Two years ago, a 30-year-old woman (RB) came to our office for an initial consult. She was in a wheelchair, covered from head to toe with a knit hat, turtleneck top, long knit pants, and Ugg boots. She was reticent to make eye contact with our staff and visibly wincing in pain.
Our initial discussion revealed a 10-year history of psoriasis that had previously only been treated with topical medications. However, RB’s disease had progressed to the point where she had been reduced to a wheelchair as a result of pain, swelling, and multiple joint stiffness, and she could no longer manage her condition on her own.
RB’s primary care physician placed her on 40 mg of prednisone and morphine for pain, which provided some relief over the course of the eight months before we met. RB told us she had had a gastrointestinal bleed about 4 months after starting prednisone that required one unit of packed red blood cells. She denied use of alcohol or street drugs, but did report a pack-a-day smoking habit for the last 6 years.
After disrobing, RB’s skin showed plaque and red scales on 90% of her total body surface area and severe alopecia on her scalp. Her Psoriatic Arthritis Severity Index (PASI) score was 6 (most severe on a scale of 1-6). The majority of RB’s pain was focused on her lower extremities. Her hip and shoulder motion was restricted. She had no proximal or distal interphalangeal joint swelling, and no dactylitis or enthesitis.
Our initial diagnosis was psoriatic arthritis (PsA).
We ordered labs and x-rays to complete our clinical picture. Lab results showed that RB was hepatitis B and C negative, but HLA-B27 positive. Her C-reactive protein was 7.5 mg/L and erythrocyte sedimentation rate was 77 mm/hr, both indicating very high levels of disease activity. She also had severe anemia. X-rays revealed the presence of bamboo spine and sacroiliac joint fusion. RB had neither erosive disease nor pencil cup deformities, but mostly axial damage.
To add to the complexity, RB’s Quantiferon test came back as “indeterminate.” In these cases, it is commonly recommended to perform an alternate test before starting any tuberculosis medications,1 so we performed a T-Spot TB test. It fortunately came back negative.
We initially started RB on daily methotrexate (MTX) 10 mg with folate, escalating to 15 mg after receiving her labs and serology report. We eventually increased the dose to 20 mg. After a few weeks with limited improvement in RB’s symptoms, we added sulfasalazine and infliximab to her regimen, while slowly tapering the prednisone. RB remained on acetaminophen/oxycodone and clonazepam for her pain.
Four months after the latest switch, RB’s PASI score had decreased to 3—a remarkable improvement in such a short amount of time. She had gone from a wheelchair to a cane and was now even beginning to walk. Her alopecia was improving as well, her hair growing back soft and curly.
RB started to smile and establish eye contact with our staff when she came to the clinic, making us all tingle with excitement at her progress. For St. Patrick’s Day, RB colored her hair bright green, telling us that “I feel beautiful and I want people to notice me.”
While for a short time we all felt good about our chances to reach remission, the heterogeneity of PsA —especially in patients who are HLA-B27 positive2—quickly put a damper on our optimism.
Six months after her initial response to infliximab, MTX, and sulfasalazine, RB’s alopecia was back, along with worsening psoriasis. Because we were unsure whether these symptoms were due to worsening of her disease or a reaction to the MTX, we first cut back the MTX to a dose of 10 mg daily, which improved the alopecia but not the disease activity. This reduction also triggered a second GI bleed and subsequent anemia. We then transitioned RB from oral, daily MTX to weekly injections to try to take advantage of the benefits of MTX while minimizing the side effects.
The treatment of RB has been a seesaw of ups and downs in the last year. We’ve tried a variety of approaches. Infliximab monotherapy worked for a while (bright red hair for the holidays) but then its effects dampened. We added sucralfate for a short time along with a proton pump inhibitor. This too briefly helped but saddled RB with anemia of chronic disease.
The heterogeneity of PsA can make management of the disease a significant challenge. Patients who appear to be doing well can and often do develop new symptoms despite our best efforts to keep their disease at bay. Associated conditions such as ulcerative colitis, Crohn’s disease, reactive arthritis, ankylosing spondylitis, and uveitis can all become involved.
RB’s latest symptoms include dactylitis and enthesitis of the elbow, adding to the challenge of peripheral damage to the already axial disease she initially presented with. We’re now waiting on her insurance to authorize a change to adalimumab.
Fortunately, RB’s spirits have remained high throughout our time together. She changes her hair color with the seasons and keeps telling us, “Just keep fixing me” whenever we talk about trying something new. It’s good to remind ourselves that when we first met, RB was confined to a wheelchair with horrible symptoms, so even her worst days now are better than those times.
Managing a patient with PsA such as RB is certainly frustrating because of the variability of the spondyloarthropathies, which makes it important to keep focused on the goal of remission (or, in some cases, low disease activity) through all the ups and downs. We’re fortunate to be practicing in an era where we have options to cycle through to try to find combinations that work for long periods of time. It just takes patience.
Jacqueline Fritz, RN, MSN, CNS, RN-BC, is Owner and Coordinator of Education at the Medical Advancement Center in Cypress, CA. Her primary responsibility is working as an advanced practice nurse for a large rheumatology practice where she is involved in patient visits, research programs, and infusion center coordination. In addition, she enjoys speaking, teaching, and learning about immunology.
1. Sterling TR, Villarino ME, Borisov AS, et al; TB Trials Consortium PREVENT TB Study Team. Three months of rifapentine and isoniazid for latent tuberculosis infection. N Engl J Med. 2011;365(23):2155-66. 2. Haroon M, Winchester R, Giles JT, Heffernan E, FitzGerald O. Certain class I HLA alleles and haplotypes implicated in susceptibility play a role in determining specific features of the psoriatic arthritis phenotype. Ann Rheum Dis. 2016;75(1):155-62