2019, Number 3
<< Back Next >>
Rev Cub de Reu 2019; 21 (3)
Wegener's granulomatosis mimicking a tumor of the lung vertex. Report of a clinical case and review of the literature
Reyes LGA, Frías PR, Mateo DP, Basulto QN, Reyes GG, Martínez QJA, Acosta LD, Carrillo LD
Language: Spanish
References: 23
Page: 1-11
PDF size: 307.08 Kb.
ABSTRACT
Granulomatosis with polyangiitis, formerly known as Wegener's Granulomatosis, is classified as systemic vasculitis, of unknown cause, affecting small and medium-sized vessels. It is characterized by the involvement of the respiratory system in its upper tract, lungs, as well as the kidneys, although it can affect other organs and systems. Neutrophil anticithoplasma antibodies are positive with a frequency that reaches over 80% of cases, and the most relevant histological feature is the presence of necrotizing granulomatous lesions. The diagnosis is based on clinical manifestations, biopsy with histological study of the affected tissues and organs as well as the presence of Neutrophil anticithoplasma antibodies. Nowadays it is grouped into the so-called Neutrophil anticithoplasma antibodies positive vasculitis. Arthromyalgia, respiratory manifestations such as thoracic and left intercostal pain, cough with mucous expectoration, and dyspnea on physical efforts in increase. The physical examination collected positive data such as mucous skin pallor, the presence of decreased vesicular murmur at the level of the left vertex of the lung with crackling rales and an image as a rounded tumor mass of the upper lobe of the lung that guided the diagnosis of a vertex neoplasm of lung. Multiple studies were carried out considering the proteiform clinical picture, the immunological studies performed, and the lung biopsy histology, which demonstrated the existence of granulomatous lesions compatible with G. Wegener. The therapeutic response was effective with steroidal and immunosuppressive drugs in the form of cyclophosphamide boluses accompanied by a strong broad-spectrum antibiotic treatment. We conclude that this is a rare case of Wegener's granulomatosis, whose debut form raised the initial diagnosis of a tumor of the lung vertex. We do not know of another similar case reported in our country.
REFERENCES
Simón Hoyos Patiño. Granulomatosis con poliangeítis: actualización y conceptos claves. Revista Cubana de Reumatología. 2016;18(1):36-44.
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Fauci AS. Wegener granulomatosis: an analysis of 158 patients. Ann InternMed. Mar 15. 1992;116(6):488-98.
García Ribes M. 2006-2016: diez años de inmersión en el mundo de las enfermedades raras. Aten Primaria [Internet]. 2016 [citado 25 Dic 2018];48(4):217-18. Disponible en: http://www.elsevier.es/es-revista-atencion-primaria-27-linkresolver-2006-2016-diez-anos-inmersion-el-S0212656716300506
Young P. Granulomatosis con poliangeítis. Medicina (B. Aires) [Internet]. 2014 [citado 25 Dic 2018];74(3). Disponible en: http://www.scielo.org.ar/scielo.php?script=sci_arttext&pid=S0025-76802014000300020
Martínez-Morillo M, Grados D, Naranjo-Hansb D, MateoL, Holgado S, Olivé A. Granulomatosis con poliangeítis (WG9. Descripción de 15casos. Reumatol Clin. [Internet]. 2012[citado 9 Nov 2018];8(1):15-9. Disponible en: www.reumatologiaclinica.org
Lane SE, Watts R, Scott DGI. Epidemiology of systemic vasculitis. Curr Rheumatol Rep. August 2005.7:270-75.
Polychronopoulos VS, Prakash UB, Golbin JM, Edell ES, Specks U. Airway involvement in Wegener's granulomatosis (Review). Rheum Dis Clin North Am. 2007;33(4):755-75.
Valero Roldán J, Nuñez Castillo D, Fernández Fígares C, López-Leiva I. Hemorragia alveolar masiva en la granulomatosis de Wegener. SEMERGEN-Medicina de Familia [Internet]. 2014 May–June [citado 25 Dic 2018];40(4):e81–e85. Disponible en: http://www.elsevier.es/es-revista-medicina-familia-semergen-40-articulo-hemorragia-alveolar-masiva-granulomatosis-wegener-S1138359313000609
Massa M, Emery NC, Bosio M, Finn BC, Bruetman JE, Young P. Estenosis subglótica y granulomatosis con poliangeítis (Wegener) en dos casos. Rev méd Chile. 2014;142(3):382-5.
Zazueta Montiel B, Flores Suárez LF. Ruta y retos diagnósticos en vasculitis primarias.Reumatol Clin. 2011;7(S3):S1–S6.
Walsh M, Merkel PA, Mahr A, Jayne D. Effects of duration of glucocorticoid therapy on relapse rate inantineutrophil cytoplasmic antibody-associated vasculitis: A meta-analysis. Arthritis Care Res (Hoboken). 2010 Aug;62(8):1166-73.
Finkielman JD, Lee AS, Hummel AM, Viss MA, Jacob GL, Homburger HA, Clair EWS. ANCA are detectable in nearly all patients with active severeWegener's Granulomatosis. Am J Med. July 2007. 120;(7):e9-e14.
Boomsma MM, Stegeman CA, van der Leij MJ, et al. Prediction of relapses in Wegener's granulomatosis bymeasurement of antineutrophil cytoplasmic antibody levels: a prospective study. Arthritis Rheum. September2000. 43(9):2025-33.
Kallenberg CGM. Pathogenesis of PR3-ANCA associated vasculitis. J Autoimmun. February-March 2008.30:29-36.
Leavitt R Y, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for theclassification of Wegener's granulomatosis. Arthritis Rheum. August. 1990;33(8):1101-7.
Kallenber CG. Antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. Curr Opin Rheumatol, 2007;(19):17-24.
Boomsma MM, Stegerman CA, Van der Lejj MJ, Oost W, Hermans J, Kallenberg CG, et al. Prediction of relapses in Wegener's granulomatosis by measurement of antineutrophil cytoplasmic antibody levels: a prospective study. Arthritis Rheum. 2000;43:2025-33
Villiger PM, Guillevin L. Microscopic polyangiitis: clinical presentation. Autoimmun Rev. 2010;9:812-9.
Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener's granulomatosis. Ophthalmology. 1983;90:279-90.
Vera-Lastra O. Granulomatosis de Wegener, abordaje diagnóstico y terapéutico. Gac Méd Méx. 2009;146:121-9.
Wolter NE, Ooi EH, Witterick IJ. Intralesional corticosteroid injection and dilatation provides effective management of subglottic stenosis in Wegener's granulomatosis. Laryngoscope. 2010; 120(12):2452-5.
Álvarez Herrera T, Santana Matheu G, Placeres Hernández JF. Enfermedad de Wegener o granulomatosis con poliangeítis. Presentación de un caso Rev. Med. Electrón. 2018;40(3):12-34.
De Groot K, Harper L, Jayne DR, Suarez LFF, Gregorini G, Gross WL, Tesar V. Pulse versus daily oral cyclophosphamide for induction of remissionin antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. May. 2009;150(10):670-80.