Long-term Outcomes of Avascular Necrosis in Sickle Cell Disease Using Joint-specific Patient-reported Outcome Measures: Results from a Multicentre Study

Overview

Journal Br J Haematol

Specialty Hematology

Date 2024 Sep 30

PMID 39344565

Authors

Maddalena Casale

Giuseppe Toro

Federica Porcelli

Alessandra Quota

Rosamaria Rosso

Vincenzo Spadola

Francesco Arcioni

Saveria Campisi

Domenico Roberti

Silverio Perrotta

Affiliations

Soon will be listed here.

Abstract

Avascular necrosis (AVN) is a prevalent and progressive complication in young patients with sickle cell disease (SCD), but no study evaluated the long-term subjective and objective outcome measures. Oxford hip score (OHS) and Oxford shoulder scores (OSS) are validated joint-specific patient-reported outcome measures (PROMs). In this prospective multicentre study, 47 SCD patients with pre-existing diagnosis of AVN occurred at a median age of 35.9 (24.2-47.6) filled out the OHS and OSS at median follow-up of 9.4 years (4.5-12.9). No patient died after diagnosis of AVN. Hip AVN was present in 34 (72%) patients, with bilateral involvement in 25 (74%); 26 (59%) underwent total hip arthroplasty (THA) at a median age of 34.6 (22.6-49.5); and 4 (15%) required re-surgery. OHS revealed moderate to severe impairment both in patients underwent THA and no hip surgery. Shoulder AVN was present in 13 (6%) patients and OSS revealed mild to moderate impairment. A high rate of compromised joint function and pain was observed 10 years after diagnosis of AVN regardless of the type of treatment, outlying the need to improve the management of this sickle-related complication. OHS and OSS are validated joint-specific PROMs easy to use in all SCD centres.

Citing Articles

Long-term outcomes of avascular necrosis in sickle cell disease using joint-specific patient-reported outcome measures: Results from a multicentre study.

Casale M, Toro G, Porcelli F, Quota A, Rosso R, Spadola V Br J Haematol. 2024; 206(1):310-319.

PMID: 39344565 PMC: 11739771. DOI: 10.1111/bjh.19802.

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