Activation syndrome

Mast cells express high-affinity IgE Fc receptors (FceRI) on their surface, contain cytoplasmic granules which are major sources of histamine and other inflammatory mediators, and are activated to release and generate these mediators by IgE-dependent and non-IgE-dependent mechanisms [1]. Disturbances either in the release of mast cell mediators or in mast cell proliferation are associated with clonal mast cell disorders including monoclonal mast cell activation syndrome (MMAS) and mastocytosis respectively, which are in turn associated with some cases of anaphylaxis [2], Molecular mechanisms have been identified which may link increased releasability of mast cell mediators and conditions leading to increased mast cell numbers [3]. Patients with mastocytosis have an increased risk to develop anaphylaxis [4, 5] and those with anaphylaxis may suffer from unrecognized mastocytosis or may display incomplete features of the disease [6-8]. 

Military Activities Syndrome Environmental destruction through war and military action Loss of biodiversity due to chemical warfare agents, permanent soil degradation due to mining, contamination caused by fuels and explosives, health hazards, greater flows of refugees... 

Beneath the black box, a headline reads warnings—Clinical Worsening and Suicide Risk. Without identifying it as such, this section contains a warning about the stimulant or activation syndrome that I first described in Toxic Psychiatry in 1991 ... 

Etanercept has reportedly caused abrupt exacerbation of the macrophage activation syndrome (14). 

Because soluble tumor necrosis factor alfa receptors are supposedly involved in the macrophage activation syndrome, the authors speculated that the administration of additional soluble receptors may have been the cause of a prolonged and exacerbated syndrome. 

Stern A, Buckley L. Worsening of macrophage activation syndrome in a patient with adult onset Still s disease after initiation of etanercept therapy. J Clin Rheumatol 2001 7 252-6. 

Other, more specific, transporter dysfunctions lead to distinct inborn errors of metabolism. Oxaluria and cystinuria, defects in oxalate and cysteine transport, respectively, manifest with renal stones. Cystinuria specifically presents with cysteine, ornithine, lysine, and arginine in the urine. The latter should not be confused with cystinosis. Lysinuric protein intolerance (LPI) is a defect in the dibasic amino acid transporter. This results in a specific amino aciduria pattern (ornithine, lysine, and arginine), which in turn results in secondary inhibition of the urea cycle. Individuals affected by LPI are at risk for hyperammonemia and also have a unique susceptibility to macrophage activation syndrome, an exaggerated systemic inflammatory response, and alveolar proteinosis. Renal damage... 

Psychiatric Activation syndrome, a known adverse effect of antidepressants, has been defined by the US Food and Drug Administration as including the following symptoms irritability, anxiety, agitation,... 

Kimura T, Yuuki S, Kuga M, Hayashida H, Furukawa H, Takamatsu J. Activation syndrome caused by paroxetine in patients with dementia with Lewy bodies. Psychogeriatrics 2009 9 139-42. 

Hematologic Macrophage activation syndrome during anakinra treatment, which is specific for systemic juvenile idiopathic arthritis, occurred in three of 140 patients followed for a mean of 10 months [20 ]. 

Ricd M, Rossi P, De Marco G, Varisco V, Marchesoni A. Macrophage activation syndrome after lellunomide treatment in an... 

Haematologic About 7% of patients with systemic juvenile idiopathic arthritis develop macrophage activation syndrome leading to inflammatory actions. Two of three children with systemic juvenile idiopathic arthritis, all treated with tocilizumab, developed cytopenias and these two children also had a history of macrophage activation syndrome suggesting that patients with the syndrome may have an increased risk of cytopenias to the mAb [208 ]. 

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