Letter to the Editor
Red Eye as the Primary Manifestation of Wegener's Granulomatosis
Ojo rojo como manifestación primaria de vasculitis sistémica
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"cabecera" => "<span class="elsevierStyleTextfn">Letter to the Editor</span>" "titulo" => "Red Eye as the Primary Manifestation of Wegener's Granulomatosis" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "193" "paginaFinal" => "194" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Ruth López-González, Olga Martínez-González, Miguel Martín-Luquero Ibáñez, Juan Pablo Valdazo de Diego" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Ruth" "apellidos" => "López-González" "email" => array:1 [ 0 => "lopezgonzalezruth@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Olga" "apellidos" => "Martínez-González" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Miguel" "apellidos" => "Martín-Luquero Ibáñez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Juan Pablo" "apellidos" => "Valdazo de Diego" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Reumatología, Complejo Hospitalario de Zamora, Zamora, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Medicina Interna, Complejo Hospitalario de Zamora, Zamora, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Ojo rojo como manifestación primaria de vasculitis sistémica" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1000 "Ancho" => 831 "Tamanyo" => 82374 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Cerebral magnetic resonance: increase in the size of the superior and lateral right orbit, compatible with an infiltrating process.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We are writing to present the atypical case of a rare first manifestation of granulomatosis with polyangiitis.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The patient is a 74-year-old woman who came to our centers’ Emergency department due to 4 days with persistent headache associated to a red right eye and ptosis of the same side. The process had an abrupt onset and was not associated to fever or trauma, persisting over time and not improving with rest. Upon examination the patient had no cardiopulmonary, abdominal, locomotor, central or peripheral nervous system alterations; just the right red eye and ptosis were found. In the urgent laboratory tests requested we only found a CRP of 78<span class="elsevierStyleHsp" style=""></span>mg/l and ESR of 109<span class="elsevierStyleHsp" style=""></span>mm/h, with the rest of the serology within normal limits.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Eye examination showed unilateral proptosis of the right eye, red eye with a negative Tyndall effect, absence of cells in the anterior or posterior chamber, without retinal lesions. The patient had a 0.6 visual acuity of the right eye and of 1 on the left eye.</p><p id="par0020" class="elsevierStylePara elsevierViewall">A cerebral magnetic resonance was performed showing an increase in the size of the right lacrimal grand with supracilliar and lateral and superior orbital affection of the right eye, compatible with dacryocystitis/infiltrative process (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>, coronal plane), leading us to the diagnosis of orbital pseudotumor with muscle affection and dacryocystitis, beginning treatment with high dose steroids and intravenous antibiotics, due to the severity of the process. A biopsy of the swollen area was performed but the result was non significant and response to medication was tapered, due to the severity of the eye manifestations.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">We only found positivity to PR3 45<span class="elsevierStyleHsp" style=""></span>U/ml (normal<span class="elsevierStyleHsp" style=""></span><3), with infectious serology (HIV, Syphillis, <span class="elsevierStyleItalic">Brucella</span>, hepatitis B and C, <span class="elsevierStyleItalic">Toxoplasma</span>), autoimmunity (RF, ANA, anti-DNA negative; ANCA: MPO negative), thyroid hormones and tumor markers negative. The chest X-ray did show a nodular pattern in the lower right lobe.