Background: Lupus nephritis (LN) is one of the major organ involvement of Systemic Lupus Erythematosus (SLE), causing significant increase of morbidity, mortality and healthcare impact. ¹ According to international guidelines, immunosuppressive therapy is indicated for LN class III and IV, while class V usually do not require this treatment, less than there is a proteinuria in the nephrotic range, and so for class II, also with a risk of evolution in a more aggressive form. ² Immunosuppressive drugs commonly used are mycophenolate mofetil/mycophenolate acid (MMF/MPA) and cyclophosphamide for the initial treatment and MMF/MPA or azathioprine for the maintenance of remission. LN treatment is often difficult because of the drug adverse events and the comorbidities that make therapeutic choices very challenging.

Objectives: We describe the case of a young woman with SLE and other major systemic comorbidities who developed severe LN. This case emphasizes the importance of considering patients in their complexity and the delicate role of the physician in tailoring the treatment strategy on the individual patient.

Methods: A 36-year-old woman presented to our Department for lymphadenopathy, headache, epistaxis, fever and weight loss. In her past medical history, she had a cerebral abscess caused by Streptococcus Constellatus with residual left hemiplegia and cervical HPV-related high-grade dysplasia, treated by ring biopsy. Upon admission, blood tests revealed severe neutropenia and lymphocytopenia, thrombocytopenia and low complement; blood cultures were negative. A total body CT scan showed multiple lymphadenopathies. Lymph node and bone marrow biopsies were performed and histological examination was negative for hematological disease. The autoimmune panel was positive for antinuclear, anti-DNAn, anti-Sm, anti-U1RNP, anti-cardiolipin and anti-beta2glycoprotein antibodies. According to clinical and laboratory results, a diagnosis of SLE was made.

Results: High-dose corticosteroid therapy was started with good clinical response and improvement of blood tests. Because of the gynecological history, immunosuppressive therapy was not started, and a wait-and-see strategy with steroid tapering and careful follow-up was started. A few months later, the patient developed an increase of serum creatinine with proteinuria at nephrotic range. Thus, a kidney biopsy was performed with evidence of class III LN. Following this result, we started high-dose corticosteroid therapy, with only partial response. After executing colposcopy with biopsy, that was negative for HPV-related lesions, we decided to start MMF, continuing with a careful follow-up. After six months, proteinuria reduced but maintained on levels around 1 gr per day. Bearing in mind the growing number of studies reporting the efficacy of belimumab in LN 3,4, we added this drug to achieve a better disease control, continuing to closely monitor the patients and potential adverse events.

Conclusion: In patients with LN the choice of the immunosuppressive therapy can be very challenging. A tailored approach to the individual patient may be the best option to improve the management of this complex disease.

REFERENCES:

[1]Gasparotto M. et al. Lupus nephritis: clinical presentations and outcomes in the 21st century. Rheumatology (Oxford). 2020 Dec 5;59(Suppl5):v39-v51

[2]Fanouriakis A. et al. 2019 Update of the Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of lupus nephritis. Ann Rheum Dis. 2020 Jun;79(6):713-723.

[3]Furie R. et al. Two-Year, Randomized, Controlled Trial of Belimumab in Lupus Nephritis. N Engl J Med. 2020 Sep 17;383(12):1117-1128.

[4]Ward M. et al. Belimumab as Add-on Therapy in Lupus Nephritis. N Engl J Med. 2020 Sep 17;383(12):1184-1185.

Disclosure of Interests: None declared