Abstract
Most epidemiological studies with Wegener’s granulomatosis (WG) patients are based on populations from the Northern hemisphere, whereas very few studies have been conducted in Southern hemisphere populations, particularly from South America. The authors performed a large retrospective, demographic study including clinical and laboratory profiles of 134 consecutive WG patients seen at one Brazilian center from 1999 to 2009. Mean age at initial WG diagnosis was 43.4 ± 15.5 years, and mean disease duration was 8.6 ± 6.6 years. Sixty-four (47.8%) patients were male and a total of 113 (84.3%) subjects were white. Ear/nose/throat involvement occurred in 85.8%. The classic lung and renal involvement were observed in 77.6% and 75.4%, respectively, followed by ocular (35.8%), musculoskeletal (33.4%), cutaneous (29.1%), neurological (20.1%), cardiac (11.2%), and genitourinary involvement in 2.2% of cases. Cytoplasmic pattern–antineutrophil cytoplasmic antibody was detected in 83 (61.9%) cases. Ten (7.5%) individuals presented limited forms of WG. Classic therapy with corticosteroids and cyclophosphamide was used in 97 cases (72.4%). There were no cases of tuberculosis or Pneumocystis jiroveci pneumonia, but cutaneous herpes zoster occurred in eight (6.0%) individuals. There were 29 deaths (21.6%). Eighteen patients died of septic shock (mainly bacterial pneumonia), whereas four died of alveolar hemorrhage, four of myocardial infarction, and three of other causes. In summary, our data from a very large retrospective and descriptive study mirrored the main clinical features of WG described in other countries, demonstrating that they may serve as a reference for South American populations.
Similar content being viewed by others
References
Hoffman GS, Kerr GS, Leavitt RY et al (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116:488–498
Koldingsnes W, Nossent H (2000) Epidemiology of Wegener’s granulomatosis in northern Norway. Arthritis Rheum 43:2481–2487
Watts RA, Scott DG (1997) Classification and epidemiology of the vasculitides. Baillieres Clin Rheumatol 11:191–217
Watts R, Gonzales-Gay M, Garcia-Porrua C et al (2000) ANCA-associated vasculitis in two European regions. Clin Exp Immunol 120(Suppl 1):60
Gonzalez-Gay MA, Garcia-Porrua C, Guerrero J (2003) The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions. Arthritis Rheum 49:388–393
Reinhold-Keller E, Zeidler A, Mock C et al (1996) Epidemiology of primary systemic vasculitis in North and South Germany [abstract]. Sarcoidosis 13(Suppl 3):272
Bligny D, Mahr A, Toumelin PL et al (2004) Predicting mortality in systemic Wegener’s granulomatosis: a survival analysis based on 93 patients. Arthritis Rheum 51:83–91
Anderson G, Coles ET, Crane M et al (1992) 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. Q J Med 83:427–438
Reinhold-Keller E, Beuge N, Latza U et al (2000) An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 43:1021–1032
Jennings CR, Jones NS, Dugar J et al (1998) Wegener’s granulomatosis—a review of diagnosis and treatment in 53 subjects. Rhinology 36:188–191
Fauci AS, Woff SM (1994) Wegener’s granulomatosis: studies in eighteen patients and a review of the literature. Medicine [Baltimore] 73:315–324
Matteson EL, Gold KN, Bloch DA (1996) Long-term survival of patients with Wegener’s granulomatosis from the American College of Rheumatology Wegener’s granulomatosis classification criteria cohort. Am J Med 101:129–134
Westman KW, Bygren PG, Olsson H et al (1998) Relapse rate, renal survival and cancer morbidity in patients with Wegener’s granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol 9:842–852
Gibson A, Stamp LK, Chapman PT, O’Donnell JL (2006) The epidemiology of Wegener’s granulomatosis and microscopic polyangiitis in a Southern Hemisphere region. Rheumatology 45:624–628
Cisternas ML, Soto L, Jacobelli S et al (2005) Características clínicas de granulomatosis de Wegener y poliangeítis microscópica em pacientes chilenos. Rev Med Chil 133:273–278
