Real-world Risk of Relapse of Giant Cell Arteritis Treated With Tocilizumab: A Retrospective Analysis of 43 Patients.

OBJECTIVE: Tocilizumab (TCZ), an interleukin 6 (IL-6) receptor antagonist, is approved for giant cell arteritis (GCA) as a cortisone-sparing strategy and in refractory patients. This study assessed the real-world efficacy, safety, and long-term outcomes of patients with GCA treated with TCZ. METHODS: We conducted a multicenter retrospective observational study at 3 French centers. All patients aged ≥ 50 years who met the American College of Rheumatology (ACR) criteria, and had received at least 1 dose of TCZ were included. Relapse was defined by therapeutic escalation, such as increased doses of corticosteroids (CS), resumption of CS after weaning, or introduction or intensification of adjuvant therapy. RESULTS: Between 2013 and 2019, 43 patients were included. Patients were followed up for a median 511 days between GCA diagnosis and inclusion, with 34/43 (79%) patients experiencing relapses. At inclusion, median age was 77 years, and median dose of CS was 15 mg/day. After inclusion, the mean cumulative dose of CS was 2.1 g/year vs 9.4 g/year before inclusion (P < 2 × 10-7), with 12/43 (28%) patients experiencing relapses on TCZ. Among 29 patients undergoing TCZ discontinuation, 18 (62%) experienced relapses. Factors associated with relapse after inclusion were introduction of TCZ > 6 months after diagnosis (P = 0.005), absence of ischemic signs at diagnosis (P = 0.006), relapse rate > 0.8/year (P = 0.03), and absence of CS tapering ≤ 5 mg/day (P = 0.03) before inclusion. Serious adverse events occurred in 18/43 patients (42%), including 4 deaths. CONCLUSION: Our results confirm the effectiveness of TCZ for CS sparing, but after discontinuation of treatment, TCZ allows for a prolonged remission in < 50% of patients. Attention must be paid to the tolerance of this long-term treatment in this elderly, heavily treated refractory population.

Association of skin hyperpigmentation disorders with digital ulcers in systemic sclerosis: Analysis of a cohort of 239 patients.

BACKGROUND: Skin pigmentation disorders in systemic sclerosis (SSc) have been sparsely described in the literature. Nevertheless, they could be a diagnostic and/or severity marker. OBJECTIVES: To assess the association between pigmentation disorders and systemic involvement in patients with SSc. METHODS: A total of 5 patterns of skin pigmentation disorders were defined: diffuse hyperpigmentation; hyperpigmentation of sun-exposed areas; hypopigmentation of the head, neck, and/or upper part of the chest; acral hypopigmentation; and diffuse hypopigmentation. RESULTS: A total of 239 patients were included; 88 patients (36.8%) had skin pigmentation disorders as follows: diffuse hyperpigmentation and hyperpigmentation of sun-exposed areas in 38.6% (n = 34) and 27.3% (n = 24) of patients, respectively; hypopigmentation of the face, neck, and/or chest in 10.2% of patients (n = 9); diffuse hypopigmentation in 12.5% (n = 11); and acral hypopigmentation in 17% (n = 15). Diffuse hyperpigmentation was associated with diffuse SSc (P = .001), increased modified Rodnan skin score (P = .001), and shorter duration of Raynaud phenomenon (P = .002) in univariate analysis but not in multivariate analysis. Moreover, diffuse hyperpigmentation was associated with digital ulcers (P = .005), as confirmed by multivariate analysis (odds ratio, 2.96; 95% confidence interval, 1.28-6.89). LIMITATIONS: This was a single-center retrospective study of a cohort of patients with SSc. CONCLUSION: Screening for skin pigmentation disorders could be useful in the management of patients with SSc to identify those with a high risk of development of digital ulcers, which is a symptom of vascular involvement in SSc.