Systemic onset Juvenile Idiopathic Arthritis (SoJIA) is rare pediatric disease, it accounts for 10% of children with Juvenile Idiopathic Arthritis. The onset of disese can be vary nonspecific and may suggest bacterial or viral infection, malignancy or other rheumatic disease. It is highly characterised by its extra-articular systemic illness features and, in some ways, it resembles a fever of unknown origin. Diagnosis is mostly clinical by using ILAR criteria (International League of Associations for Rheumatology).
Antiphosphospholipid syndrome (APS) secundary to systemic lupus erythematosus (SLE) can be recognised in children with arterial or venous thrombosis. Amaurosis due to thrombosis of central retinal vene is rarely presenting manifestation of SLE with secondary APS.
Common variable immunodeficiency (CVID) or acquired hypogammaglobulinemia is the type of primary immunodeficiency. Deregulation of the immune system, leading to hypogammaglobulinemia, defective activation and proliferation of T cells and dendritic cells, and malfunction of the cytokines are observed in CVID. The clinical picture of CVID varies, any organ or system can be affected, therefore the diagnosis is often difficult and delayed and sometimes is not always possible. This article describes a twelve years old boy with all the clinical signs of immunodeficiency, as confi rmed by laboratory. The main treatment consists of life-long immunoglobulin substitution in intravenous or subcutaneous form.
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