Montesino ÁI, Galiano LM, Falcón LOS, Díaz LLD, Seijas RI, Collazo MY, Rodríguez CN, Sánchez OJG, Suárez PR, Pérez GI

Language: Spanish
References: 8
Page:
PDF size: 345.83 Kb.

ABSTRACT

Wegener's granulomatosis (WG) is a collagen disease, potentially lethal, it is a systemic necrotizing granulomatous vasculitis that primarily affects the respiratory tract and kidneys , it is uncommon or rare, idiopathic with a short life expectancy, without specific treatments, with Multisystemic affectations, generally a consequence of ischemia due to vascular occlusion, including ophthalmological ones, can affect almost all ocular structures..The clinical picture of a 66-year-old female patient with a history of Wegener's granulomatosis diagnosed in the CIMEQ 28 years ago with multiple manifestations was described, previous surgeries of the perinasal sinuses, breast, lung and orbit, habitual treatment Prednisone, Cyclophosphamide and Azulfidine, reaches consultation of the CIMEQ Ophthalmology Guard Corps in March 2011, reporting intense pain in the left eye. The ophthalmological examination revealed a conjunctival scleral necrosis of the entire inferior nasal bulbar area with exposure of the uveal tissue, which progressed rapidly with extensive peripheral corneal thinning. Initially, complementary tests were indicated, 3 coatings with amniotic membrane and tarsorrhaphy were performed without achieving satisfactory results, vision and eye perforation becomes imminent, loss was evaluated with various specialists, then a fistula appears that exposes material from implants from orbital surgery, He performed the explant, subsequently enucleation with a rotated temporal graft with rehabilitation (ocular prosthesis), the medical treatment and the evaluation by internal medicine has been stable Prednisone, Cyclophosphamide and Azulfidine, good vision in his only eye, although dacryocystitis has appeared for 3 years Right chronic mild, opting for conservative treatments, currently maintains complementary in normal ranges, well-tolerated outpatient medical treatment with oral Prednisone and Azulfidine, topical with artificial tears and timolol 0.5% and good quality of life.

REFERENCES

1. Perry SR, Rootman J, White VA. The clinical and pathologic constellation of Wegener granulomatosis of the orbit. Ophthalmology 1997; 104: 683-694.

2. Molinari L, Javier I. Melamud2, Luciana Ferrari2, Pablo Landi2, Guillermo Semeniuk1, Silvia A. Quadrelli1. Granulomatosis de Wegener y tuberculosis. Una mala combinación; Medicina (B. Aires) v.69 n.6 Ciudad Autónoma de Buenos Aires nov./dic. 2009 versión On-line ISSN 1669-9106.

3. Cairoli E, Silvariño R, Méndez E. Granulomatosis de Wegener: clínica, diagnóstico y tratamiento a propósito de cinco casos. Rev. Méd. Urug. v.24 n.1 Montevideo mar. 2008. versión impresa ISSN 0303-3295

4. Jennette JC, Falk RJ. Small vessel vasculitis. N Engl J Med 1997; 337(21): 1512-23.

5. Bosch X, Guilabert A, Font J. Antineutrophil cytoplasmic antibodies. Lancet 2006; 368(9533): 404-18.

6. Alvarez de Mon M. Granulomatosis de Wegener. En: Farreras Valentí P, Rozman C. Medicina Interna. 13° ed. España-Madrid: Mosby/Doyma libros, 1995:vol.1:1112-1113.

7. Ladrón de Guevara D, Cedra F, Carreño MA, Piottante A, Bitar P. Actualización en el estudio de Granulomatosis con poliangeitis (Granulomatosis de Wegener). Rev. Chil Radiol 2019; 25(1): 26-34.

8. Ramírez E, Blas OA, Carreño – Gayoso EA. Granulomatosis con poliangeitis: una perspectiva dermatológica. Reporte de un caso. Dermatología CMQ 2021; 19(1): 43 – 6.