Coming Up with Our Own Recipes | Lupus

Every healthcare provider knows that lupus is the great imposter. One day, it can affect one organ; the next, a whole different system can be targeted.

One lupus patient I have been treating for more than a year has traversed a difficult path since the day I saw her, swallowed up by the unpredictability of her disease.

NS is a 31-year-old woman who drove more than an hour to my office for her first visit in October 2016. She came to me with severe joint and chest pain, as well as a full body rash, which tested positive on biopsy for systemic lupus erythematosus (SLE).

During our initial conversations, NS told me that she had been sick since the birth of her daughter in 2014, but since she had no health insurance for the last 2 years, she could not afford a visit to a healthcare provider or any medication to treat her condition until she got on Medicaid.

An initial lab workup showed that NS was producing an overabundance of antibodies. She had high anti-nuclear antibodies (ANA) titers (1:1280) and tested positive for both anti-SSA and anti-SSB antibodies. NS also tested positive for rheumatoid factor (RF) with a high titer. Her anti-double stranded DNA test was negative, as was her anti-cyclic citrullinated peptide.

After her first visit, I knew we had to do something quickly. NS had recently been hospitalized for bronchitis, and I was concerned about organ damage if her SLE continued to progress.

NS was started on hydroxychloroquine 200 mg twice daily, along with 10 mg of daily prednisone. After four doses of this combination, she returned to my office complaining of uncontrolled, irregular tremors. NS was convinced they were being caused by her new medications, yet even when we stopped the hydroxychloroquine and prednisone, the tremors continued. While NS waited for an appointment with a neurologist to get more clarity on the source of her tremors, we started her on daily azathioprine 50 mg (increased to 100 mg after the first week).

After a full workup, the neurologist concluded that NS’s tremors were of unknown origin. The irregularity of the tremors was deemed “suspicious.” Along with azathioprine; she also reported to me that she was taking medical marijuana to help suppress her symptoms and sleep at night.

Several weeks later, NS’s rash had not improved and her lab results remained concerning. Her azathioprine was increased to 150 mg daily and prednisone 10 mg daily was restarted. Unfortunately, that regimen also proved ineffective as NS’s symptoms persisted and her cognition continued to decline. Even more worrisome, NS began reporting chest pain and shortness of breath. An echocardiogram and chest X-ray failed to reveal any organ damage.

We were starting to run out of options.

Our next step was a trial of IV belimumab. NS tolerated the treatments well and finally began showing some improvement over the next 4 months. Her cognition and depression both improved significantly; it was like she was a new person. Although her joint pain persisted, she was able to stop using medicinal marijuana and sleep more normally. Unfortunately, NS continued to suffer sequelae of SLE, including hair loss, oral and nasal sores, night sweats, and oral dryness. Her labs showed little improvement, her neutropenia persisted, and urinary casts began appearing. Because of the positive momentum we were able to gain with belimumab, we decided to keep trying, adding leflunomide to her regimen. Unfortunately, three months later, NS continued to have debilitating joint pain and stiffness, and she reported that she was struggling to care for her young daughter.

“We all probably know a patient who runs through the gamut of ups and downs on various treatment regimens”

While not approved by the U.S. Food and Drug Administration for the treatment of SLE, I knew that there were some positive results of abatacept in patients with severe lupus nephritis.1,2 Not being able to bear seeing NS suffering through another belimumab infusion by rocking herself back and forth to ward off her joint pain, I decided to venture into the unknown territory (at least for me) of abatacept in SLE. Using her positive RF as justification, I was able to get NS’s insurance company to approve the drug; a few days later, NS received her first infusion.

NS tolerated the abatacept infusion just fine, but reported to me a few days later that her rash had worsened. We bumped up her prednisone from 5 to 10 mg daily prior to her second infusion. NS’s reaction to the second abatacept infusion was worse than the first—her whole body broke out in an angry, red, pruritic rash. A dermatology consultation resulted in a biopsy that showed a drug-induced rash due to the abatacept.

So now what? I consulted with our in-house team and NS, and we jointly decided that, since there were at least some cognitive and psychological improvements with belimumab, we would give that another try. If only to attempt to mix things up, we also introduced subcutaneous weekly methotrexate (MTX) 17.5 mg to NS’s regimen.

After only a month, we started seeing dramatic improvements in NS’s lab results. Her ANA titers—previously sky-high at 1:1280—dipped to 1:80. Her neutropenia resolved. Her compliments improved. The urinary cases disappeared. NS’s rash slowly began to disappear and she was left with only four spots on her skin. Even her joint pain, which had previously been resistant to every other regimen we tried, began to slightly improve.

It’s hard to imagine that these improvements were due to the MTX after such a short period of use, so we were left with more questions than answers. Did the use of the T-cell inhibitor (abatacept) modify NS’s immune system for the better? Was this a rare case where use of MTX actually did result in a more rapid improvement than expected? It’s one of those cases where the medical literature isn’t helpful, so we can only guess.

SLE patients are complicated. We all probably know a patient like NS who runs through the gamut of ups and downs on various treatment regimens. It would be great if there was a reliable recipe to follow that showed us which medications we should try first and which have the best chance to work in various SLE patients. But since there is so much that remains mysterious about this disease, we are often left to experiment, trying a little of this and a little of that if only to see incremental gains. Yet we need to keep trying, no matter how frustrating it gets, for that glimmer of hope before the breakthrough finally comes.


Iris Zink, MSN, NP, RN-BC is a nurse practitioner at Lansing Rheumatology in Lansing, Michigan, and Immediate Past President of the Rheumatology Nurses Society.



1. Furie R, Nicholls K, Cheng TT, et al. Efficacy and safety of abatacept in lupus nephritis: a twelve-month, randomized, double-blind study. Arthritis Rheumatol. 2014;66(2):379-89.
2. ACCESS Trial Group. Treatment of lupus nephritis with abatacept: the Abatacept and Cyclophosphamide Combination Efficacy and Safety Study. Arthritis Rheumatol. 2014;66(11):3096-104.