RESUMO
BACKGROUND: Bullous pemphigoid (BP) affects older patients with numerous comorbidities. The impact of BP on patient autonomy remains poorly understood. OBJECTIVES: To assess the frequency and factors associated with functional decline (FD) in BP. METHODS: In this retrospective monocentric study, we selected patients aged ≥75 years with BP diagnosed between 1 January 2015 and 31 December 2021 and followed for more than 3 months. We assessed activity limitation at diagnosis and included patients with no or mild activity limitation. We described FD and its associated factors at 6 weeks and 3 months. FD was qualified as moderate or severe. Age, comorbidities (CIRS-G score), BP characteristics at diagnosis (including palms and soles involvement) and hospitalization were collected. Factors associated with FD were investigated through univariate and multivariate logistic regression models. Worsening of previous comorbidities or new comorbidities at Week 6 were collected. RESULTS: One hundred and thirty-one patients were included (mean age 85 years), 75 (57.3%) had no activity limitation, and 56 (42.7%) a mild activity limitation at baseline. At Week 6, FD was observed in 44 (33.6%) patients, including 23 out of 75 (30.7%) with no activity limitation and 21 out of 56 (37.5%) with mild activity limitation at baseline. FD was moderate in 20 cases (45.5%) and severe in 24 (54.5%) and persisted at Month 3 in 37 (84%) patients. By multivariate analysis, factors associated with FD were a CIRS-G score >7, palms and soles involvement and anti-BP180 antibodies level. Fifty (38.2%) patients experienced a worsening of their pre-existing comorbidities or a new one. CONCLUSION: FD is often observed in patients with BP, especially in those with severe symptoms, palms and soles involvement and comorbidities. A comprehensive, multidisciplinary approach involving geriatric assessments should be adopted to manage these patients.
RESUMO
BACKGROUND: Following the RITUX 3 therapeutic trial, the French national diagnosis and care protocol (NDCP) for the treatment of pemphigus was updated in 2018. The updated protocol recommends initial treatment with rituximab (RTX) followed by maintenance therapy at 12 and 18â¯months, and potentially at 6â¯months where there are risk factors for early relapse. We evaluated these recommendations regarding the management of our own patients. PATIENTS AND METHODS: Our single-center retrospective study included all patients with pemphigus diagnosed between 01/2015 and 10/2020 and receiving at least one initial infusion of RTX. We collected the following data: type of pemphigus, severity, levels of anti-desmoglein 1 and 3 antibodies at diagnosis and between 2 and 6â¯months after initial RTX, presence or absence of maintenance therapy and modalities, time to first relapse and duration of associated systemic corticosteroid therapy ≥5â¯mg/day. Maintenance treatment modalities were as follows: no maintenance treatment, maintenance "on demand" (MT1) i.e. not performed at the rate imposed by the NDCP, and maintenance "according to NDCP" (MT2). RESULTS: Fifty patients were included (women 54%, median age 58â¯years, pemphigus vulgaris 68%, moderate to severe 68%). Initial RTX was combined with systemic corticosteroid therapy at 0.5 to 1â¯mg/kg in 74% of cases. Twenty-seven patients (54%) received no maintenance therapy, 13 were on an MT1 regimen (26%), and 10 were on an MT2 regimen (20%). Median follow-up was 42â¯months. At the last follow-up, 39 patients (78%) were in complete remission. A total of 25 patients (50%) relapsed: 18/27 (67%) patients without maintenance, 5/13 (38%) with MT1, and 2/10 (20%) with MT2 (pâ¯=â¯0.026). The probability of relapse over time was significantly lower in patients receiving maintenance therapy compared to those who receiving none (pâ¯=â¯0.022). The median time to relapse was 15â¯months in patients without maintenance, and 30 and 28 in those with maintenance (pâ¯=â¯0.27). The median duration of systemic corticosteroid therapyâ¯≥â¯5â¯mg/day in the no-maintenance group was 10â¯months, compared to 7 and 9â¯months respectively in MT1 and MT2 (pâ¯=â¯0.91). CONCLUSION: Our study confirms the value of RTX maintenance therapy in pemphigus in real life.