No abstract

No abstract

The administration of 20 % SCIg was beneficial and safe in maintaining a quiescent disease and in inducing a complete remission in moderately active disease in patients with DM or PM. The treatment with 20 % SCIg led to the possibility to discontinue and to reduce the use of glucocorticoids and/or the immunosuppressants. Patients reported their satisfaction in terms of contact with health professionals, quality of treatment-related information and administration convenience, with an improved quality of life.

Common variable immunodeficiency is the most common symptomatic primary immunodeficiency in adulthood. Pregnant women with common variable immunodeficiency have different needs from other patients with the same disease. Because of immature state of the fetal and neonatal immune system, transplacental transfer of immunoglobulin G (IgG) has a relevant role in protecting the infant. We here report on a high-risk pregnant woman with common variable immunodeficiency with adverse reactions to intravenous immunoglobulin that was successfully rescued with a new Ig human intravenous, 10% liquid preparation. The treatment was tailored to the health status and characteristics of the patient. The new product was safe and well tolerated. The mother did not report any infections during pregnancy and the baby had a healthy course with ‘protective’ serum IgG levels. Our case is a further demonstration that intravenous immunoglobulin tolerability in patients with immunodeficiency could be linked to a product's characteristics.

Background We here report the incidence, the clinical features and treatment response of interstitial lung disease (ILD) associated to idiopathic inflammatory myopathies (IIM), Sjogren’s Syndrome (SS) and systemic lupus erythematosus (SLE). Methods We reviewed the clinical notes of 224 consecutive patients followed by our department from 1990 to 2011. IIM was present in 77 patients, SS in 100 subjects and SLE was documented in 87 cases. Demographic, clinical and serological data were collected through a standardised protocol. ILD was defined by specific spirometric and tomographic patterns. Results ILD was documented in 20 (26%) patients with IIM, in 15 (15%) with SS and in 13 (15%) with SLE. All patients were treated with steroid, alone or associated with an immunosuppressant. All of them had a restrictive pattern at the pulmonary function testing with a reduced diffusing capacity for carbon monoxide. In patients with SS- and LES- associated ILD the response to the treatment was satisfactory, whereas in patients with myositis, ILD-related deaths occurred in 25% of the cases. This percentage was higher when compared to the percentage of the disease-related death (17%). Conclusions Despite major progress in the treatment, ILD in the course of CTDs remain a severe complication, significantly affecting subjects’ survival and quality of life. The relatively high frequency of asymptomatic subjects, with subclinical ILD documented by the presence of a restrictive spyrometric pattern and typical radiological features, suggests the importance of early diagnosis. Disclosure of Interest None Declared

Objectives To evaluate the efficacy of subcutaneous of immunoglobulins (SCIg) in 13 patients with refractory polymyositis (PM) and dermatomyositis (DM). Methods We described 13 patients (10 female and 3 male) with PM (5) and DM (8), diagnosed according to the Bohan and Peter's criteria, with a mean age of 50 years and a mean disease duration of 72 months. Prior SCIg treatment, all patients received high dose oral glucocorticoids associated to several immunosuppressive agents with no sustained improvement. Mean follow-up period was 20 months. We used a standardized protocol of infusion consisting on the administration of a weekly dose of 0.2 g/kg/week of SCIg, by the means of a programmable pump, changing sites of administration every week. After a three weeks of in hospital training period, patients were switched to home management. Results Treatments response was evaluated using clinical and laboratory data. We documented a significant improvement on serum CK levels, MRC score and Rankin modified score. Prednisone daily dose was reduced in all patient. Neither systemic adverse events nor relapses were reported. Conclusions Our data showed the effectiveness and the good tolerance of SCIg as a valid treatment option in active and severe form of myopathies, also as steroid-sparing agents. However, a longer follow-up period is necessary to evaluate their efficacy as an alternative to intravenous route of administration (IVIg treatment). Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3610

Background We have previously demonstrated the beneficial effect and the safety of subcutaneous immunoglobulin (SCIg) administration in active and refractory inflammatory myopathies. Hizentra (IgPro20; CSL Behring) is the first, 20% liquid preparation of human IgG specifically formulated for subcutaneous infusions. We here report on the switch to weekly subcutaneous infusions of Hizentra(®) in inflammatory myopathies. Objectives We demontrate efficacy of the new formulation of subcutaneous immunoglobulins with quality of life improvement. Methods Six severe idiopathic myositis (four with DM, two with PM), diagnosed according to the Bohan and Peter’s criteria, were switched to weekly subcutaneous infusions of Hizentra(®) at doses equivalent to their previous subcutaneous treatment. A standardized protocol including the Medical Research Council (MRC) scale, the modified Rankin score, CK serum levels and daily prednisone dose, was used to evaluate patients and to assess disease activity, treatment response and quality of life. Results All patients showed a favourable clinical response with normal CK serum levels and improvement in MRC and Rankin modified scores. No relapse of the disease occurred: patients reported a good tolerance to Hizentra (®). Local reactions were mild and self-limiting (only one patient showed swelling). No serious Hizentra(®)-related adverse events were reported whereas they pointed an improved quality of life. Patients’ satisfaction was related to the shorter infusion times due to smaller infusion volumes, the easiness to handle the product and the possibility to store it a temperature up to 25°C. Conclusions Hizentra is an advance in the field of immunoglobulin therapy, which might offer benefits for home therapy, even in patients with autoimmune diseases. Disclosure of Interest None Declared

No abstract