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Journal

2008 | 3 | 1 | 119-122

Article title

Fulminant Wegener’s granulomatosis with multiorgan dysfunction

Content

Title variants

Languages of publication

EN

Abstracts

EN
Fulminant cases of Wegener’s granulomatosis (WG) with severe lung and kidney dysfunction may resemble pneumonia with sepsis and multiorgan dysfunction. Increased serum levels of antineutrophyl citoplasmic autoantibodies (c-ANCA) and proteinase 3 antibodies (PR3) confirm the correct diagnosis and are essential to start early immunosupressive treatment to improve the outcome. With the emphasis on diagnostic dilemmas, we present a 61-year old patient with fulminant WG, resembling pneumonia with sepsis and multiorgan dysfunction. After antibiotics and supportive measures increased serum levels of c-ANCA and PR3 confirmed the suspicion of WG and adequate immunosupressive treatment improved the patient’s condition. We concluded that it is important to include vasculitis in the differential diagnosis of unexplained multiorgan dysfunction, because only early adequate immunosuppressive therapy in combination with other resuscitation measures improves survival.

Publisher

Journal

Year

Volume

3

Issue

1

Pages

119-122

Physical description

Dates

published
1 - 3 - 2008
online
1 - 3 - 2008

Contributors

  • Department of Medical Intensive Care, Teaching Hospital Maribor, Maribor, Slovenia
author
  • Department of Rheumatology, Teaching Hospital Maribor, Maribor, Slovenia

References

  • [1] Dellinger R.P., Carlet J.M., Masur H., Gerlach H., Calandra T., Cohen J., et al., for the Surviving Sepsis Campaign Management Guidelines Committee, Surviving sepsis campaign management guidelines committee. Surviving sepsis campaign guidelines for management of severe sepsis and septic shock., Crit. Care Med., 2004, 32, 858–873 http://dx.doi.org/10.1097/01.CCM.0000117317.18092.E4[Crossref]
  • [2] Semple D., Keogh J., Forni L., Venn R., Clinical review: Vasculitis on the intensive care unit-part 1: diagnosis, Crit. Care, 2005, 9, 92–97 http://dx.doi.org/10.1186/cc2936[Crossref]
  • [3] Griffith M., Brett S., The pulmonary phisicion in critical care. Illustrative case 3: pulmonary vasculitis., Thorax, 2003, 58, 543–546 http://dx.doi.org/10.1136/thorax.58.6.543[Crossref]
  • [4] Schwarz M.I., Brown K.K., Small vessel vasculitis of the lung., Thorax, 2000, 55, 502–510 http://dx.doi.org/10.1136/thorax.55.6.502[Crossref]
  • [5] Holc I., Pahor A., Krajnc I., Wegener’s granulomatosis - a diagnostic challenge, Wien Klin Wochenschr, 2000, 112, 680–683
  • [6] Loscar M., Hummel T., Haller M., Briegel J., Wiebecke B., Samtleben W., et al., ARDS und Wegener Granulomatose, Anesthesist, 1997, 46, 969–973 http://dx.doi.org/10.1007/s001010050494[Crossref]
  • [7] Semple D., Keogh J., Forni L., Venn R., Clinical review: Vasculitis on the intensive care unit - part 2: treatment and prognosis, Crit. Care., 2005, 9, 193–197 http://dx.doi.org/10.1186/cc2937[Crossref]
  • [8] Vassilopoulos D., Niles J.L., Villa-Forte A., Arroliga A.C., Sullivan E.J., Merkel P.A., et al., Prevalence of antineutrophil cytoplasmatic antibodies in patients with various pulmonary diseases or multiorgan dysfunction, Arthritis. Rheum., 2003, 49, 151–155 http://dx.doi.org/10.1002/art.10997[Crossref]
  • [9] Castelli G.P., Pognani C., Cita M., Studin A., Sgarbi L., Paladini R., Procalcitonin, C-reactive protein, white blood cells and SOFA score in ICU: diagnosis and monitoring of sepsis, Minerva. Anestesiol., 2006, 72, 69–80 [PubMed]
  • [10] Savage C.O.S., Harper L., Cockwell P., Adu A., Howie A.J., ABC of arterial and vascular disease: Vasculitis, B.M.J., 2000, 320, 1325–1328 http://dx.doi.org/10.1136/bmj.320.7245.1325[Crossref]
  • [11] Calabrese L.H., Duna G., Antineutrophil cytoplasmic antibody-associated vasculitis, In: Harris ED, Budd RC, Firestein GS, Genovese MC, Gargent JS, Ruddy S, et al., (Eds.), Kelley’s textbook of rheumatology, 7th ed., Elsevier Saunders, Philadelphia 2005
  • [12] Cruz B.A., Ramanoelina J., Mahr A., Cohen P., Mouthon L., Cohen Y., et al., Prognosis and outcome of 26 patients with systemic necrotizing vasculitis admitted to the intensive care unit. Rheumatology, 2003, 42, 1183–1188 http://dx.doi.org/10.1093/rheumatology/keg322[Crossref]

Document Type

Publication order reference

Identifiers

YADDA identifier

bwmeta1.element.-psjd-doi-10_2478_s11536-007-0067-6
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