GASTROİNTESTİNAL SİSTEM, RENAL VE AKCİĞER TUTULUMUNUN BİRLİKTELİĞİ: NADİR BİR WEGENER GRANULOMATOZU OLGUSU
Year 2014,
Volume: 28 Issue: 2, 113 - 118, 01.10.2014
Zühre Sarp Taymaz
Aydan Mertoğlu
Emel Tellioğlu
Günseli Balcı
Yasemin Yurt
Zekiye Aydoğdu
Abstract
Wegener granulomatozu (WG), özellikle üst ve alt solunum yolları ile böbrekleri tutabilen nekrotizan granulomatöz vaskülitle tanımlanan nadir sistemik bir hastalıktır. Bu hastalığın nadir de olsa başka organ sistemlerini de etkilediği bilinmektedir. 72 yaşında erkek hastamızda benign prostat hipertrofisi operasyonu için yapılan preoperatif değerlendirme için çekilen toraks bilgisayarlı tomografisinde “sağ akciğer üst lob posterior segment lokalizasyonunda geniş bir tabanla plevraya invazyon gösteren yaklaşık 4x5cm ebatlarda santralinde geniş nekroz alanları da saptanan düzensiz konturlu malign kitle lezyonu, sağ akciğer üst lob anterior segmentte subplevral parankimde yaklaşık 2x2.5cm çaplara ulaşan düzensiz sınırlı nodül” izlendi. Yapılan tetkiklerle sistemik tip WG tanısı kondu. Hastada sistemik tedaviye başlanmadan önce tetkikleri sırasında yaşamı tehdit eden hemoptizi ve gastrointestinal kanama gelişti. Hastada endoskopi ve kolonoskopi yapıldı. WG'un barsak tutulumu olduğu düşünülerek kortikosteroid ve siklofosfamid tedavisi başlandı ve tedaviyle dramatik yanıt alındı. Hastanın gastrointestinal kanamasının uygulanan kortikosteroid ve siklofosfamid tedavisi ile dramatik iyileşme göstermesi ve WG'da gastrointestinal sistem tutulumun nadir görülmesi nedeni ile hasta literatür bilgileri eşliğinde sunuldu.
References
- 1. Storesund B,Gran JT, Koldingnes W. Severe
intestinal involvement in Wegener Granulomatosis:
report of two cases and review of
literature. Br J Rheumotol 1998;37:387-390.
- 2. Walton EW. Giant-cell granuloma of the
respiratory tract (Wegener’s granulomatosis). Br
Med J 1958; 2: 265 -270.
- 3. C Pagnoux, A Mahr, P Cohen, L Guillevin, Loic MD,
Presentation and Outcome of Gastrointestinal
Involvement in Systemic Necrotizing Vasculitides:
Analysis of 62 Patients With Polyarteritis Nodosa,
Microscopic Polyangiitis, Wegener Granulomatosis,
Churg-Strauss Syndrome, or Rheumatoid
Arthritis-Associated Vasculitis, Medicine March
2005;Volume 84-Issue 2: 115- 128.
- 4. Deniz K, Özşeker HS, Balas S, Akpınar E,
Sökmensüer C. Intestinal involvement in
Wegener's granulomatosis. J Gastrointestin
Liver Dis. 2007 Sep;16(3):329 -31.
- 5. Haworth SJ, Pusey CD. Severe intestinal
involvement in Wegener’s granulomatosis.Gut
1984;25:1296-1300.
- 6. Beppu K, Osada T, Matsumoto K, Shibuya T,
Sakamoto N, Kawabe M, Nagahara A, Ogihara T,
Watanabe S, Intestinal involvement in Wegener’s
granulomatosis diagnosed and followed up by
double balloon enterescopy, Intern
Med.2011;50(3):219- 222.Epub 2011 Feb 1.
- 7. Nazif Zeybek, Faik Yaylak, Özcan Altinel, Özgur
Albuz, Ayper Kaya, Yusuf Peker, Multiple
Intestinal Perforation Due To Wegener’s
Granulotamosis: Case Report. J.Clin. Anal. Med.
2010; 1(2): 34 -36.
- 8. Tamer Akça, Tahsin Çolak, Mehmet
Çağlıkülekçi, Koray Öcal, Suha Aydın, Intestinal
perforation in Wegener’s granulomatosis: a case
report, Wegener granülomatozunda intestinal
perforasyon: Olgu Sunumu, Turkish Journal of
Trauma & Emergency Surgery Ulus Travma
Derg 2005
- 9. Leavitty RY, Fauci AS, Bloch DA,et al.The American
College of Rheumatology 1990 criteria fort he
classification of Wegener Granulomatosis.Arthritis
Rheum 33:1101-1107,1990
- 10. Fauci AS, Haynes BF, Katz P, Wolff SM.
Wegener’s Granulomatozis Prospective clinical
and therapeutic experience with 85 patients for
21 years Ann Intern Med. 1983; 98: 76- 85.
- 11. Takwoingi YM, Dempster JH. Wegener’s
Granulomatosis: an analysis of 33 patients seen
over a 10 year period. Clin Otolaryngol. 2003;
28: 187- 194.
- 12. Chow FY, Hooke D, Kerr PG. Severe intestinal
involvement inWegener’s granulomatosis. J
Gastroenterol Hepatol 2003; 18:749-750.
- 13. Veinot JP, Logan CA, Thomas MJ. Wegener’s
granulomatosis arteritis causing small bowel
infarction. Pathology 2003; 35:268-269.
- 14. Pulimood AB, Peter S, Ramakrishna BS, et al.
Segmental colonoscopic biopsies in the
differentiation of ileocolic tuberculosis from
Crohn’s disease. J Gastroenterol Hepatol
2005;20: 688-696.
