Wegener granulomatosis; multisystem involvement

Abstract

Wegener Granulomatozis (WG) is a systemic autoimmune disorder characterized by necrotizing granulomatous vasculitis that primarily affects the upper and lower respiratory tracts and kidneys. We report a case of WG with multisystem involvement. This 39-year-old man has ocular, paranasal, mediastinal, lung, splenic and meningeal involvement of WG. We describe and correlate the CT,US and cytological findings. W egener Granulomatosis (WG) is a disorder characterized by necrotizing granulomatous vasculitis that primarily affects the upper and lower respiratory tracts and kidneys (1,2). The classic form of WG is a multisystem process that includes respiratory tract granulomas, generalized vasculitis and glomerulonephritis, whereas the limited form of WG involves focal disease without glomerulonephritis (3). We report a case of WG with multisystem involvement.

Keywords

• Wegener Granulomatosis
• CT
• US
• Cytological findings

Wegener granulomatozis; multisistem tutulumu

Abstract

Wegener Granülomatozis (WG) ender görülen, primer olarak üst ve alt solunum yolları ile böbreklerin nekrozitan granülomatöz vasküliti ile karakterize sistemik bir otoimmün hastalıktır. Biz multisistem tutulumu olan bir WG li olguyu sunmayı amaçladık. 39 yaşındaki erkek hastada oküler, paranazal, mediastinal, akciğer, dalak ve meningeal tutulum BT, US ve sitolojik bulguları ile sunuldu.

Wegener Granülomatozis (WG) ender görülen, primer olarak üst ve alt solunum yolları ile böbreklerin nekrozitan granülomatöz vasküliti ile karakterize sistemik bir otoimmün hastalıktır. Biz multisistem tutulumu olan bir WG li olguyu sunmayı amaçladık. 39 yaşındaki erkek hastada oküler, paranazal, mediastinal, akciğer, dalak ve meningeal tutulum BT, US ve sitolojik bulguları ile sunuldu.

Keywords

• Wegener Granülomatozis
• BT
• US
• sitolojik bulgular

References

1. Clark WJ, Broumand V, Ruskin JD et al: Erythematous, granular, soft tissue lesion of the gingiva. J Oral Maxillofac Surg 1998; 56:962-7.
2. Weinberger LM, Cohen ML, Remler BF et al. Intracranial Wegener’s Granulomatosis. Neurology 1993; 43:1831-1834.
3. Provenzale JM, Mukherji S, Allen NB et al. Orbital involvement by Wegener’s Granulomatosis. AJR 1996; 166:929-934.
4. Hoffman GS, Kerr GS, Leavitt RY et al. Wegener Granulomatosis: an analysis of 158 patient. Ann Intern Med 1992; 116:488-498.
5. Provenzale JM, Allen NB. Wegener Granulomatosis. AJNR 1996; 17:785- 792.
6. Wadsworth DT, Siegel MJ, Day DL. Wegener’s Granulomatosis in childeren: chest radioghraphic manifestations. AJR 1994;163:901-904.
7. Weir IH, Muller NL, Chiles C, et al. Wegener’s Granulomatosis: Şndings from computed tomography of the chest in 10 patients. Can Assoc Radiol J 1992; 43:31-34.
8. Geoge TM, Cash JM, Farver C et al. Mediastinal mass and hilar adenopathy: rare thoracic manifestations of Wegener’s Granulomatosis. Artritis Rheum 1997; 40:1992-1997.

9. Bullen CL, Liesegang TJ, McDonald TJ et al. Ocular complication of Wegener’s Granulomatosis. Ophthalmology 1997; 90:279-290.
10. Haynes B, Fishman ML, Fauci AS et al. Ocular complications of Wegener’s Granulomatosis: Şfteen years experience and review of the literature. Am J Med 1997; 63:131-141.
11. Drahman DD. Neurologıcal complications of Wegener’s Granulomatosis. Arch Neurol 1993; 8:145-155.
12. Fauci AS, Haynes BF, Katz P et al. Wegener’s Granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983; 98:76-85.
13. Andersen JM, Jamiesen DG, Jefferson JM. Non-healing granuloma and the nervous system. Q J Med 1975; 44:309-323.
14. Nishino H, Rubino FA, De Remee RA et al. Neurologıcal involvement in Wegener’s Granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol 1993; 33:4-9.
15. Kalaitzoglou I, Drevenlengas A, Palladas P et al. MRI Appearance of pulmonary Wegener’s Granulomatosis with concomitant splenic infarction. Eur Radiol 1998; 8:367-370.
16. Spranger M, Schwab S, Meinck HM. Meningeal involvement in Wegener's granulomatosis conŞrmed and monitored by positive circulating antineutrophil cytoplasm in cerebrospinal fluid. Neurology 1997; 48:263-5
17. Mc Hugh K, Manson D, Eberhard BA et al. Splenic necrosis in Wegener’s Granulomatozis. Pediatr Radiol 1991; 21:588-589.