Among the spectrum of disease manifestations associated with systemic lupus
erythematosus, lupus nephritis is particularly concerning due to the potential for
renal failure. This autoimmune attack may not, however, be limited to the kidney and
is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome
(aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies
(TMAs) – a group of disorders characterized by microangiopathic hemolytic
anemia, thrombocytopenia, and end organ damage. Although plasma exchange is
considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA
classically associated with autoimmune depletion of ADAMTS-13 – aHUS
demonstrates less reliable responsiveness to this modality. Instead, use of the late
complement inhibitor Eculizumab has emerged as an effective modality for the
management of such patients. Diagnosis of aHUS, however, is largely clinically based,
relying heavily upon a multidisciplinary approach. Herein we present the case of a
patient with atypical HUS successfully treated with Eculizumab in the setting of
Class IV-G (A) lupus nephritis and hypocomplementemia.
Adherence to central line insertion practices can significantly reduce infections and is used as a hospital benchmark for quality. However current national standards for CLIP compliance calculation do not include missing CLIP forms. We found adherence rates significantly decreased when accounting for all lines at one academic medical center.
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