Macrophage Activation Syndrome as the Initial Manifestation of Severe Juvenile Onset Systemic Lupus Erythematosus. Favorable Response to Cyclophosphamide

Macrophage activation syndrome as the initial manifestation of severe juvenile onset systemic lupus erythematosus. Favorable response to cyclophosphamide.

The macrophage activation syndrome is a rare but potentially fatal complication of patients with autoimmune rheumatic diseases. This is a clinicopathological entity characterized by activation of histiocytes with prominent hemophagocytosis in the bone marrow and other reticuloendothelial systems. In patients with lupus it may mimic an exacerbation of the disease or infection. We report the case...

Oral contraceptive-related transverse sinus thrombosis as an initial manifestation of antiphospholipid syndrome in the absence of systemic lupus erythematosus

Autoimmune response in children with dengue. Case reports.

Dengue is an infectious disease caused by the dengue virus (DV), which can progress to dengue hemorrhagic fever and dengue shock syndrome. DV causes the production of auto-antibodies against human cells. A variety of factors have been associated with macrophage activation syndrome, including infections, drugs and autoimmune pathologies (systemic lupus erythematosus, systemic onset juvenile idio...

Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus

Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Ira...

PReS-FINAL-2186: Monoallelic mutations of familial hlh-related genes associated to macrophage activation syndrome

Introduction Macrophage Activation Syndrome (MAS) is a severe complication of rheumatic diseases, frequently associated with systemic juvenile idiopathic arthritis (sJIA), but also described in others pediatric inflammatory disorders including Juvenile Systemic Lupus Erythematosus (SLE) and Kawasaki disease. Due to the close resemblance to a group of histiocytic disorders collectively known as ...