AB0542 Blocking the Human B Lymphocyte Stimulator Molecule (BLYS) Using A Monoclonal Antibody (Belimumab) in Systemic Lupus Erythematosus: First Results in Real-Life Spanish Patients with Refractory Disease (Biogeas-Semi Registry)

Abstract

Objectives To analyze the efficacy and safety of treatment with belimumab, a monoclonal antibody that specifically binds the soluble form of the protein human B lymphocyte stimulator B (BLyS), in SLE patients refractory to standard treatment. Methods In 2006, the Study Group on Autoimmune Diseases (GEAS) of the Spanish Society of Internal Medicine created the BIOGEAS registry, a multicenter study recruiting patients with refractory systemic autoimmune diseases treated with biological agents. Results On December 31, 2013, a total of 10 patients treated with belimumab were included in the BIOGEAS Registry, 9 females and 1 male, with a mean age of 41.8 years (range 24-71 years). These patients were heavily treated, with a mean of 7 years of corticosteroid treatment, antimalarials and at least one immunosuppressive agent, mycophenolate in 9, azathioprine in 7, metothrexate in 4, cyclosphosphamide in 2, cyclosporine A in 2, tacrolimus in 2 and talidomide in 2. Belimumab (10mg/kg) was administered in combination with corticosteroids (in all patients), antimalarials (6 patients) and immunosuppressive agents (8 patients, 6 with mycophenolate). The baseline average activity score (SELENA-SLEDAI) was 12 (range 6-33); 8 patients had high titers of anti-dsDNA and/or hypocomplementemia. Therapeutic indication included refractory mucocutaneous involvement (4 patients), refractory vasculitis (3 patients), systemic disease (2 patients) and refractory lupus nephritis (1 patient). After a mean follow-up of 7 months (range 2-12), clinical response could be evaluated in 9 patients, 8 classified as responders (improvement greater than 80% in 5 patients), while the remaining patient was classified as stable. Two patients (20%) developed adverse events (raised transaminases and systemic cryptococcosis due to severe CD4 lymphopenia); there were no deaths or lupus flares. Conclusions These postmarketing data in Spanish SLE patients in whom belimumab was used following the recommendations approved by the FDA/EMA (refractory disease to standard treatment, clinical and immunological active disease, SELENA-SLEDAI>6) have showed promising results, with a good therapeutic response and a safety profile similar to other biological therapies used off-label in patients with SLE. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5447

Use of belimumab in real-world in Spain: a scoping review about characteristics of SLE patients

“…Three studies were conducted in hospital settings from Madrid [ 34 , 38 , 40 ], 3 in Barcelona [ 26 , 29 , 39 ], 2 conducted in Murcia Region [ 31 , 41 ], and one each other in Alcalá de Henares [ 35 ], Alicante [ 33 ], Ciudad Real [ 28 ], Granada [ 30 ], Pamplona [ 36 ], Torrelavega [ 37 ], Valencia [ 24 ], and Vizcaya [ 27 ]. The remaining 3 publications [ 22 , 32 , 42 ] were multicenter studies. The number of included patients varied widely within these case series from 5 [ 40 ] to 64 [ 22 ] SLE patients.…”

“…Only 3 retrospective analysis reported the baseline average activity score (SELENA-SLEDAI), with scores ranging from 5.9 [ 35 ] to 12 [ 42 ] (mean 9.1). In general terms, the mean duration of disease from onset of symptoms to diagnosis ranged between 10 months [ 31 ] and 27 years [ 33 ] (mean duration 9.0 years), with a shorter duration for case reports subgroups.…”

“…Hematological co-morbidities ( n = 29, 13.1%), renal involvement ( n = 16, 7.2%), lung or heart involvement ( n = 11, 5.0%), and vasculitis ( n = 4, 1.8%) were documented. In two retrospective analysis [ 36 , 39 ] and one case report [ 26 ], 7 patients were reported to have antiphospholipid syndrome and other 3 retrospective analyses [ 39 , 40 , 42 ] included 4 patients with LN. Only 3 patients were reported as associated Sjögren syndrome and 2 as autoimmune thyroiditis [ 39 ].…”

“…Only 5 retrospective studies [ 32 , 36 , 37 , 39 , 42 ] and 6 case reports [ 26 – 31 ] ( n = 93 patients) provided data about the number and type of previous treatments to belimumab in included patients. None of the prospective studies described any information about this outcome.…”

“…None of the prospective studies described any information about this outcome. No data about the duration of each treatment were reported, although one retrospective study [ 42 ] registered a mean of 7 years of previous treatment.…”