Murat YALÇINSOY, Sevim DÜZGÜN, Ferda AKSOY, Ebru DAMADOĞLU, Yağcı Leyla TUNCER, Adnan YILMAZ

A relapsing case of Wegener’s granulomatosis presenting as an endobronchial mass

Wegener granülomatozis (WG) nüksü sıktır. Akciğer tutulumu hastaların %85’inde meydana gelmesine rağmen, endobronşiyal lezyon yaygın değildir. Biz, endobronşiyal kitleyle seyreden bir WG nüks olgusunu sunduk. Ellialtı yaşında erkek hasta 14 aylık siklofosfamid ve prednizolon tedavisini ve 36 aylık tam remisyonu takiben nüks olarak başvurdu. İlk hastalıkta böbrek, akciğer, üst solunum yolu, deri, eklemler ve göz tutulumlu olgu olarak tanı konulmuştu. Akciğer grafisi bilateral yama tarzında konsolidasyonlar gösteriyordu. Sitoplazmik antinötrofil sitoplazmik antikorlar (c-ANCA) yüksek titredeydi. Tedavi sonrası c-ANCA negatifleşti. Nüks zamanında, nazal yakınmalar ve hemoptiziyle başvurdu. Göğüs grafisi sağ parakardiyak bölgede yoğunluk artışı gösteriyordu. Fiberoptik bronkoskopi sağ alt lob girişini totale yakın kapatan kitle saptadı. Hastaya 10 aydır siklofosfamid ve azalan dozlarda prednizolon verildi.

Endobronşiyal kitle gibi nükseden Wegener granülomatozis olgusu

Wegener’s granulomatosis (WG) relapse is frequent. Although lung involvement occurs in 85% of patients, endobronchial presentation of the disease is uncommon. We reported a relapsing case of WG presenting as an endobronchial mass. A 56- year-old man present ed with recurrence of WG following 14 months of cyclophosphamide and prednisolone therapy and 36 months of complete remission. At his first presentation, he was diagnosed as having WG with involvement of kidney, lung, upper airways, skin, joints and eyes. His chest X-ray showed bilateral patchy consolidation. Cytoplasmic-anti-neutrophil cytoplasmic antibodies (c-ANCA) was also present in high titres. c-ANCA was negative after therapy. At the time of relapse, he presented with nasal symptoms and hemoptysis. His chest X-ray showed right paracardiac opacity. Fiberoptic bronchoscopy revealed a mass lesion subtotally obstructing the proximity of right lower lobe. He has been given prednisolone in tapering doses and cyclophosphamide for 10 months.

PDF

___

1. van der Werf TS, Stegeman CA, Meuzelaar KJ, Timens W. Late recurrence of Wegener's granulomatosis presenting as solitary upper lobe pulmonary mass, Eur Respir J 1994; 7: 1365-68.
2. Yi ES, Colby TV. Wegener's granulomatosis. Semin Diagn Pathol 2001; 18:34-46
3. Jolly M, Molta C, Hoffman G. Wegener's granulomatosis: Pitfalls in the management of pulmonary disease. A case of Wegener's granulomatosis with a hilar mass. J Rheumatol 2000; 27:2511-2.
4. Langford CA, Hoffman GS. Wegener's granulomatosis. Thorax 1999; 54: 629-37.
5. Aberle DR, Gamsu G, Lynch D. Thoracic manifestations of Wegener granulomatosis: Diagnosis and course. Radiology 1990; 174: 703-9.
6. Lee KS, Kim TS, Fujimoto K, et al. Thoracic manifestation of Wegener's granulomatosis: CT findings in 30 patients. Eur Radiol 2003; 13: 43-51.
7. Lee SJ, Berry GJ, Husari AW. Wegener's granulomatosis presenting as right middle lobe obstruction. Chest 1993; 103: 1623-4.
8. Shin MS, Young KR, Ho KJ. Wegener's granulomatosis upper respiratory tract and pulmonary radiographic manifestations in 30 cases with pathogenetic considera tion. Clin Imag 1998; 22: 99-104.
9. Cordier JF, Valeyre D, Guillevin L, et al. Pulmonary Wegener's granulomatosis. A clinical and imaging study of 77 cases. Chest 1997; 97: 906-12.
10. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: An analysis of 158 patients. Ann Intern Med 1992; 116:488-98.
11. Weiner SR, Paulus HE. Treatment of Wegener's granulomatosis with cyclophosphamide: an outcome analysis.Arthritis Rheum 1987; 30: 16.
12. Mahr A, Girard T, Agher R, Guillevin L Analysis of factors predictive of survival based on 49 patients with systemic Wegener's granulomatosis and prospective followup. Rheumatology 2001; 40: 492-8.
13. Lie JT. Illustrated histopathologic classification criteria for selected vasculitis syndromes. Arthritis Rheum 1990; 33: 1074-87.
14. Anderson G, Coles ET, Crane M, et al. Wegener's granuloma. A series of 265 British cases seen between 1975 and 1985. A report by a Sub-committee of the British Thoracic Society Research Committee. Quart J Med 1992; 83:427-38.
15. Langford CA, Talar-Williams C, Barron KS, Sneller MC. Use of a cyclophosphamide-induction methotrexatemaintenance regimen for the treatment of Wegener's granulomatosis: Extended follow-up and rate of relapse. Am J Med 2003; 114: 463-9.
16. Dugowson CE, Aitken ML. unusual presentation of recurrent Wegener's granulomatosis. Chest 1991; 99: 781-4.
17. Farelly CA. Wegener's granulomatosis: a radiological review of the pulmonary manifestations at initial presentation and during relapse. Clin Radiol 1982; 33:545-51.
18. Fauci AS, Haynes BF, Katz P, Wollf SM. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98: 76-85.
19. Foltz V, Koeger AC, de Sauverzax C, et al. Relapse of Wegener's granulomatosis. Concerning a case after 20 years of remission. Joint Bone Spine 2001; 68:262-6.