Abstract
Systemic lupus erythematosus (SLE) and bullous pemphigoid (BP) are chronic autoimmune diseases in which B cells play an important pathogenic role in the different stages of the disease. B cell-targeted therapies have been suggested as a new rational approach for treating SLE. Rituximab (RTX), an anti-CD20 chimeric monoclonal antibody, failed to achieve primary endpoints in two clinical trials (EXPLORER and LUNAR) despite multiple observational and retrospective studies showing its beneficial effect on SLE. Moreover, RTX is recommended in cases of BP that is unresponsive to conventional treatments. Belimumab (BLM), a human immunoglobulin G1 λ monoclonal antibody that inhibits soluble B-lymphocyte stimulator (BlyS)/B-cell activating factor (BAFF), is the only biological treatment approved for standard therapy of refractory autoantibody-positive active SLE. Animal models and a few case reports have supported the efficacy of the combined use of RTX followed by BLM as maintenance therapy in severe lupus nephritis (LN), suggesting that their combined use may be more effective than their single use, without compromising safety. In this study, we describe the clinical case of a SLE patient with predominant renal involvement in overlap with BP, refractory to conventional therapy including RTX alone, achieving significant steroid sparing and clinical remission under sequential treatment of RTX-BLM. Moreover, we describe the first case of BP successfully treated with BLM. This case report may encourage further clinical research studies in B lymphocyte targeted combination therapy in patients affected by SLE with major organ involvement or with refractory disease, suggesting that RTX and BLM sequential therapy may be a valid option for the treatment of SLE manifestations, including conventional therapy and RTX-resistant LN.
Highlights
Systemic lupus erythematosus (SLE) and bullous pemphigoid (BP) are chronic autoimmune diseases in which B lymphocytes play a primary pathogenic role as they are implicated in the induction and progression of these diseases [1, 2]
The patient described in this clinical case had long-standing relapsing lupus nephritis (LN) and BP, with skin and kidney biopsies showing persistent tissue inflammation
BLM has been used in patients with LN, mainly to maintain remission [25, 30]; some clinical cases have been described reporting the use of BLM in the treatment of multiple therapy refractory patients with LN [31]
Summary
Systemic lupus erythematosus (SLE) and bullous pemphigoid (BP) are chronic autoimmune diseases in which B lymphocytes play a primary pathogenic role as they are implicated in the induction and progression of these diseases [1, 2]. We reported the clinical case of a SLE patient with predominant renal involvement in overlap with BP, refractory to conventional therapy including RTX alone, achieving significant steroid sparing and clinical remission under sequential treatment of RTX-BLM. Positivity (ACLA IgM 46 U/ml and antiB2GP1 IgM 38 U/ml), and complement level reduction (C3 78 mg/dl and C4 8 mg/dl)] with a Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) of 10; intravenous therapy with steroid (methylprednisolone pulses 250 mg for three consecutive days) was started followed by oral prednisone 1 mg/kg daily, subsequently tapered, and combined with mycophenolate mofetil (MMF) 2,000 mg daily and angiotensin-converting enzyme inhibitors, obtaining renal and cutaneous remission. RTX was discontinued and intravenous BLM (SLE therapeutic scheme, 10 mg/kg monthly) in association with MMF 2,000 mg daily and low-dose prednisone (10 mg daily) was started, leading to a progressive improvement of both renal and skin manifestations. The patient is continuing therapy with BLM and MMF and with low dose (2.5 mg/daily) of prednisone without further SLE and BP flares
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