- 15. Ozdemir Abbas, Seyahi Nurhan, Durak
Haydarand Kılıcarslan Isın. Intestinal Perforation
due to Vasculitis in a Patient with Wegener’s
Granulomatosis. BANTAO Journal 2009; 7 (1):
46-48.
GASTROINTESTINAL, RENAL AND PULMONARY İNVOLVEMENT: A RARE WEGENER GRANULOMATOSIS CASE
Year 2014,
Volume: 28 Issue: 2, 113 - 118, 01.10.2014
Zühre Sarp Taymaz
Aydan Mertoğlu
Emel Tellioğlu
Günseli Balcı
Yasemin Yurt
Zekiye Aydoğdu
Abstract
Wegener granulomatosis (WG) is a rare systemic disease which is characterized with necrotizing granulomatous vasculitis affecting upper and lower respiratory tract and kidneys. It is known that this disease may rarely affect other organ systems. 72-years-old male patient was diagnosed to have “a 4x5 cm diameter irregular contoured malign mass lesion with a large necrotic center localized and invasing adjacent pleura in right upper lobe posterior segment and another 2x2.5 cm diameter irregular contoured nodule localized in subpleural region of right upper lobe anterior segment” in his thorax computerized tomography during preoperative evaluation for benign prostate hypertrophy operation. He was diagnosed to have systemic type WG. Before starting systemic therapy, during the diagnosis period, he had lifethreatening heamoptysis and gastrointestinal bleeding. Endoscopy and colonoscopy were performed. Corticosteroid and cyclophosphamide therapy was initiated as intestinal involvement of WG was considered and had a dramatic response after treatment. He is presented because the gastrointestinal bleeding recovered dramatically by corticosteroid and cyclophosphamide therapy and because gastrointestinal system involvement is a rare entity in WG.
References
- 1. Storesund B,Gran JT, Koldingnes W. Severe
intestinal involvement in Wegener Granulomatosis:
report of two cases and review of
literature. Br J Rheumotol 1998;37:387-390.
- 2. Walton EW. Giant-cell granuloma of the
respiratory tract (Wegener’s granulomatosis). Br
Med J 1958; 2: 265 -270.
- 3. C Pagnoux, A Mahr, P Cohen, L Guillevin, Loic MD,
Presentation and Outcome of Gastrointestinal
Involvement in Systemic Necrotizing Vasculitides:
Analysis of 62 Patients With Polyarteritis Nodosa,
Microscopic Polyangiitis, Wegener Granulomatosis,
Churg-Strauss Syndrome, or Rheumatoid
Arthritis-Associated Vasculitis, Medicine March
2005;Volume 84-Issue 2: 115- 128.
- 4. Deniz K, Özşeker HS, Balas S, Akpınar E,
Sökmensüer C. Intestinal involvement in
Wegener's granulomatosis. J Gastrointestin
Liver Dis. 2007 Sep;16(3):329 -31.
- 5. Haworth SJ, Pusey CD. Severe intestinal
involvement in Wegener’s granulomatosis.Gut
1984;25:1296-1300.
- 6. Beppu K, Osada T, Matsumoto K, Shibuya T,
Sakamoto N, Kawabe M, Nagahara A, Ogihara T,
Watanabe S, Intestinal involvement in Wegener’s
granulomatosis diagnosed and followed up by
double balloon enterescopy, Intern
Med.2011;50(3):219- 222.Epub 2011 Feb 1.
- 7. Nazif Zeybek, Faik Yaylak, Özcan Altinel, Özgur
Albuz, Ayper Kaya, Yusuf Peker, Multiple
Intestinal Perforation Due To Wegener’s
Granulotamosis: Case Report. J.Clin. Anal. Med.
2010; 1(2): 34 -36.
- 8. Tamer Akça, Tahsin Çolak, Mehmet
Çağlıkülekçi, Koray Öcal, Suha Aydın, Intestinal
perforation in Wegener’s granulomatosis: a case
report, Wegener granülomatozunda intestinal
perforasyon: Olgu Sunumu, Turkish Journal of
Trauma & Emergency Surgery Ulus Travma
Derg 2005
- 9. Leavitty RY, Fauci AS, Bloch DA,et al.The American
College of Rheumatology 1990 criteria fort he
classification of Wegener Granulomatosis.Arthritis
Rheum 33:1101-1107,1990
- 10. Fauci AS, Haynes BF, Katz P, Wolff SM.
Wegener’s Granulomatozis Prospective clinical
and therapeutic experience with 85 patients for
21 years Ann Intern Med. 1983; 98: 76- 85.
- 11. Takwoingi YM, Dempster JH. Wegener’s
Granulomatosis: an analysis of 33 patients seen
over a 10 year period. Clin Otolaryngol. 2003;
28: 187- 194.
- 12. Chow FY, Hooke D, Kerr PG. Severe intestinal
involvement inWegener’s granulomatosis. J
Gastroenterol Hepatol 2003; 18:749-750.
- 13. Veinot JP, Logan CA, Thomas MJ. Wegener’s
granulomatosis arteritis causing small bowel
infarction. Pathology 2003; 35:268-269.
- 14. Pulimood AB, Peter S, Ramakrishna BS, et al.
Segmental colonoscopic biopsies in the
differentiation of ileocolic tuberculosis from
Crohn’s disease. J Gastroenterol Hepatol
2005;20: 688-696.
- 15. Ozdemir Abbas, Seyahi Nurhan, Durak
Haydarand Kılıcarslan Isın. Intestinal Perforation
due to Vasculitis in a Patient with Wegener’s
Granulomatosis. BANTAO Journal 2009; 7 (1):
46-48.