One of our office’s most interesting and complicated juvenile spondyloarthritis (SpA) cases involved AZ, who presented to us as a 13-year-old male with a 3-year history of bilateral hip pain and approximately 6 months after bilateral hip pinning for slipped capital femoral epiphysis (SCFE), a hip condition that occurs in growing teens and pre-teens. The bilateral nature of AZ’s SCFE was somewhat unusual, as it is a condition that is usually limited to one side.
AZ’s initial radiographic history was consistently normal, and he never complained of any other joint pain besides in his hips. Following the hip pinning, AZ had initially progressed well and he was able to walk approximately 100 feet with mild pain, but after 3 months, his mobility began to decrease and his pain increased. He came to us bound to a wheelchair.
Prior to his appointment in our office, an MRI with/without contrast was ordered that showed left sacroiliitis and degenerative disc disease at L5-S1. By this time, AZ had been homeschooled for most of 7th and all of 8th grade, and he said that he was feeling socially isolated. His parents strongly disagreed about the source of AZ’s issues—his father thought he was “faking” his symptoms to get attention while his mother was convinced that there was a true source of the pain that needed to be discovered.
Upon physical examination, AZ was found to have excruciating pain with any movement of his legs or back. He needed significant assistance just to get up onto the exam table and was consistently anxious throughout the exam. AZ had a swollen and mildly warm left knee, though he said that it did not bother him. He complained of consistent pain that did not necessarily worsen at any particular time of day. AZ slept on the first floor of his house as getting up the stairs to his bedroom was too difficult. In general, he appeared defeated and depressed, which was not surprising based upon his symptoms and quality of life.
AZ’s lab results showed highly elevated acute phase reactants (APRs) with a C-reactive protein of 180 mg/L, erythrocyte sedimentation rate of 65 mm/hr, white blood cell count of 15,000 cells/mL, hemoglobin of 10.5 g/dl, and platelet count of 459,000 cells/μl. X-rays of his pelvis and hips showed dysmorphic appearance of femoral heads with spurring and joint space narrowing. We obtained another bilateral hip MRI with/without contrast that was negative for bone marrow edema in the spine, pelvis, and proximal femurs, and showed no synovitis or joint effusion. However, the radiologist remarked that it was difficult to get a good view of the femoral heads due to artifact caused by the hip pins. AZ’s sacroiliac joints were unremarkable.
After our full workup, the diagnosis remained murky, so we began AZ on a conservative regimen of naproxen 500 mg (10 mg/kg) BID. The differentials included juvenile SpA, an infection, or an underlying inflammatory disorder of the gastrointestinal tract. After a consultation with orthopedics, AZ’s hip pins were removed, and we collected fluid cultures near the affected area. We again ordered an MRI with/without contrast to get a better look at the femoral heads and epiphysis.
The repeat MRI showed synovial enhancement, acetabular cartilage loss, labral tears, and joint space narrowing with findings that strongly correlated with juvenile idiopathic arthritis. Fluid cultures were positive for gram positive bacteria. We now felt confident beginning more aggressive treatment for a SpA, but we first knew we had to treat AZ’s possible infection before a possible progression to biologic therapy. AZ therefore received 30 days of antibiotics via a PICC line at his home. He also continued with physical therapy.
After seven months, we added sulfasalazine and methotrexate to AZ’s treatment regimen, but he remained unable to attend school and was only able to walk approximately 100 feet using a cane or walker. As with his prior treatment following the hip pinning surgery, AZ’s improvements had plateaued, and his pain began to increase.
We referred AZ back to orthopedics for re-evaluation, and they recommended a bilateral hip replacement. Our team felt that a trial of infliximab would be more appropriate before such a drastic surgery. AZ’s mother was torn between these recommendations. All she knew for certain was that her once active and popular son could not attend school or have friends over because of his current physical limitations. She requested a second opinion from another pediatric rheumatologist, who concluded that AZ did not have a SpA and agreed with the orthopedist that a bilateral hip replacement was warranted. It was explained to AZ’s mother, however, that this was a serious surgery and that, once performed, there was no going back. She was told that AZ would still likely be limited in some of his activities. Both the adult and pediatric orthopedist were reticent to perform such a procedure on a young, growing boy, even with the potential benefits.
During these months of multiple evaluations from multiple specialists, AZ’s APR’s fluctuated wildly, making it difficult to determine if his pain was being caused by inflammation or infection. Prior to scheduling the hip replacement, AZ’s orthopedist agreed to try bilateral steroid injections, and AZ was walking with less pain within three days. His left knee swelling also finally resolved.
For the next several weeks, AZ’s improvement—consistent with his prior history—waxed and waned, and we finally convinced his mother to allow us to try monthly infliximab in addition to the naproxen, sulfasalazine, and MTX. Within a month, AZ’s APRs normalized and, most importantly, they stayed normal for 6 months. AZ began walking up and down stairs and had a steadier gait with a more upright posture. Everything was not, however, perfect. Three months after starting infliximab, AZ started complaining of gastritis, which we learned was due to his complacency in taking his oral medications, a common issue in teenaged patients who are responsible for managing their own medication regimens.
As he neared the start of ninth grade, we encouraged AZ’s mom (his father had mostly disappeared as a medical decision maker in recent months) to send her son back to school. As many of his friends would be starting a new school—high school—we all felt it was an opportune time to re-engage AZ with his peers. It took many months of difficult conversations to convince them, and that first year was extremely bumpy, but AZ did indeed go back to school, where he stayed for 4 years. Adherence to a complex medication regimen remained an issue throughout AZ’s high school years, although it got better as he matured.
We’re now more than 4 years out from AZ’s initiation of infliximab, and he’s seeing less and less benefit from biologic therapy. His bilateral hip pain is again increasing, and the most recent MRI is negative for inflammation. Our team is now in agreement that a bilateral hip replacement is needed at this time. We did our best to delay AZ’s bilateral hip replacement as long as possible since his bones and muscles were still growing rapidly, and a bilateral hip replacement could ultimately cause a severe arthritis flare down the line.
There is currently a suspicion that AZ may have had a reaction to the metal used in the SCFE pinning; he will be making a trip to an allergist before his orthopedist decides which type of hip hardware to use during his upcoming surgery. AZ should have an excellent outcome after bilateral hip replacement now that he is almost 19 years old and won’t be growing much more. He is also in a much better place emotionally and feels ready to handle the next steps in his care.
A wise rheumatologist frequently reminds our multidisciplinary team that it is possible for our patients to have concomitant conditions that need to be managed carefully, along with the importance of systematically progressing through differential diagnoses. Cases like AZ are very tough. While we weren’t certain that, for example, the addition of infliximab would help, we saw that AZ was physically and emotionally deteriorating and felt this was our best option. It was either going to help him or it wasn’t, plain and simple. We never ordered any narcotics for AZ, and his mother never asked for them. We were all in agreement that the risk of potential opioid addiction was too great and would add an unnecessary layer of complexity to his care.
AUTHOR PROFILE:
Cathy Patty-Resk, MSN, RN, CPNP-PC is a certified pediatric nurse practitioner in the Division of Rheumatology at Children’s Hospital of Michigan in Detroit, MI, where she provides medical services to inpatient and outpatient pediatric rheumatology patients.
Reference
1. Peck DM, Voss LM, Voss TT. Slipped Capital Femoral Epiphysis: Diagnosis and Management. Am Fam Physician. 2017;95(12):779-784.