[Intravenous immunoglobulin treatment in autoimmune diseases]. / La terapia con immunoglobuline endovena nelle malattie autoimmuni.

Our experience with intravenous immunoglobulin (IVIg) in autoimmune diseases is reported. In 16 subjects with polymyositis and dermatomyositis, IVIg has been given in case of steroid resistance or dependency. Subjects treated with IVIg achieved a clinical and functional remission in a higher percentage (81%), that was maintained after a mean five year follow-up period (p < 0.001), as compared to control group. In twelve subjects with new-onset Churg-Strauss disease, IVIg was added to standard treatment. In these patients, IVIg permitted to achieve a long-term stable remission with a good functional recovery with lower incidence of the steroid-associated side effects. In conclusion, IVIg can be safely employed in subjects with immune-mediated diseases, even in those with severe and refractory disease.

Impact of treatment on survival in polymyositis and dermatomyositis. A single-centre long-term follow-up study.

OBJECTIVE: To assess the long-term outcome in polymyositis (PM) and dermatomyositis (DM), with a particular emphasis on mortality and influence of treatment. METHODS: Diagnosis was based according to the Bohan and Peter's criteria. Patients have been followed up by a standardised protocol. Deaths were registered and causes of death were ascertained. Survival probability at 5 and 10years was estimated according to the Kaplan-Meier method, in the overall series and by a diagnostic group and an initial treatment. Mortality hazard ratios (95% CI) for major clinical and demographic features were estimated through univariate and multivariate Cox proportional hazard models. RESULTS: 91 patients (43 PM and 48 DM) were available for the study. Baseline characteristics were not different from those previously reported. Twenty-two patients (24%) died after a median follow-up of 8.7years. As for idiopathic myositis, the survival probabilities at 5 and 10years from the diagnosis were 96.2% and 88.8% for PM respectively; and 93.9% for DM, whereas a higher mortality was documented for cancer-associated myositis and overlap myositis. Male sex [HR=2.4, 95% CI 1.0 to 5.6], heart involvement (HR=1.8), interstitial lung disease (HR=2.3) and arthritis (HR=1.8) increased the risk of mortality, these risk excesses were confirmed in the multivariate analysis. Independent of these features, a higher mortality was documented for patients treated with glucocorticoids (HR=2.3) or immunosuppressants (HR=2.1) when compared to patients treated with immunoglobulins. CONCLUSION: Our study, with longitudinal and statistical analyses, suggests that survival has considerably increased in patients with PM/DM. Prognostic factors for mortality are male sex, and heart and lung involvement. Immunoglobulin treatment, intravenously or subcutaneously, is associated with a better survival.

Management of a pregnant woman with common variable immunodeficiency and previous reactions to intravenous IgG administration.

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.

Recurrent myelitis in common variable immunodeficiency successfully managed with high-dose subcutaneous immunoglobulin.

Acute myelitis is an aetiologically heterogeneous inflammatory disorder of the spinal cord. We report on a 71-year-old woman with a recurrent cervical and thoracic myelitis who presented with a new relapse of the disease. Neuromyelitis optica was ruled out such as other possible causes of acute and/or recurrent myelopathy. Serum immunoglobulin levels and specific antibody responses were consistent with the diagnosis of common variable immunodeficiency (CVID). She was treated with high-dose methylprednisolone and intravenous immunoglobulin. As a remission-maintaining drug, we decided to treat her with subcutaneous immunoglobulin (CSL Behring) at 0.2 g/kg/week at doses higher than usually employed in replacement therapy in CVID. At 3-year follow-up, the response to treatment was good. No relapses occurred. Our case suggests the effectiveness and safety of subcutaneous immunoglobulin in maintaining remission and in sparing prednisone in a woman with recurrent myelitis associated with CVID.

Intravenous Immunoglobulin and Mycophenolate Mofetil for Long-Standing Sensory Neuronopathy in Sjögren's Syndrome.

Sensory neuronopathy is described in association with the Sjögren's syndrome (SS). We studied a 55-year-old woman with a 4-year history of progressive asymmetric numbness, distal tingling, and burning sensation in upper and lower limbs. In a few months, she developed ataxia with increased hypoanaesthesia. Electrodiagnostic tests revealed undetectable distal and proximal sensory nerve action potential in upper and lower limbs. Cervical spine magnetic resonance showed a signal hyperintensity of posterior columns. Previous treatment with high-dose glucocorticoids and azathioprine was ineffective. A combined treatment with intravenous immunoglobulin and mycophenolate mofetil was followed by a progressive and persistent improvement. This case documented the efficacy and the safety of the coadministration of intravenous immunoglobulin and mycophenolate mofetil in sensory neuronopathy associated with SS refractory to conventional immunosuppressive therapy.

Subcutaneous immunoglobulin in polymyositis and dermatomyositis: a novel application.

OBJECTIVES: To describe the feasibility and safety of subcutaneous immunoglobulin (SCIg) in polymyositis (PM) and dermatomyositis (DM). METHODS: Since 2009, we used SCIg to treat patients with severe idiopathic myositis (4 with DM, 3 with PM), diagnosed according to the Bohan and Peter's criteria. SCIg (Vivaglobin®; CSL Behring) was administered by a programmable pump at the patient's usual IVIg monthly dose fractioned into equal doses given subcutaneously at weekly intervals. A standardized protocol was used to evaluate patients and to assess disease activity, treatment response and quality of life. RESULTS: Seven female patients were studied. All were Caucasians, with a median age of 53 years and a median disease duration of 72 months. The median follow-up period was 14±4 months. During treatment period, no relapse of the disease occurred. All patients showed a favourable clinical response and reported a good tolerance to the treatment with an improved quality of life. CK serum levels decreased over time with a concomitant improvement in MRC and Rankin modified scores. Three patients were able to discontinue the immunosuppressant and all to reduce the daily maintenance prednisone dose. CONCLUSIONS: Our experience demonstrates the beneficial effect and the safety of SCIg administration in active and refractory inflammatory myopathies.