Upper Respiratory Tract Symptoms, Renal Involvement and Vasculitis: A Case Report and Review of Wegener Granulomatosis

Wegener’s Granulomatosis is a condition associated with systemic vasculitis which can present with upper respiratory tract symptoms initially. On September 2001, a 15-year-old girl presented with symptoms of nasal block for 3 weeks. She later developed joint pains and worsening renal status requiring dialysis. A renal biopsy was performed which showed pauci-immune cresentric glomerulonephritis. Her cANCA levels were positive. She was treated with oral cyclophosphamide and steroids and later responded. Keywords Wegener granulomatosis; Young girl; Cyclophophamide; cANCA

[1]  Richard W. Lee,et al.  Novel Therapies for Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , 2012, Drugs.

[2]  U. Demkow,et al.  Chronic crusting, nasal carriage of Staphylococcus aureus and relapse rate in pulmonary Wegener's granulomatosis. , 2008, Journal of physiology and pharmacology : an official journal of the Polish Physiological Society.

[3]  E. Jwarah,et al.  Wegener's granulomatosis in pregnancy , 2006, Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology.

[4]  P. Lamprecht,et al.  Maintenance of remission with leflunomide in Wegener's granulomatosis. , 2003, Rheumatology.

[5]  R. Waldherr,et al.  Expression of cytokines and growth factors in human glomerulonephritides , 1993, Pediatric Nephrology.

[6]  C. Savage,et al.  Mechanisms of endothelial cell injury in vasculitis , 2004, Springer Seminars in Immunopathology.

[7]  J. Stone,et al.  Etanercept combined with conventional treatment in Wegener's granulomatosis: a six-month open-label trial to evaluate safety. , 2001, Arthritis and rheumatism.

[8]  L. Bostad,et al.  Renal histopathology and clinical course in 94 patients with Wegener's granulomatosis. , 2001, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[9]  C. Kallenberg,et al.  Novel therapies for anti-neutrophil cytoplasmic antibody-associated vasculitis , 2001, Current opinion in nephrology and hypertension.

[10]  P. Limburg,et al.  Prediction of relapses in Wegener's granulomatosis by measurement of antineutrophil cytoplasmic antibody levels: a prospective study. , 2000, Arthritis and rheumatism.

[11]  C. Langford,et al.  Use of methotrexate and glucocorticoids in the treatment of Wegener's granulomatosis. Long-term renal outcome in patients with glomerulonephritis. , 2000, Arthritis and rheumatism.

[12]  C. Langford,et al.  A staged approach to the treatment of Wegener's granulomatosis: induction of remission with glucocorticoids and daily cyclophosphamide switching to methotrexate for remission maintenance. , 1999, Arthritis and rheumatism.

[13]  C. Kallenberg,et al.  Monocyte activation in patients with Wegener’s granulomatosis , 1999, Annals of the rheumatic diseases.

[14]  M. Haubitz,et al.  Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmic antibody-associated vasculitis and renal involvement: a prospective, randomized study. , 1998, Arthritis and rheumatism.

[15]  B. Hoen,et al.  A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. , 1997, Arthritis and rheumatism.

[16]  R. Kimberly,et al.  Anti-neutrophil cytoplasmic antibodies engage and activate human neutrophils via Fc gamma RIIa. , 1994, Journal of immunology.

[17]  A. Borne,et al.  Relevance of classic anti‐neutrophil cytoplasmic autoantibody (C‐ANCA)‐mediated inhibition of proteinase 3‐α1‐antitrypsin complexation to disease activity in Wegener's granulomatosis , 1993, Clinical and experimental immunology.

[18]  A. Rees,et al.  HLA class II specificities in vasculitis with antibodies to neutrophil cytoplasmic antigens. , 1992, Kidney international.

[19]  F. Ognibene,et al.  Bronchoalveolar lavage analysis in Wegener's granulomatosis. A method to study disease pathogenesis. , 1991, The American review of respiratory disease.

[20]  V. A. Lisovskii,et al.  [Wegener's granulomatosis]. , 1972, Klinicheskaia meditsina.