Journals / Archives of Basic and Clinical Research / 2021 / Cilt: 3 - Sayı: 1

A Case of Familial Wegener Granulomatosis Presented withLife-Threatening Diffuse Alveolar Hemorrhage and TreatedSuccessfully

Hayatı Tehdit Eden Diffüz Alveoler Hemoraji ile Prezente Olan ve Başarılı Bir Şekilde Tedavi Edilen Ailesel Wegener Granülomatozis Olgusu

Pages
38–42
Publication Date
2022-10-12
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Here, we report the case of a young male patient who underwent treatment for tuberculosis because of the appearance of a miliary involvement upon lung imaging at a previous medical center and present the fast progression of the disease, good response from the treatment, and the loss of his father from the same disease.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Granulomatosis with polyangiitis is a systematic disease characterized by necrotizing granulomatous vasculitis, which can involve the upper respiratory tract, lungs and kidneys.
📊
Key Findings & Data:The development of cavitary lesions owing to their necrotizing feature also requires diagnosis with many diseases, especially tuberculosis and malignancies.
💡
Conclusion & Impact:Here, we report the case of a young male patient who underwent treatment for tuberculosis because of the appearance of a miliary involvement upon lung imaging at a previous medical center and present the fast progression of the disease, good response from the treatment, and the loss of his father from the same disease.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Granulomatosis with polyangiitis is a systematic disease characterized by necrotizing granulomatous vasculitis, which can involve the upper respiratory tract, lungs and kidneys. The development of cavitary lesions owing to their necrotizing feature also requires diagnosis with many diseases, especially tuberculosis and malignancies. Although the lungs are frequently involved in Wegener granulomatosis, diffuse alveolar hemorrhage is rarely observed and it progresses mortally. Additionally, the reported number of cases of familial Wegener granulomatosis is very low. Here, we report the case of a young male patient who underwent treatment for tuberculosis because of the appearance of a miliary involvement upon lung imaging at a previous medical center and present the fast progression of the disease, good response from the treatment, and the loss of his father from the same disease.

Özet

Polianjitis ilişkili granülomatozis; daha çok üst solunum yolları, akciğerler ve böbrekleri tutabilen nekrotizan granülomatöz vaskülit olarak tanımlanan sistemik bir hastalıktır. Nekrotizan olması sebebiyle kaviter lezyonların gelişmesi, başta tüberküloz ve malignitelerle beraber pek çok hastalıkla ayırıcı tanı gerektirir. Wegener Granulomatoz’unda akciğerler sıklıkla tutulur. Ancak diffuz alveoler hemoroji nadir olarak görülmekte ve çoğunlukla ölümcül olarak seyretmektedir. Ayrıca bildirilen ailesel WG olgu sayısı oldukça azdır. Önceki başvurduğu sağlık merkezinde akciğer görüntülemesinde miliyer tutulum benzeri görünüm olması ve enfeksiyon tablosu sebebi ile tüberküloz tedavisi başlanan genç erkek olgumuzu; hastalığının hızlı progresyon göstermesine rağmen, tedaviden iyi yanıt alınması ve babasının da aynı hastalık sebebi ile kaybedilmesi nedeniyle sunmayı uygun bulduk.

🔖

Cite This Article

TOSUN, M., TEKE, T. (2022). A Case of Familial Wegener Granulomatosis Presented withLife-Threatening Diffuse Alveolar Hemorrhage and TreatedSuccessfully. Archives of Basic and Clinical Research, Cilt: 3 - Sayı: 1, 38–42. https://ajindex.com/articles/447776

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (1)

  1. Hoffman GS, Kerr GS, Leavit RY, Leavitt RY, Hallahan CW, Lebovics RS, et al. Wegener granulomatosis: An analysis of 158 patients. Ann Intern Med 1992; 116: 488-98. [Crossref] 2. Frankel SK, Sullivan EJ, Brown KK. Vasculitis: Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa, and Takayasu arteritis. Crit Care Clin 2002; 18: 855-79. [Crossref] 3. Gómez-Gómez A, Martínez-Martínez MU, Cuevas-Orta E, Bernal-Blanco JM, Cervantes-Ramírez D, Martínez-Martínez R, et al. Pulmonary manifestations of granulomatosis with polyangiitis. Reumatol Clin 2014; 10: 288-93. [Crossref] 4. Yapıcıoğlu S, Erer OF, Yalçın YA, Özkan SA. Wegener granülomatozu: tanısal zorluk yaşanan bir olgu nedeniyle Türkiye’den bildirilen olguların literatür eşliğinde incelenmesi. İzmir Göğüs Hastalıkları Dergisi 2004; 1: 33-40. 5. Savage COS, Harper L, Adu D. Primary systemic vasculitis. Lancet 1997; 349: 553-8. [Crossref] 6. Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical Pathology of the Lung in Wegeners Granulomatosis - Review of 87 Open Lung Biopsies from 67 Patients. Am J Surg Pathol 1991; 15: 315-33.[Crossref] 7. Lie JT. Diagnostic Histopathology of Major Systemic and Pulmonary Vasculitic Syndromes. Rheum Dis Clin North Am 1990; 16: 269-92. 8. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337: 1512-23. [Crossref] 9. Prendecki M, Cairns T, Pusey CD. Familial vasculitides: granulomatosis with polyangitis and microscopic polyangitis in two brothers with differing anti-neutrophil cytoplasm antibody specificity. Clin Kidney J 2016; 9: 429-31. [Crossref] 10. Sewell RF, Hamilton DV. Time associated Wegener’s granulomatosis in two members of a family. Nephrol Dial Transplant 1992; 7: 882. 11. Fraser RS, Müller NL, Colman N, Pare PD. Diagnosis of diseases of the chest. Saunder Company USA 1999; 1489-532. 12. Sneller MC. Wegener Granulomatosis. JAMA 1995; 273: 1288-91. [Crossref] 13. Bamberty P, Sakhuja V, Bhusnurmath SR, Jindal SK, Deodhar SD, Chugh KS. Wegener’s Granulomatosis: Clinical experi ence with eighteen patients. J Assoc Physicians India 1992: 40: 597-600. 14. Gökhan P, Erhan T, Ömer NP. Massif Alveoler Hemorojiyle Seyreden Wegener Granulomatozu; Olgu Sunumu. Balkan Med J 2011; 28: 463-5. 15. Yurt S, Koşar F. Alveoler hemoroji sendromları. Solunum 2008; 10: 86-91. 16. Çetinkaya E, Sökücü SN, Kadakal F, Altın S, Günlüoğlu G, Ürer N. c-ANCA negatif Wegener Granülomatozu iki vaka sunumu. Solunum 2009; 1