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BY-NC-ND 3.0 license Open Access Published by De Gruyter Open Access March 1, 2008

Fulminant Wegener’s granulomatosis with multiorgan dysfunction

Andreja Sinkovic EMAIL logo and Artur Pahor
From the journal Open Medicine


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.

[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 in Google Scholar PubMed

[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 in Google Scholar PubMed PubMed Central

[3] Griffith M., Brett S., The pulmonary phisicion in critical care. Illustrative case 3: pulmonary vasculitis., Thorax, 2003, 58, 543–546 in Google Scholar PubMed PubMed Central

[4] Schwarz M.I., Brown K.K., Small vessel vasculitis of the lung., Thorax, 2000, 55, 502–510 in Google Scholar PubMed PubMed Central

[5] Holc I., Pahor A., Krajnc I., Wegener’s granulomatosis — a diagnostic challenge, Wien Klin Wochenschr, 2000, 112, 680–683 Search in Google Scholar

[6] Loscar M., Hummel T., Haller M., Briegel J., Wiebecke B., Samtleben W., et al., ARDS und Wegener Granulomatose, Anesthesist, 1997, 46, 969–973 in Google Scholar PubMed

[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 in Google Scholar PubMed PubMed Central

[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 in Google Scholar PubMed

[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 Search in Google Scholar

[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 in Google Scholar PubMed PubMed Central

[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 Search in Google Scholar

[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 in Google Scholar PubMed

Published Online: 2008-3-1
Published in Print: 2008-3-1

© 2008 Versita Warsaw

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

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