Vaso-Occlusive Events Precipitated By Intraarticular Steroid Injections in Patients with Sickle Cell Disease
Authors: Banks Landan , Gowhari Michel , Molokie Robert 
Published: November 2020

Introduction:
Sickle cell disease (SCD) is a group of inherited hemoglobin diseases (Hb), caused by a point mutation in the b-globin gene, that affects more than 100,00 people in the US and millions worldwide. No organ system is left unaffected by SCD, including the musculoskeletal system; complications which can greatly decrease quality of life and contribute to significant burden for these patients. Orthopedic complications include bone pain, dactylitis, growth retardation and atypical skeletal development, osteomyelitis and septic arthritis, bone demineralization, motor/mobility impairment, osteonecrosis and other causes of arthritis and rheumatologic disease. While surgical repair by arthroplasty is often a definitive solution to joint pain, conservative, non-operative treatments are first-line, including physical therapy and intraarticular corticosteroid injections. Corticosteroid injections can be used as a convenient outpatient solution to reduce inflammation and relieve pain without undergoing surgical measures. The use of steroids for non-orthopedic complications of SCD is not benign for many patients, and may be associated with the development of rebound vaso-occlusive pain events, requiring hospitalization, as well as hemorrhagic stroke. A review of the literature revealed two cases of patients with SCD who received intraarticular steroid injections for rheumatoid arthritis in whom the injections precipitated an acute vaso-occlusive episode that required admission. In view of these findings, we are interested in determining if any adults in our adult SCD clinic who had an intraarticular injection of a steroid developed worsening of pain.
Methods:
After approval by the UIC IRB, a retrospective chart review was conducted for subjects enrolled in the UIC Adult Sickle Cell Center treated with an intraarticular steroid injection. Data collected included: type of medication used, injection location, indication, pain complication and timeline of symptoms, admission, age, sex, genotype and BMI. Vaso-occlusive events described were unusually severe compared to the pre-corticosteroid clinical course and not reported with other triggers for SCD vaso-occlusive events.
Results:
Four patients were identified. Characteristics of these patients and injections are listed in table 1. Patients received one or more injections of knees, hips, wrists and fingers before recognizing the associated pain with the injection. Indications for injections included primary osteoarthritis, osteoarthritis secondary to avascular necrosis, tendonitis and tenosynovitis. All injections were a combination of local anesthetic and glucocorticoid. The majority of injections were 2 cc lidocaine 1%, 1 cc triamcinolone acetonide-40mg. Other local anesthetics used were xylocaine and bupivacaine.
Discussion:
With improvements in medical care, more people with SCD are surviving into adulthood and developing orthopedic complications of SCD, as well as aging, such as osteoarthritis. While the development of acute pain after treatment of the acute chest syndrome with steroids is known, there were only 2 reported cases of SCD patients who developed severe pain associated with intraarticular injections. We report here four additional cases of adults with SCD who received an injection and developed worsening of their pain, often requiring admission. While these numbers are small, we believe that this may be a much more common complication of steroid injections and providers should discuss this with their patients. The observations that are made in this study raise into question the idea that while temporary pain relief is the intended outcome, this is not the result in all patients- the fact of which should be a consideration made by all clinicians when offering steroid injections as opposed to non-steroid joint injections to patients with SCD. The pathophysiology behind this effect of steroid injections is unclear. In the event that a patient with SCD presents with pain symptoms, either worsening pain or pain uncharacteristic of their usual symptoms, a consideration for potential causes should be recent steroid injection for joint pain. These considerations may help guide clinical decision making.

Disclosures
No relevant conflicts of interest to declare.