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Two weeks later the patient presented non specific muscle and joint pain of the upper limbs, a single ulcer on the base of the tongue and fever. We performed a thoracic computed tomography which revealed several symmetric and random nodules, mainly on the right lung base, measuring 5 and 15<span class="elsevierStyleHsp" style=""></span>mm, some of them cavitated, possibly related to granulomatosis. The lateral segment of the middle right lobe showed a condensation possibly related to the same process.</p><p id="par0035" class="elsevierStylePara elsevierViewall">A transbronchial lung biopsy was performed showing a granulomatous vasculitic process, with segmental vascular necrosis and neutrophil and monocyte infiltration, making us reach a conclusion of granulomatosis with polyangiitis.</p><p id="par0040" class="elsevierStylePara elsevierViewall">With respect to later treatment, once the diagnosed was reached we began treatment with: (a) pulse steroid 1.000<span class="elsevierStyleHsp" style=""></span>mg for three doses, continuing with oral prednisone 1<span class="elsevierStyleHsp" style=""></span>mg/kg for 2–4<span class="elsevierStyleHsp" style=""></span>weeks and reducing to 20<span class="elsevierStyleHsp" style=""></span>mg/day at the end of the second month, with a progressive downward adjustment for 6 months, (b) associated with cyclophosphamide boluses for 4 weeks intravenously, and (c) trimetoprim-sulphamethoxazole, orally, 3/week for <span class="elsevierStyleItalic">Pneumocystis carinii</span> prophylaxis.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Granulomatosis with polyangiitis is a systemic vasculitis of unknown cause that affects small and medium caliber vessels. It is characterized by respiratory system and kidney affection but may affect other organs. ANCA are frequently positive and the most important histologic characteristic is the presence of necrotizing granulomas.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Their incidence in Spain reaches 2.95 cases/million inhabitants for granulomatosis with polyangiitis, 7.91 cases/million for microscopic polyarteritis and 1.31 cases/million for Churg-Strauss’ disease.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Throughout the disease process, the incidence of ophthalmic manifestations described in this type of vasculitis<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> goes from 20 to 50% of cases. Per region, the incidence is: conjunctivitis, keratitis (12%–20%); episcleritis and scleritis (12%–27%); uveitis (2%–7%); optic neuritis (12%–16%), and proptosis (15%–57%), but being very atypical as a presenting symptom.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Eye affection is a primordial manifestation of granulomatosis with polyangiitis and it distinguishes it from other systemic or ANCA positive vasculitidies.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6,7</span></a> The most common initial manifestation is proptosis, but as in this patients case, the presence of diplopia and loss of visual acuity have also been previously described.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The differential diagnosis of orbital affection in a patient with granulomatosis with polyangiitis must include infections, fungal or bacterial, orbital lymphoma, sarcoidosis, Graves disease and orbital pseudotumor.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Treatment, based on adequate immunosuppression with steroids associated to cyclophosphamide, has led to the control of the disease in a high percentage of patients, although it is true that the loss of visual acuity is in many cases irreversible and requires a strict control by Ophthalmology as well as Rheumatology and primary care.