Why are there so many different kinds of Lupus? | Handling the Hard Questions Series


A chapter from HANDLING THE HARD QUESTIONS:
What Our Patients Are Asking Us About Rheumatic Disease

Read and download this booklet on our Workforce Resource homepage

Each day, rheumatology nurses, nurse practitioners, and physician assistants field dozens of questions from their patients with rheumatic diseases, and they need to be able to properly and effectively communicate appropriate responses. This pocket guide includes a brief summary of evidence surrounding some of the most common—and challenging—questions that patients with rheumatoid arthritis, psoriatic arthritis, gout, and systemic lupus erythematosus are asking about. We hope you find this guide useful for your professional development and that it assists you with your day-to-day patient management.

Why are there so many different kinds of Lupus?

As mentioned in the previous question, lupus is a heterogenous disease with many clinical presentations, ranging from skin lesions in the absence of any other symptoms to life-threatening, multisystem manifestations. In fact, some researchers believe lupus should be viewed as a syndrome, rather than a single disease.1

Beyond SLE, other common lupus types include the following:

  • Cutaneous lupus erythematosus, and especially discoid lupus erythematosus, which manifests as a skin rash that may wax and wane with treatment
  • Drug-induced lupus, a lupus-like condition caused by certain prescription drugs such as isoniazid or hydralazine. Symptoms usually disappear after the responsible drug is discontinued.
  • Neonatal lupus, a rare condition affecting some infants of women with lupus that puts the newborn at risk for congenital heart block. Neonatal lupus usually resolves within a few months, often with no lasting effects.2,3

Like SLE, cutaneous lupus erythematosus is believed to be caused by a combination of genetics and environment, with exposure to ultraviolet light thought to play a key role in both triggering of the disease and causing flares.2 Patients with cutaneous lupus erythematosus may have either an acute, subacute, or chronic form of the condition. Acute cutaneous lupus erythematosus usually is accompanied by other signs and symptoms of SLE. In contrast, both subacute and chronic cutaneous lupus erythematosus may occur in the absence of SLE.

Furthermore, there are several subtypes of chronic cutaneous lupus erythematosus, including discoid lupus erythematosus, lupus erythematosus profundus, lupus erythematosus tumidus, and chilblain cutaneous lupus.4 Both neonatal and drug-induced lupus are considered systemic diseases; as such, they may affect multiple organ systems. Neonatal lupus is believed to be the result of maternal autoantibodies crossing the placenta, whereas drug-induced lupus is the result of an autoimmune response following an exposure to a medication.2

References

1. Bertsias G, Cervera R, Boumpas D. EULAR Textbook on Rheumatic Diseases: Chapter 20. Systemic lupus erythematosus: Pathogenesis and clinical features. Available at www.eular.org/myUploadData/files/ sample%20chapter20_mod%2017.pdf. Accessed March 1, 2019.
2. Maidhof W, Hilas O. Lupus: an overview of the disease and management options. P&T. 2012;37(4):240-249.
3. Stannard JN, Kahlenberg JM. Cutaneous lupus erythematosus: updates on pathogenesis and associations with systemic lupus. Curr Opin Rheumatol. 2016;28(5):453-459.
4. Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol. 2013;27(3):391-404.