrieved data on hand function, pain and overall health status (all using a VAS), morning stiffness, ferritin levels and phlebotomy. Ultrasound was conducted at 40 joints (hand joints, hips, knees, ankles) by one rheumatologist blinded to clinical data using an ESAOTE Twice ultrasound device. Synovial hypertrophy and/or joint effusion (SH/E), Power Doppler (PD), osteophytes and erosions were subjectively graded from 0 to 3 in accordance with prior publications.
Results: Twenty-six (52.0%) HH patients were classified as HH-A. Mean age [57.1 (±SD 13.0) vs. 57.8 (±SD 9.7) years], median disease duration [7.5 (3.2-22.8) vs. 10.3 (0.8-27.6) years), median ferritin levels [83.6 (23-1060) vs. 66.1 ng/ml (14-853) as well as median duration [6.0 (0-23) vs. 6.0 (0-26) years] and number of phlebotomies/year [3 (0-5) vs. (2.5 (0-12)] were comparable between HH patients Without Arthropathy (HH-WA) and HH-A patients.<span”>Patients with HH-A and HOA had a similar number of tender [4 (0-29) vs. 3 (0-40)] and swollen joints [0 (0-7) vs. 0 (0-6)], and similar scores for hand function [39.5 (0-100) vs. 47.0 (0-92) mm] and hand pain [31.0 (0-72) vs. 23.0 (0-86) mm]. These findings were absent/low in HH-WA patients by definition.Using ultrasound, we observed ≥1 erosion in 10 (41.7%) HH-WA patients, 12 (46.2%) HH-A, and 21 (55.3) HOA patients (p>0.2). Similarly, ≥1 osteophyte was observed in 23 (95.8%), 26 (100%) and 38 (100%) patients, respectively (p>0.2); median osteophyte score, however, was higher in HH-A than in HH-WA patients [19 (0-53) vs. 30 (3-69), p=0.019] and comparable between HH-A and HOA [36 (8-68)]. SH/E were observed in a high portion of HH-WA, HH-A and HOA patients [20 (83.3%), 25 (96.2%) and 38 (100%), respectively] whereas PD-findings were more common in the HH-A [n=21 (80.8%)] and the HOA [n=31 (81.6%)] than in the HH-WA group [n=12 (50.0%), p<0.05]. Also, SH/E scores were comparable between the three groups [HH-WA: 6.5 (0-25), HH-A 9 (0-32) and HOA 11.5 (1-30)] whereas PD-scores were higher in HH-A [2.5 (0-17] and HOA [2 (0-17] than in HH-WA cases [0.5 (0-9), p<0.05). In HH-A patients, there was a weak correlation between PD-score and hand function (0.23, p=0.031), whereas the other clinical parameters were unrelated to ultrasound results.
Conclusion: A high prevalence of ultrasound verified inflammatory and structural lesions were found in patients with hereditary hemochromatosis. Higher PD scores were observed in patients with arthropathy, and these were related to limited hand function.
To cite this abstract in AMA style:Dejaco C, Stadlmayr A, Trimmel V, Duftner C, Husic R, Krones E, Zhandieh S, Husar-Memmer E, Zollner G, Hermann J, Gretler J, Lackner A, Ficjan A, Datz C, Axmann R, Zwerina J. Ultrasound Verified Inflammation and Structural Abnormalities in Patients with Hemochromatosis with and without Associated Arthropathy [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/ultrasound-verified-inflammation-and-structural-abnormalities-in-patients-with-hemochromatosis-with-and-without-associated-arthropathy/. Accessed November 20, 2019.
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