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: López-González R, Martínez-González O, Martín-Luquero Ibáñez M, Valdazo de Diego JP. Ojo rojo como manifestación primaria de vasculitis sistémica. Reumatol Clin. 2014;10:193–194.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1000 "Ancho" => 831 "Tamanyo" => 82374 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Cerebral magnetic resonance: increase in the size of the superior and lateral right orbit, compatible with an infiltrating process.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Wegener's Granulomatosis vasculitis and granuloma" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "A.M. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 6 | 4 | 10 |
| 2024 October | 47 | 30 | 77 |
| 2024 September | 46 | 23 | 69 |
| 2024 August | 74 | 36 | 110 |
| 2024 July | 63 | 32 | 95 |
| 2024 June | 72 | 38 | 110 |
| 2024 May | 62 | 32 | 94 |
| 2024 April | 60 | 22 | 82 |
| 2024 March | 61 | 40 | 101 |
| 2024 February | 50 | 26 | 76 |
| 2024 January | 38 | 24 | 62 |
| 2023 December | 44 | 23 | 67 |
| 2023 November | 57 | 27 | 84 |
| 2023 October | 50 | 883 | 933 |
| 2023 September | 56 | 29 | 85 |
| 2023 August | 54 | 17 | 71 |
| 2023 July | 40 | 28 | 68 |
| 2023 June | 56 | 24 | 80 |
| 2023 May | 57 | 22 | 79 |
| 2023 April | 29 | 9 | 38 |
| 2023 March | 75 | 29 | 104 |
| 2023 February | 54 | 25 | 79 |
| 2023 January | 40 | 23 | 63 |
| 2022 December | 47 | 24 | 71 |
| 2022 November | 66 | 28 | 94 |
| 2022 October | 47 | 38 | 85 |
| 2022 September | 44 | 40 | 84 |
| 2022 August | 46 | 40 | 86 |
| 2022 July | 47 | 40 | 87 |
| 2022 June | 43 | 40 | 83 |
| 2022 May | 51 | 40 | 91 |
| 2022 April | 52 | 44 | 96 |
| 2022 March | 77 | 49 | 126 |
| 2022 February | 88 | 30 | 118 |
| 2022 January | 83 | 32 | 115 |
| 2021 December | 42 | 37 | 79 |
| 2021 November | 57 | 50 | 107 |
| 2021 October | 63 | 41 | 104 |
| 2021 September | 57 | 35 | 92 |
| 2021 August | 38 | 42 | 80 |
| 2021 July | 43 | 27 | 70 |
| 2021 June | 50 | 42 | 92 |
| 2021 May | 54 | 32 | 86 |
| 2021 April | 144 | 57 | 201 |
| 2021 March | 48 | 21 | 69 |
| 2021 February | 30 | 22 | 52 |
| 2021 January | 40 | 12 | 52 |
| 2020 December | 34 | 11 | 45 |
| 2020 November | 40 | 18 | 58 |
| 2020 October | 29 | 18 | 47 |
| 2020 September | 37 | 21 | 58 |
| 2020 August | 29 | 21 | 50 |
| 2020 July | 25 | 13 | 38 |
| 2020 June | 28 | 23 | 51 |
| 2020 May | 28 | 11 | 39 |
| 2020 April | 30 | 18 | 48 |
| 2020 March | 17 | 6 | 23 |
| 2020 February | 3 | 0 | 3 |
| 2018 May | 3 | 1 | 4 |
| 2018 April | 26 | 10 | 36 |
| 2018 March | 39 | 6 | 45 |
| 2018 February | 30 | 5 | 35 |
| 2018 January | 18 | 3 | 21 |
| 2017 December | 27 | 4 | 31 |
| 2017 November | 29 | 6 | 35 |
| 2017 October | 16 | 3 | 19 |
| 2017 September | 29 | 10 | 39 |
| 2017 August | 37 | 8 | 45 |
| 2017 July | 37 | 7 | 44 |
| 2017 June | 74 | 9 | 83 |
| 2017 May | 72 | 18 | 90 |
| 2017 April | 44 | 6 | 50 |
| 2017 March | 45 | 2 | 47 |
| 2017 February | 18 | 2 | 20 |
| 2017 January | 25 | 7 | 32 |
| 2016 December | 57 | 13 | 70 |
| 2016 November | 49 | 7 | 56 |
| 2016 October | 58 | 10 | 68 |
| 2016 September | 49 | 8 | 57 |
| 2016 August | 38 | 5 | 43 |
| 2016 July | 25 | 6 | 31 |
| 2015 December | 2 | 0 | 2 |
| 2015 September | 2 | 0 | 2 |
| 2015 August | 2 | 0 | 2 |
| 2015 July | 9 | 7 | 16 |
| 2015 June | 34 | 10 | 44 |
| 2015 May | 46 | 12 | 58 |
| 2015 April | 73 | 8 | 81 |
| 2015 March | 65 | 4 | 69 |
| 2015 February | 62 | 3 | 65 |
| 2015 January | 61 | 10 | 71 |
| 2014 December | 36 | 9 | 45 |
| 2014 November | 26 | 13 | 39 |
| 2014 October | 42 | 11 | 53 |
| 2014 September | 25 | 8 | 33 |
| 2014 August | 31 | 19 | 50 |
| 2014 July | 32 | 13 | 45 |
| 2014 June | 59 | 18 | 77 |
| 2014 May | 68 | 18 | 86 |
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