Again, in emergent situations, withholding biologics may not be possible. Therefore, the
physicians’ best judgment weighing benefits and risks of delaying surgery on morbidity and
mortality is crucial.
Mimics of Sepsis in Diseases of Immune Dysregulation
Macrophage activation syndrome (MAS) or hemophagocytic syndrome is a rare syndrome of
aberrant T-cell activation, resulting in diffuse phagocytosis of blood cells that occurs in patients
with autoimmune disease especially systemic-onset juvenile RA, Still’s disease, some immune
deficiencies, and systemic lupus erythematosus. MAS is fatal if not recognized and may mimic
a severe disease flare or sepsis or septic-disseminated intravascular coagulation. Presenting
signs may include persistent fever, neurologic symptoms such as mental status changes or
irritability suggestive of meningitis, splenomegaly, and rash. Laboratory values may show
pancytopenia, transaminase elevation, and coagulopathy with hypofibrinogenemia. Often
present is the discerning clue of a plummeting erythrocyte sedimentation rate (ESR) due to
consumption of coagulation proteins. Marked elevation of serum ferritin is often present.
Accepted treatment for MAS, in contrast to sepsis, includes high-dose glucocorticoids and
cyclosporine and may require intravenous immunoglobulin or plasmapheresis.
Thrombotic thrombocytopenic purpura (TTP)may occur as a secondary phenomenon to
autoimmune diseases such as systemic lupus erythematosus as well as to immunosuppressant
medications such as cyclosporine. It may mimic complications related to sepsis in a patient on
immunosuppressant medications. Diagnostic, clinical features, and treatment of secondary
TTP are the same as that for primary TTP.
Adverse Event Reporting
This chapter is built on systematic reviews of biologic agents and is reliant on data from
limited trials and through adverse event reporting systems (AERS) in the United States and
abroad (59). It is important to understand the shortcomings of passive reporting systems such
as in the States (60,61). Underreporting of adverse events is caused by an unrecognized
association resulting from transfer of care, length of time interval from treatment to event, and
lack of familiarity with these agents. Also, commonly acquired pathogens are less likely to be
reported (37). Clinicians may not be aware of reporting systems or how to access them. They
may not perceive reporting as a responsibility, or find the reporting system too cumbersome. It
is presumed that data presented here are incomplete in numbers and that serious infections are
of more relevance and far-reaching than this chapter would suggest (62). It is the inherent
responsibility of at least one treating physician to file a report and should be discussed with the
prescribing physician.
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