Primary immunodeficiency diseases in different age groups: a report on 1,008 cases from a single Brazilian reference center.

Primary immunodeficiencies (PIDs) represent a large group of diseases that affect all age groups. Although PIDs have been recognized as rare diseases, there is epidemiological evidence suggesting that their real prevalence has been underestimated. We performed an evaluation of a series of 1,008 infants, children, adolescents and adults with well-defined PIDs from a single Brazilian center, regarding age at diagnosis, gender and PID category according to the International Union of Immunological Societies classification.

Immune response and tolerability of varicella vaccine in children and adolescents with systemic lupus erythematosus previously exposed to varicella-zoster virus.

Objectives: The aim of the present paper is to evaluate the immune response and tolerability of varicella vaccine in children and adolescents with systemic lupus erythematosus previously exposed to varicella-zoster virus.

Methods: We performed a prospective and controlled study on a group of 54 SLE patients that were chosen at random to be or not to be vaccinated (28 were vaccinated and 26 were not). Twenty-eight healthy controls, of matching age and sex were also vaccinated.

Vaccination practice in children with rheumatic disease.

Introduction/objectives: Evaluate clinical practice through assessment of vaccination card and recommendation of specific vaccines in pediatric patients with rheumatic diseases in use of different drugs and reveal the possible association between vaccination frequency and time of the clinical practice of pediatric rheumatologists in the state of São Paulo.

Material And Methods: A questionnaire was sent to pediatric rheumatologists of the Departamento de Reumatologia da Sociedade de Pediatria de São Paulo. This instrument included questions about practice time on Pediatric Rheumatology, vaccination of patients with juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), and immunization according to the treatments used.

Understanding systemic lupus erythematosus physiopathology in the light of primary immunodeficiencies.

Introduction: Associations between systemic lupus erythematosus (SLE) and primary immunodeficiencies (PIDs) were analyzed to gain insight into the physiopathology of SLE. Some PIDs have been consistently associated with SLE or lupus-like manifestations: (a) homozygous deficiencies of the early components of the classical complement pathway in the following decreasing order: in C1q, 93% of affected patients developed SLE; in C4, 75%; in C1r/s, 57%; and in C2, up to 25%; (b) female carriers of X-linked chronic granulomatous disease allele; and (c) IgA deficiency, present in around 5% of juvenile SLE.

Discussion: In the first two groups, disturbances of cellular waste-disposal have been proposed as the main mechanisms of pathogenesis.

Gonad evaluation in male systemic lupus erythematosus.

Objective: To assess gonad function in male patients with systemic lupus erythematosus (SLE).

Methods: Thirty-five consecutive male patients with SLE according to the criteria of the American College of Rheumatology were prospectively evaluated for demographic and clinical features as well as previous and current treatment. Patients underwent urologic evaluation and testicular Doppler ultrasound.

Renal involvement in Henoch-Schönlein purpura: a multivariate analysis of initial prognostic factors.

Objectives: To identify initial predictive factors of renal involvement in children and adolescents with Henoch-Schönlein purpura.

Methods: We reviewed the medical records of 142 patients admitted to our University Hospital over a 21-year period with a diagnosis of Henoch-Schönlein purpura. The initial predictive factors assessed, observed during the first 3 months, included: demographic data, clinical manifestations (persistent palpable purpura, arthritis, abdominal pain, severe abdominal pain, gastrointestinal bleeding, orchitis, central nervous system involvement and pulmonary hemorrhage), laboratory tests (serum IgA levels) and treatment given (corticosteroids, intravenous immunoglobulin and immunosuppressive drugs).

[Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis].

Objective: To describe the characteristics of macrophage activation syndrome associated with juvenile idiopathic arthritis.

Description: This is a retrospective study involving 462 patients with juvenile idiopathic arthritis. Seven (1.

[Chronic recurrent multifocal osteomyelitis of the mandible: report of three cases].

Objective: To report three cases of chronic recurrent multifocal osteomyelitis of the mandible, an inflammatory disease affecting one or more bones with absence of isolated microorganisms in affected areas.

Description: The first case is a 13 year-old female presenting with pain and fever after dental treatment. The patient received antibiotic treatment for osteomyelitis, but developed progressive enlargement of the mandible and palmoplantar pustulosis.