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Refractory Systemic Lupus Erythematosus: Identification and Pharmacological Management

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Abstract

Systemic lupus erythematosus (SLE) is characterized by an aberrant immune response, leading to an extremely heterogeneous clinical presentation, potentially affecting different systems and organs. Despite the fact that SLE mortality has greatly decreased since the introduction of steroids, some forms of refractory/severe SLE still have the potential to result in permanent organ damage as well as increased mortality and morbidity. Furthermore, SLE patients with multiple comorbidities may face a clinical conundrum and have a bad prognosis. An improved prognosis for severe refractory SLE depends on prompt and appropriate treatment. Due to the scarcity of solid data from a well-characterized group of patients with refractory/severe SLE coming from randomized controlled studies, this review aims to shed light on this with real-world evidence from clinical research performed at our Unit, the University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID) (Turin, Italy). In order to determine the key clinical and prognostic features, and therapeutic approaches for severe and/or refractory SLE, our experience will be described together with existing literature, primarily focused on dermatological, neuropsychiatric, and renal symptoms.

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Fig. 1
Fig. 2

Modified from Roccatello et al. [63]. CR complete response, CYC cyclophosphamide, IBCTD intensified B cell depletion protocol, MTP methylprednisolone, NR no response, PND prednisone, PR partial response

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Author notes

  1. Dario Roccatello and Savino Sciascia contributed equally.

  2. Daniela Rossi and Roberta Fenoglio are equal senior authors.

Authors and Affiliations

  1. University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital, Piazza del Donatore di Sangue 3, 10154, Turin, Italy

    Dario Roccatello, Savino Sciascia, Daniela Rossi & Roberta Fenoglio

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Correspondence to Dario Roccatello.

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Roccatello, D., Sciascia, S., Rossi, D. et al. Refractory Systemic Lupus Erythematosus: Identification and Pharmacological Management. Drugs 83, 117–134 (2023). https://doi.org/10.1007/s40265-022-01824-x

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