[Evaluation of the effectiveness of treatment with rituximab associated to cyclophosphamide in patients with resistant idiopathic inflammatory myopathy].

Background And Objective: We report our experience with rituximab plus cyclophosphamide in the treatment of patients with resistant idiopathic inflammatory myopathies.

Patients And Method: Open-label uncontrolled prospective study on 17 patients.

Results: Evaluation was completed after 1, 6 and 12 months in 95'2, 85'7 y 52'4% of cycles, respectively.

[Thyroid dysfunction in patients with pulmonary arterial hypertension. A cohort study of 58 patients].

Background And Objective: Thyroid disease (TD) is more prevalent in patients with pulmonary arterial hypertension (PAH) than in the general population. The frequency and the cause of this association are not well established. We aimed to quantify and analyze the incidence and characteristics of TD in a cohort of PAH patients (idiopathic or preferentially associated with systemic diseases) and review the literature.

[Efficacy of rituximab combined with cyclophosphamide in a patient with systemic lupus erythematosus and peritoneal vasculitis refractory to conventional inmunosupressive therapy].

Peritoneal vasculitis is a rare and severe clinical manifestation of systemic lupus erythematosus. We report a patient who presented with ascites due to peritoneal vasculitis and cutaneous, articular, hematological and renal inflammatory activity. Treatment with glucocorticoids and immunosuppressive drugs was ineffective.

[Experience with imatinib to treat pulmonary arterial hypertension].

Despite advances in the treatment of patients with pulmonary arterial hypertension (PAH), survival has not improved greatly (is still very affected). Imatinib, an antagonist of platelet-derived growth factor with antiproliferative activity, has been effective in experimental models and clinically in several published reports. We report the results of imatinib therapy in 4 patients with PAH (functional class IV) who were refractory to treatment with drug combinations for this condition.

[Rituximab for treatment of patients with systemic autoimmune diseases].

Background And Objective: To assess the value of rituximab in systemic autoimmune diseases which are refractory to others treatments.

Patients And Method: Prospective study on 12 patients -7 with systemic lupus erythematosus (SLE), 4 with Wegener's granulomatosis (WG), and 1 with overlapping connective disease and autoimmune thrombocytopenia-, controlled in a specialized unit of a tertiary hospital. Four weekly doses of rituximab, 2 biweekly doses of cyclophosphamide, and glucocorticoids were administered to all patients, and other immunosuppressants were also administered as considered necessary in each case.

[Transverse myelitis in systemic lupus erythematosus].

Objective: Transverse myelitis (TM) is a rare complication in patients with systemic lupus erithematosus (SLE). We reviewed a series of our SLE patients to determine the prevalence of TM, and evaluate the clinical characteristics, medical tests, evolution and response to the treatment.

Patients And Method: Six patients with TM were identified and underwent a neurological evaluation, MRI, electrophysiologic study and were all subjected to the same treatment.

[Complete auriculoventricular blockage in adult patients with systemic lupus erythematosus. Case series and a review of the literature].

Background And Objective: Congenital complete atrioventricular heart block (CHB) is due to the lesion of the cardiac conduction system by specific transplacental antibodies of maternal origin. In adults with systemic lupus erythematosus (SLE), cardiac toxicity is very questionable and has been related to treatment with synthetic antimalarial drugs (AM). Here we evaluate, in our geographic area, the presence of non congenital CHB in adult patients with SLE and its possible association with AM treatment.

[Vogt-Koyanagi-Harada disease. Characteristics of a series of Andalusian patients].

Background: To describe the characteristics of a series of patients diagnosed of Vogt-Koyanagi-Harada disease (VKHD) and controlled by an Uveitis Unit (composed of ophthalmologists and internists) in our population.

Patients And Method: Retrospective descriptive study of 11 patients with VKHD (5 males and 6 women; median age at diagnosis 32.6 years old) followed-up between 1980 and 2003.

[Experience with rituximab in the treatment of systemic erythematosus lupus].

Objective: To assess the short-term effectiveness and tolerance of rituximab in patients with systemic lupus erythematous and distinct clinical manifestations.

Patients And Methods: Thirteen patients were studied. Rituximab (RTX) was indicated for refractory nephritis in 6 patients, severe thrombocytopenia in 5, aplastic anemia in 1 and peritoneal vasculitis associated with nephritis in 1.

[Iloprost for severe Raynaud's phenomenon and ischaemic ulcers related with systemic diseases].

Background And Objective: To evaluate the long term efficacy of treatment with intravenous iloprost for severe Raynaud's phenomenon (RP) and ischemic leg ulcers in patients with autoimmune systemic diseases.

Patients And Method: Prospective observational study over 2 years with iloprost (intravenous infusions, 0.5 to 2 ng/kg/min, initial cycle of 5 consecutive days and maintenance infusions during 24 h monthly, lengthened when it was needed) in patients with severe RP and ischemic leg ulcers whithout response to conventional therapy.

[Combined treatment with intravenous prostacyclin and sildenafil in patients with pulmonary hypertension: report of 4 cases].

Background And Objective: Here we report the experience obtained from a combined treatment with intravenous (i.v) prostacyclin and oral sildenafil in patients with severe pulmonary hypertension (PHT) who had a poor response to prior treatment with prostacyclin alone.

Patients And Method: Sildenafil was added to the treatment in four patients with PHT (primary in two patients and secondary to collagenosis in the other two) with no adequate response to i.

[Iliopsoas abscess and systemic lupus erythematosus].

Iliopsoas muscle abscess (IPA) is an uncommon condition, and it is usually associated with immunosuppression. Three out of a cohort of 552 patients diagnosed of systemic lupus erythematosus (SLE) developing an IPA, are reported herein. Patients showed fever and other symptoms related to SLE.

[Usefulness of brain SPECT with HMPAO-99mTc and psychological tests for diagnosis of neurological involvement in Behçet's disease].

Background: The purpose of this study was to establish the usefulness of single photon emission computed tomography (SPECT) and psychological tests for diagnostic of neuro-Behçet (NB) and to evaluate the clinical significance of neurological symptoms that are difficult to interpret and asymptomatic abnormalities in diagnostic tests.

Patients And Method: Forty patients with Behçet's disease (BD) were enrrolled for being studied with magnetic resonance imaging (MRI), SPECT and psychological tests.

Results: MRI findings were abnormal in 52,9% of patients with neurological involvement and 23.