The patient was successfully treated with rituximab and intravenous immunoglobulin.
Patients with flares of systemic lupus erythematosus (SLE), multiple organ involvement, and infections can pose a treatment challenge for clinicians.
A new report in Case Reports in Rheumatology shows that alternatives to standard-of-care therapy may be warranted in certain cases.
High-dose glucocorticoids with or without immunosuppressive therapy is considered the standard of care for neuropsychiatric SLE, lupus nephritis, and lupus myocarditis, but given the wide range of presentations and organ manifestations associated with SLE, some patients may require alternative approaches, wrote the study authors.
They recounted the case of a 35-year-old male patient who complained of fatigability, significant weight loss, fever, night sweats, oral ulcer, and body aches, among other symptoms. The patient’s blood pressure was mildly elevated, and he had a history of high blood pressure and myocarditis. Upon examination, he had marked proximal and distal muscle weakness and enlargement of lymph nodes.
A lymph node biopsy suggested follicular and paracortical hyperplasia, but investigators wanted to rule out lymphoma, so they sought a second opinion, which confirmed hyperplasia. SLE was diagnosed based on criteria including his oral ulcer, positive antinuclear antibody, anti–double-stranded DNA, anti-Smith antibody readings, and low C3 levels.
He was started on prednisolone and hydroxychloroquine and discharged from the hospital. Less than a week later, however, he returned to the hospital with severe musculoskeletal symptoms. The patient soon experienced spikes in fever and leukocyte increases suggestive of sepsis. He was given broad-spectrum antibiotics and a series of tests. A blood culture showed persistent Gram-negative Salmonella non-Typhi and methicillin-sensitive Staphylococcus aureus (MSSA) bacteremia. The patient was admitted to the intensive care unit (ICU), where he was put on vasopressors and a ventilator and soon underwent continuous renal replacement therapy. A kidney biopsy revealed diffuse global lupus nephritis with active lesion class IV, the authors said.
Infectious disease clinicians examined the patient and diagnosed infective endocarditis, and the patient was given intravenous meropenem (Merrem). The symptoms of septic shock improved over the next week, but the patient soon began exhibiting psychiatric symptoms as well as signs of myocarditis. The investigators opted against cyclophosphamide, since the patient was recovering from severe sepsis.
“However, weighing risks and benefits and after a multidisciplinary meeting with ICU, infectious disease, rheumatology, and nephrology teams, the decision was made to give him a combined IVIG (intravenous immunoglobulin) of 0.4 g/kg/day for 5 days and RTX (rituximab; Rituxan) 500 mg once with pulse steroid therapy 1g/day for 5 days,” they wrote.
After 5 days, the patient’s psychiatric manifestations improved, as did biochemical markers.
Three weeks later, hewas given mycophenolic acid (Myfortic) along with prednisolone and hydroxychloroquine. He continued to improve over the following months, and his lupus nephritis was in complete remission. He was prescribed belimumab (Benlysta) to achieve full disease control.
The study researchers said infections can significantly raise a patient’s risk of mortality, and they can sometimes be difficult to identify given similarities with SLE disease flares. In this case, the patient had both infections and flare-ups, “which was a puzzling scenario due to the lack of formal guidelines for co-management of sepsis and involvement of 3 major organs.”
The investigators said their decision to use rituximab, IVIG, and pulse steroids led to improvement; this regimen was well tolerated and had a better adverse-effect profile than other regimens. They said it is important for treatment decisions to be based on the patient’s specific case and characteristics, but in this case, the combination seemed to work.
“Of course, it would be hard to attribute the response to one agent more than the others, but this strategy effectively reduced inflammation and prevented imminent death,” they concluded. “Furthermore, to our knowledge, this is the first case with this dilemma in treatment that was successfully treated.”
Cheikh MM, Bahakim AK, Aljabri MK, et al. Neuropsychiatric lupus and lupus nephritis successfully treated with combined IVIG and rituximab: an alternative to standard of care. Case Rep Rheumatol. Published online August 28, 2022. doi:10.1155/2022/5899188