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Vasculitis

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Pediatric Nephrology in the ICU

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Core Messages

Vasculitis can present with a myriad of signs and symptoms, some of which are classic, but in many instances the presentation overlaps with other diseases (Table 17.1). The rarity of vasculitis in conjunction with an incomplete presentation may delay diagnosis, potentially increasing morbidity, and mortality. From a nephrological perspective, nephritis or hypertension typically leads to consultation. Work-up can be broad and might include laboratory testing, imaging, and biopsy (Table 17.2). Therapeutic options and aggressiveness are often dictated more by the severity of symptoms than the etiology of the disease:

  • Pulse dose steroids should be considered up front as empiric therapy as long as there is reasonable certainty regarding the absence of malignancy or underlying infection.

  • Patients with an immediately life-threatening presentation should also be considered for therapeutic plasma exchange, though its benefit is not always clear in all forms of vasculitis.

  • Cytotoxic agents have a proven role in most forms of vasculitis.

  • IVIG is less well studied (the exception being Kawasaki syndrome), but the literature is full of anecdotal successes with its use.

  • Newer immunomodulatory agents are also undergoing evaluation.

The outlook for many contemporary pediatric cases is not as bleak as it once was, particularly if the diagnosis is made early.

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  1. Department of Pediatrics, SUNY Upstate Medical University, 750 E. Adams St, Syracuse, NY, 13210, USA

    James A. Listman & J. Scott Schurman

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  1. Division of Pediatric Nephrology and Renal Transplantation, Kentucky Children's Hospital University of Kentucky, 740 South Limestone Street Room J460, Lexington, KY, 40536, USA

    Stefan G. Kiessling

  2. Nephrology and Hypertension Division Medical Director, Kidney Transplantation Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH, 45229-3039, USA

    Jens Goebel

  3. Division of Nephrology, Harvard Medical School Children's Hospital Boston, 300 Longwood Avenue, Boston, MA, 02115, USA

    Michael J. G. Somers

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Listman, J.A., Schurman, J.S. (2009). Vasculitis. In: Kiessling, S.G., Goebel, J., Somers, M.J.G. (eds) Pediatric Nephrology in the ICU. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-74425-2_17

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