Pulling Back the Curtain on “The Great Imposter” | Systemic Lupus Erythematosus (SLE)

“SLE IS LIKE A GREY WOLF LURKING AT THE EDGE OF THE WOODS, WAITING PATIENTLY TO ATTACK AN INDIVIDUAL’S BLOODSTREAM WHEN IT IDENTIFIES AN OPPORTUNITY…”

Systemic lupus erythematous (SLE) has been called “The Great Imposter.” A sneaky disease that frequently changes its tactics to target its prey’s deepest vulnerabilities, SLE is like a grey wolf lurking at the edge of the woods, waiting patiently to attack an individual’s bloodstream when it identifies an opportunity.

Of all the autoimmune diseases, SLE is the most difficult to treat and treat successfully. Its effects can be devastating to a patient’s quality of life and potentially life threatening down the road. Developing effective strategies takes patience and persistence.

My personal education about SLE was accelerated early in my career by JD, a 50-year-old Caucasian female who presented to me with SLE that manifested in multiple ways. Despite having lived with SLE for more than 20 years, JD had a wonderful outlook on life and a sharp sense of humor.
Since being diagnosed with SLE in her mid-20s, JD’s disease morphed every few years, attacking new systems and causing new problems with each adjustment.

JD’s medical history was significant and—in addition to the traditional oral sores, nasal sores, hair loss, skin sores, and recurrent fevers—included the following:

• A period of intractable migraines that required several hospitalizations
• Anemia and neutropenia that required multiple blood transfusions
• Vascular inflammation near the optic nerve that caused short-term blindness lasting 7 days
• Cardiac inflammation and pericardial effusions
• Pleurisy and pleural effusions
• Avascular necrosis of the hip due to high doses of steroids

And these are just some of the long list of issues JD had battled over the years. Her SLE just never let up. She started on hydroxychloroquine, switched to mycophenolate mofetil, and then eventually moved on to IV cyclophosphamide after a bout of renal nephritis.

One of the reasons caring for JD was such a valuable personal learning experience was because it occurred early in my professional career, just as I was learning about the insidious nature of autoimmune diseases. At the time I began caring for JD in the mid‑1990s, we did not know a lot about how or why SLE developed and progressed. Treating the disease mostly required guesswork. In JD’s case, we would try a new approach that seemed to work for a short time, only to watch in disbelief as another organ system came under attack.

The end of my journey with JD came when “the great imposter” attacked her liver. With no apparent cause, JD’s liver enzymes shot up to 400 virtually overnight. She was sent to a hepatologist for evaluation, who promptly counseled her about her “obvious alcoholism” and the need to quit drinking immediately. The only problem was that JD did not drink. Her doctor, however, did not believe her and insisted that she must be a closet drinker and a liar, as there was no other possible explanation for her cirrhosis of the liver.

JD returned to our office in tears. We were horrified by the accusations made by JD’s doctor, and I reassured her that I believed her and felt that the only logical answer was that her SLE was affecting her liver. A few years later, the literature confirmed our beliefs, showing that approximately 25-50% of patients with SLE may have liver abnormalities, including inflammation and scarring.1

Shortly after fibrosis of the liver was diagnosed, JD was hospitalized with pneumonia and eventually developed sepsis. Due to years of steroid exposure, JD was at high risk of infection, so this did not come as a complete surprise. Once sepsis set in, JD’s kidneys failed, and her body simply stopped fighting. She looked at her husband and daughter and told them that this was the end. At age 50, JD died due to complications of SLE.

What JD taught me is that we can’t fit any of our patients, and especially those with SLE, into a typical “bucket” of symptoms. While SLE has been shown to be most aggressive in non-Caucasians,2 JD was a Caucasian patient who still developed multiple serious and life-threatening complications. She was a fighter with a “can-do” attitude who tried to—and usually did—overcome every obstacle thrown her way. Her case is not one that any nurse would have read about in a textbook, but has been a valuable lesson in showing me the range of possibilities in patients with SLE.

AUTHOR PROFILE:
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.

 

 

References
1. Bessone F, Poles N, Roma MG. Challenge of liver disease in systemic lupus erythematosus: Clues for diagnosis and hints for pathogenesis. World J Hepatol. 2014;6(6):394-409.
2. González LA, Toloza SM, McGwin G Jr, Alarcón GS. Ethnicity in systemic lupus erythematosus (SLE): its influence on susceptibility and outcomes. Lupus. 2013;22(12):1214-24.