Wegener’s granulomatosis with thrombotic thrombocytopenic purpura – neurologic manifestations


Authors: J. Vítová
Authors‘ workplace: Interní oddělení Nemocnice Č. Budějovice, a. s.
Published in: Čes. Revmatol., 16, 2008, No. 2, p. 92-96.
Category: Case Report

Overview

Wegener’s granulomatosis (WG) is a systemic vasculitis of medium-sized and small-sized vessels. Most of all, the kidneys and respiratory tract, peripheral nerves, less frequently CNS are affected with either vasculitis or granulomas. Other organs may be affected, as well. Thrombotic thrombocytopenic purpura (TTP) is a disease caused by deficiency of von Willebrand factor’s protease resulting in hyperadhesiveness of thrombocytes with various clinical consequences. The case report of a patient with coincidental course of WG and TTP is described. The disease was recurrent, diffusing and potentiating the clinical manifestations, which took on dramatic picture. The case draws attention to the possibility of combination of two rare conditions and the need of thorough examination of clinical manifestations and laboratory findings, which do not fit in the image of one disease.

Key words:
Wegener’s granulomatosis, thrombotic thrombocytopenic purpura, neurologic manifestations


Sources

1. Oimomi M, Suehiro I, Mizuno N, Baba S, Okada S, Kanazawa Y. Wegener’s granulomatosis with intracerebral granuloma and mammary manifestation. Report of a case. Arch Intern Med. 1980 Jun; 140 (6): 853–854.

2. Shuang Ye, Cheng de Yang. How could we make a diagnosis of Wegener’s granulomatosis? Clin Rheumatol 2007; 26: 784–786.

3. Bartůňková J, Tesař V, Šedivá A. Diagnosis and pathogenetic role of antineutrophil cytoplasmic autoantibodies. Clin Immunol 2003; 106: 73–82.

4. Bjerkeli V, Damäs JK, Fevang B, Holter JC, Aukrust P, Froland SS. Increased e expression of fractalkine (CX3CL1) and its receptor, CX3CR1 in Wegener’s granulomatosis – possible role in vascular inflammation. Rheumatology (Oxford) 2007 Sep; 46 (9); 1422–1427.

5. Elkon K, Sutherland DC, Rees A, et al. HLA-A antigens of patients with Wegener’s granulomatosis. Tissue Antigens 1978; 11: 129.

6. Ronco P,Verroust P, Mignon F, Kourilsky O, Vanhille P, Meyrier A, et al. Immunopathological studies of polyarteritis nodosa and Wegener’s granulomatosis: a report of 43 patients with 51 renal biopsies. Q J Med.1983 Spring; 52 (206): 212–223

7. Dervenoulas J, Tsirigotis P, Bollas G, Pappa V, Xiros N, Economopoulos T, et al. Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS): treatment outcome, relapses, prognostic factors. A single-center experience of 48 cases Ann Hematol: 2000; 79: 66–72.

8. Olivencia - Simmons I. Wegener’s granulomatosis: symptoms, diagnosis and treatment. J Am Acad Nurse Pract 2007 Jun; 19 (6): 315–320.

9. Finkielman JD, Lee AS, Hummel AM, Viss MA, Jacob GL, Homburger HA, et al. WGET Research Group. ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis. Am J Med 2007 Jul; 120(7); 643.e9–14.

10. Ahmed M, Niffenegger JH, Jakobiec FA, Ben-Arie-Weintrob Y, Gion N, Androudi S, et al. Diagnosis of limited ophthalmic wegener granulomatosis: distinctive pathologic features with ANCA test confirmation. Int Ophthalmol Jun 23 (Epub ahead of print).

11. Lidar M, Carmel E, Kronenberg Y, Langevitz P. Hearing loss as the presenting feature of systemic vasculitis. Ann N Y Acad Sci. 2007 Jun; 1107: 136–141.

12. O’Donnell JL, Hayman MW, Spellerberg MB, McLellan AD, Brooksbank K, Chapman PT, et al. Antineutrophil cytoplasmic antipody measurement: advantages and disadvantages of a capture PR3 ELISA and a direct PR3 ELISA. Pathology 200 Apr; 39(2): 258–263.

13. Murphy JM, Gomez-Anson B, Gillard JH, Antoun NM, Cross J, Elliot JD, et al. Wegener granulomatosis, MR imaging findings in brain and meninges. Radiology 1999 Dec; 213 (3): 3: 794–799.

14. Kuross S, Davin T, Kjellstrand CM. Wegener’s granulomatosis with severe renal failure; clinical course and results of dialysis and transplantation. Clin.Nephrol 1981 Oct; 16 (4):172–180.

15. Hoffmann GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; Mar 15; 116 (6): 488–498.

16. Hoffmann GS, Leavitt RY, Fleisher TA, Minor JR, Fauci AS. Treatment of Wegener’s granulomatosis with intermittent high-dose intravenous cyclophosphamide. Am J Med 1990 Oct; 89 (4): 403–410.

17. Specks U, DeRemee RA. Granulomatous vasculitis. Wegener’s granulomatosis and Churg-Strauss syndrome. Rheum Dis Clin North Am 1990 May; 16 (2): 377–397.

18. Haubitz M. ANCA – associated vasculitis: diagnosis, clinical characteristics and treatment. Vasa 2007 May; 36 (2): 81–89.

19. Villa-Forte A, Clark TM, Gomes M, Carev J, Mascha E, Karafa MT, et al. Substitution of methotrexate for cyclophosphamide in Wegener granulomatosis: a 12- year single-practice experience. Medicine (Baltimore) 2007 Sep; 86 (5): 269–277.

Labels
Dermatology & STDs Paediatric rheumatology Rheumatology
Login
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account