Sustained clinical response after single course of rituximab as first-line monotherapy in adult-onset asthma and periocular xanthogranulomas syndrome associated with IgG4-related disease

Abstract

Rationale:IgG4-related disease (IgG4-RD) is an emerging immune-mediated disease characterized by multi-organ involvement and variable clinical behavior.Patient concerns:We describe the case of a 50-year-old woman affected by a rare variant of IgG4-RD, characterized by eyelid xanthelasmas, adult-onset asthma and salivary and lacrimal glands enlargement. Multiple lymphadenopathies and a pulmonary mass were present at initial evaluation.Inteventions:After a single course of rituximab (2g in 2 refracted doses), an almost complete clinical remission was achieved without chronic steroid administration.Outcomes:Magnetic resonance imaging (MRI), high-resolution computed tomography (HRCT) of the thorax, and positron emission tomography (18FDG-PET-CT) confirmed good response to treatment. Circulating plasmablasts dropped to undetectable levels as well. Xanthelasmas only remained unchanged. Remission persisted at 1-year follow-up.Lessons:Steroid therapy is still considered standard first-line therapy in IgG4-RD. However, high doses are generally required and relapses are common during the tapering phase. Rituximab is a well described steroid-sparing strategy, so far reserved to refractory cases only. In our experience, rituximab has been used as first-line monotherapy, showing great and sustained efficacy and optimal tolerability. The peculiar variant of IgG4-RD affecting our patient, the relatively low baseline plasmablast concentration, and the early placement of rituximab therapy may have facilitated the good response.

Percutaneous dilatational tracheostomy: A prospective analysis among ICU patients

“…Some studies have shown that the performance of tracheostomy early than later aids in easier nursing care, weaning and decreases the incidence of ventilator associated pneumonia. 29 However, the TracMan trial did not show any advantage of early (≤ 4 days) tracheostomy in terms of improvement in 30-day mortality and other important secondary outcomes. 30 Other studies indicated that the only advantage of an early tracheostomy to a later tracheostomy was the earlier weaning times.…”

“…30 Other studies indicated that the only advantage of an early tracheostomy to a later tracheostomy was the earlier weaning times. 29 In our hospital's setup, patient turnover and demand for ICU is very high. It is because of this reason that earlier weaning from mechanical ventilation is an important goal.…”

Adult-onset asthma and periocular xanthogranuloma – A rare infiltrative disease of the orbit and eyelid

“… 3 , 6 , 8 , 10 , 12 , 26 The addition of other immunosuppressive agents, such as cyclophosphamide and cyclosporin, have also shown efficacy in patients with this disease, 1 , 30 and it has been suggested that their respective B cell and T cell suppressive effects could be used to tailor treatments based on histopathological lymphocyte characteristics. 1 Rituximab has also shown to have a durable response in patients with AAPOX due to the likely B cell driven pathogenesis 14 , 31 , 32 though it remains unclear whether histological evidence of strongly positive CD20 + lymphocytes is necessary for successful treatment.…”

Prospects for Anti-B-Cell Therapy in Immuno-Inflammatory Rheumatic Diseases

“…Это послужило основанием для разработки новых подходов к фармакотерапии IgG4-СЗ, в том числе связанных с подавлением патологической активации В-клеток, играющих фундаментальныю роль в иммунопатогенезе заболевания [288]. Эффективность РТМ при IgG4-СЗ (около 90%) продемонстрирована в серии клини-ческих наблюдений [289][290][291][292][293][294][295][296][297][298][299][300][301], материалах национальных регистров [302,303] и открытом неконтролируемом исследовании [304]. Результаты применения РТМ при IgG4-СЗ подробно обсуждены в нашем обзоре [284].…”