Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by antibodies to nuclear and cytoplasmic antigens, multisystem inflammation, protean clinical manifestations, and a relapsing and remitting course. More than 90% of cases of SLE occur in women, frequently starting at childbearing age.
Signs and symptoms of SLE
SLE is a chronic inflammatory disease that can affect almost any organ system, although it mainly involves the skin, joints, kidneys, blood cells, and nervous system. Its presentation and course are highly variable, ranging from indolent to fulminant.
The classic presentation of a triad of fever, joint pain, and rash in a woman of childbearing age should prompt investigation into the diagnosis of SLE.
In childhood-onset SLE, the following clinical manifestations are more commonly found than in adults  :
- Malar rash
- Ulcers/mucocutaneous involvement
- Kidney involvement, proteinuria, urinary cellular casts
- Hemolytic anemia
Diagnosis of SLE
The diagnosis of SLE is based on a combination of clinical findings and laboratory evidence. Familiarity with the diagnostic criteria helps clinicians to recognize SLE and to subclassify this complex disease based on the pattern of target-organ manifestations.
Management of SLE often depends on the individual patient’s disease severity and disease manifestations, although hydroxychloroquine has a central role for long-term treatment in all SLE patients.
Medications used to treat SLE manifestations include the following:
- Antimalarials (eg, hydroxychloroquine)
- Corticosteroids (eg, methylprednisolone, prednisone), short-term use recommended
- Nonbiologic DMARDS: Cyclophosphamide, methotrexate, azathioprine, mycophenolate, cyclosporine
- Nonsteroidal anti-inflammatory drugs (NSAIDS; eg, ibuprofen, naproxen, diclofenac)
- Biologic DMARDs (disease-modifying antirheumatic drugs): Belimumab, rituximab, anifrolumab, and/or IV immune globulin.
Cancer Medicine could be an option for refractory Lupus
In a first-of-its-kind clinical trial, researchers in Germany used a cancer-killing cell therapy to successfully treat lupus in a small number of patients.
All of the patients were treated with genetically engineered T cells known as chimeric antigen receptor (CAR) T-cell therapy, a treatment regularly used to kill cancer cells. Researchers harvested the patients’ immune cells and engineered them to destroy dysfunctional cells when infused back into the body.
All five patients were able to stop their standard treatments for as long as 17 months following the therapy, the study found. The patients also stopped experiencing severe symptoms such as lung inflammation, fibrosis of the heart valves, arthritis, and fatigue. The patients have not relapsed.
Preclinical studies involving mice previously showed that CAR T-cell therapy could help to reset the immune system. However, this latest study also found that patients did not need to continue any of their previous therapies, even after they regained their B cells about 4 months after the therapy.
While the single-treatment therapy is promising, transfused T cells do carry risks. Some of the patients in the study experienced fever and muscle pain following the procedure, the authors noted, more serious risks for this kind of therapy may include organ injury.
Experts also said the procedure is also costly and requires access to labs that can engineer a patient’s own T cells after they’ve been donated.
The study authors say they intend to create a larger trial to further explore which type of patient may benefit the most from this treatment, and for how long.
Andreas Mackensen, Fabian Müller, Dimitrios Mougiakakos, Sebastian Böltz, Artur Wilhelm, Michael Aigner, Simon Völkl, David Simon, Arnd Kleyer, Luis Munoz, Sascha Kretschmann, Soraya Kharboutli, Regina Gary, Hannah Reimann, Wolf Rösler, Stefan Uderhardt, Holger Bang, Martin Herrmann, Arif Bülent Ekici, Christian Buettner, Katharina Maria Habenicht, Thomas H. Winkler, Gerhard Krönke & Georg Schett, (September 15, 2022). Anti-CD19 CAR T cell therapy for refractory systemic lupus erythematosus. Nature Medicine: https://www.nature.com/articles/s41591-022-02017-5