For non-renal manifestations of disease, Dr. Ardoin explained that this is a bit of the Wild West in that more variability in practice patterns exists even among experts in the field, although this should improve with the publication of guidelines for the management of SLE presented at this year’s Convergence. When a patient has possible neuropsychiatric lupus (NPSLE), Dr. Ardoin noted that it is important to first ask a few questions: Could this be something else, like infection, malignancy or a toxic exposure? Why is the patient flaring now, and is it related to medication adherence? Is the patient pregnant or planning to become pregnant? If NPSLE does appear to be the correct diagnosis, then the clinician must consider if the driving issue is ischemia, in which case anticoagulation is important, or inflammation, which would require consideration of glucocorticoids, cyclophosphamide and anti-CD20 directed therapies. Symptomatic treatments, such as antipsychotics and antidepressants, may also have a role to play, and Dr. Ardoin recommended shared decision making with patients around treatment strategies whenever this is possible. For cutaneous disease, Dr. Ardoin stressed the importance of checking for medication adherence and the use of topical therapies and photoprotection, and stressed highly encouraging patients who smoke to quit as this may have dramatic effects on improving their skin disease. Options for medical management include dapsone, quinacrine, anifrolumab, anti-CD20 therapy and lenalidomide. For articular disease, Dr. Ardoin has had more success with methotrexate and leflunomide than with mycophenolate mofetil and azathioprine, and she sometimes borrows from treatment strategies for juvenile idiopathic arthritis and rheumatoid arthritis (RA) in considering treatment with tocilizumab, abatacept or Janus kinase inhibitors, although the latter may have concerns in patients with risk factors for thrombotic or cardiovascular disease.
Dr. Ardoin noted considerations that are particular to pediatric vs. adult patients with SLE that clinicians must keep in mind. Pediatric patients often present with more severe disease and may have monogenic forms of lupus, such as that seen with C1q deficiency, and given their earlier age of onset they have more potential years to be exposed to the effects of high disease activity or to incur the effects of years of drug exposure. Adults, meanwhile, often have more comorbidities and, thus, a greater degree of polypharmacy. Both pediatric and adult patients may face challenges with medication adherence, social impacts on their families, struggles with mental health, absences from work or school and impacts on their reproductive health/family planning.