Gamron S, Eugenia Muscellini M, Onetti L et al (2006) Wegener's granulomatosis: its prevalence in a ten-year period in the rheumatology service of the Clinic Hospital, Cordoba, Argentina. Rev Fac Cien Med Univ Nasc Cordoba 63:53–56
Leavitt RY, Fauci AS, Bloch DA et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101–1107
The WGET Research Group (2002) Design of the Wegener’s Granulomatosis Etanercept Trial (WGET). Control Clin Trials 23:450–468
Exley AR, Bacon PA, Luqmani RA et al (1998) Examination of disease severity in systemic vasculitis from the novel perspective of damage using the vasculitis damage index (VDI). Br J Rheumatol 37:57–63
Seo P, Min YI, Holbrook JT et al (2005) Damage caused by Wegener's granulomatosis and its treatment: prospective data from the Wegener's Granulomatosis Etanercept Trial (WGET). Arthritis Rheum 52:2168–2178
Bajema IM, Hagen EC, van der Woude FJ et al (1997) Wegener's granulomatosis: a meta-analysis of 349 literary case reports. J Lab Clin Med 129:17–22
Faurschou M, Mellemkjaer L, Sorensen IJ, Thomsen BS, Dreyer L, Baslund B (2009) Increased morbidity from ischemic heart disease in patients with Wegener's granulomatosis. Arthritis Rheum 60:1187–1189
Merkel PA, Lo GH, Holbrook JT et al (2005) Brief communication: high incidence of venous thrombotic events among patients with Wegener granulomatosis: The Wegener’s Clinical Occurrence of Thrombosis (WeCLOT) study. Ann Intern Med 142:620–626
Allenbach Y, Seror R, Pagnox C et al (2009) High frequency of venous thromboembolic events in Churg–Strauss syndrome, Wegener's granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients. Ann Rheum Dis 68:564–567
Aasarod K, Inversen BM, Hammerstrom J et al (2000) Wegener's granulomatosis: clinical course in 108 patients with renal involvement. Nephrol Dial Transplant 15:611–618
Krafcik SS, Covin RB, Lynch JP et al (2006) Wegener’s granulomatosis in the elderly. Chest 109:430–437
Le Thi Huong DU, Wechsler B, Cabane J et al (1988) Wegener’s granulomatosis: clinical aspects, nosologic problems: review of the literature a propos of 30 cases. Ann Med Interne 139:169–182
Vassallo M, Shepherd RJ, Iqbal P et al (1997) Age-related variations in presentation and outcome in Wegener’s granulomatosis. J R Coll Physicians 31:396–400
Fauchais AL, Michon-Pasturel M, Rugale C et al (2001) Wegener’s granulomatosis in the elderly patient [in French]. Rev Med Interne 22:127–131
Romas E, Murphy BF, d’Apice Aj et al (1993) Wegener’s granulomatosis: clinical features and prognosis in 37 patients. Aust NZ J Med 23:168–175
Zycinska K, Wardyn KA, Tyszko P et al (2007) Analysis of early death based on the prediction model in Wegener's granulomatosis with pulmonary and renal involvement. J Physiol Pharmacol 58(Suppl 5):829–837
Jarrousse B, Guillevin L, Bindi P et al (1993) Increased risk of Pneumocystis carinii pneumonia in patients with Wegener's granulomatosis. Clin Exp Rheumatol 11:615–621
Godeau B, Coutant-Perronne V, Du Huong LT et al (1994) Pneumocystis carinii pneumonia in the course of connective tissue disease: report of 34 cases. J Rheumatol 21:246–251
Godeau B, Mainardi JL, Roudot-Thoraval F et al (1995) Factors associated with Pneumocystis carinii pneumonia in Wegener's granulomatosis. Ann Rheum Dis 54:991–994
Gatenby PA, Lucas RM, Engelsen O et al (2009) Antineutrophil cytoplasmic antibody-associated vasculitides: could geographic patterns be explained by ambient ultraviolet radiation? Arthritis Rheum 61:1417–1424
Disclosures
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
de Souza, F.H.C., Radu Halpern, A.S., Valente Barbas, C.S. et al. Wegener’s granulomatosis: experience from a Brazilian tertiary center. Clin Rheumatol 29, 855–860 (2010). https://doi.org/10.1007/s10067-010-1408-4
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-010-1408-4