Optic neuritis in a teenage girl with granulomatosis with polyangiitis (original) (raw)
Related papers
Arquivos Brasileiros de Oftalmologia, 2016
INTRODUCTION Granulomatosis with polyangiitis (Wegener's) predominantly affects the upper and lower respiratory tracts, lungs, and kidneys and represents a rare multisystem necrotizing granulomatous vasculitis of small-sized vessels. Central nervous system, cutaneous, heart, gas trointestinal tract, orbital, and ocular involvement may also be observed (1). Ocular or orbital involvement in granulomatosis with po lyangiitis is reportedly occurs in 20-50% of patients (2,3). Bilateral acute angle-closure (AAC) glaucoma is a very rare clinical entity that is generally caused by the use of various topical or systemic medications, many of which are known to cause pupillary dilatation, emotional stress, dim illumination, surgical anesthesia, viral infections, systemic vasculitis, and subarachnoid hemorrhage (4-7). To our knowledge, this is the first reported case of granulomatosis with polyangiitis presenting with bilateral acute angle-closure glaucoma. CASE REPORT A 59-year-old Caucasian man presented to a local ophthalmologist with acute bilateral onset of ocular pain, blurred vision, and a severe headache. He was diagnosed with bilateral AAC glaucoma and ABSTRACT We report a case of bilateral acute angle-closure glaucoma in a patient with undiagnosed granulomatosis with polyangiitis (Wegener's). A 59-year-old man presented with a severe headache, ocular pain, blurred vision, shortness of breath, and mild fever. Clinical examination revealed conjunctival chemosis, corneal edema, and shallow anterior chambers. Closed angles were observed bilaterally on gonioscopy. The patient was treated with intravenous mannitol, oral acetazolamide, and anti-glaucomatous eye drops. Over the following two days, his vision improved and intraocular pressures decreased. Subsequently, laser iridotomies were performed bilaterally and the patient attended consultations with our departments of respiratory medicine, nephrology, and rheumatology and was subsequently diagnosed with granulomatosis with polyangiitis. Bilateral acute angle-closure glaucoma is a very rare ocular manifestation of granulomatosis with polyangiitis. The association of this clinical entity with Wegener's granulomatosis remains unknown.
Diagnostics
Granulomatosis with polyangiitis (GPA) is a systemic autoimmune disease consisting of necrotizing granulomatosis of the respiratory tract, necrotizing vasculitis, and necrotizing glomerulonephritis. It is under the category of ANCA-associated vasculitis, which involves small vessels. The nose, sinus, and ear were the most affected sites besides lung and kidney in localized form. They might precede other disease manifestations before progressing to the systemic form. Our patient presented with an intractable headache, followed by acute vision loss. His symptoms deteriorated regardless of antibiotic treatment for paranasal sinusitis. The sequential CT/MRI images showed the inflammatory raid of the orbital apex and cavernous sinus within days. The sinus biopsy and elevated PR3-anti-neutrophil cytoplasmic antibody led us to the diagnosis of GPA. Fortunately, the patient’s vision improved gradually after steroid and immunosuppressant treatment.
International journal of clinical and experimental pathology, 2012
The limited form of Granulomatosis with Polyangiitis (GPA), formerly known as Wegener's Granulomatosis (WG) primarily involves the head and neck region, including the orbit, but is often a diagnostic challenge, particularly as it commonly lacks positive anti-neutrophil cytoplasm antibody (ANCA) titres or classical features on diagnostic orbital biopsies. The purpose of this study was to relate biopsy findings with clinical outcome and to determine which histopathological features are predictive of a clinical diagnosis of GPA. Retrospective case series of 234 patients identified from the database of the UCL Institute of Ophthalmology Department of Eye Pathology as having had orbital biopsies of orbital inflammatory disorders performed between 1988 and 2009. Clinical records were obtained for the patients and analysed to see whether patients had GPA or not, according to a standard set of diagnostic criteria (excluding any histopathological findings). Biopsy features were then corr...
Ocular involvement in granulomatosis with polyangiitis: A single-center cohort study on 63 patients
Autoimmunity Reviews, 2019
Among 308 patients with a new diagnosis of GPA in accordance with the American College of Rheumatology classification criteria and/or revised Chapel Hill nomenclature definitions, we identified those with ocular involvement and a subsequent follow up in our center. Results: The prevalence of ocular involvement in our GPA series was 38.6%; 63 patients were analysed with a median follow-up of 50.5 months (IQR: 17.8-82.8). Scleritis (18 patients, 26.8%) and episcleritis (18 patients, 26.8%) were the most common ophthalmologic manifestations, followed by orbital disease (13 patients, 20.6%). Bilateral involvement and visual acuity loss was seen in 29.1% and 16.7% of patients, respectively. Ocular involvement was the first GPA manifestation in 9 patients (14.3%), concomitant with systemic manifestation in 36 (57.1%), and occurred only during follow-up in 18 (28.6%). The indication for GPA treatment was suggested by ocular involvement in 12 patients (19.4%), by systemic features in 40 (64.5%) and by both ocular and systemic involvement in 10 (16.1%). Remission of ocular involvement was achieved in 51 patients (80.9%). In the remaining 12 (19.1%), symptoms persisted or even worsened, finally leading to rituximab (RTX) therapy in 8 of them (66.7%). Altogether, when used as first line or for refractory disease, ocular remission was achieved in 11 of the 12 cases (91.7%) treated with RTX versus 34 of the 47 cases (72.3%) treated with CYC (P = .260). Eye disease relapsed in 14 patients (22.2%). RTX allowed achievement of remission in 8 of them (57.1%). In the remaining six, other immunosuppressive drugs were used. Conclusions: Scleritis and episcleritis are the most common ocular manifestations in GPA, most of the time associated with other systemic manifestations. In > 40% of cases, ocular manifestations were refractory to initial treatment or recurrent and led to RTX prescription, which appeared to be useful in these situations.
Optic neuritis as ocular manifestation of Wegener’s granulomatosis — case report
Ophthalmology Journal, 2016
Wegener's granulomatosis (WG) is a systemic, autoimmune, granulomatous necrotising inflammation of unknown aetiology. WG is described by the triad, which includes: rhinitis, and pulmonary and kidney dysfunction. The ocular signs are inflammatory orbital disease, nasolacrimal ducts obstruction, conjunctivitis, episcleritis, scleritis uveitis, retinal vasculitis, and optic neuritis. This article describes the case of a patient with optic neuritis associated with WG.
Reumatologia/Rheumatology
Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is a vasculitis with various organ involvement. There have been a few cases of CNS stroke and rare cases of lateral medullary infarction (LMI) as a manifestation of GPA. Also there have been reports of sinuses, nose and laryngeal masses mistakenly referred as carcinomas and subsequently GPA was diagnosed in their pathological reports. Another severe fulminant manifestation can be necrotizing scleritis leading to perforation of sclera. Therefore, here we present some rare and fulminant manifestations of GPA in 3 separate cases for further emphasis of the unusual manifestations of GPA that should always be kept in mind.
Ophthalmic Plastic and Reconstructive Surgery, 2019
Purpose: To review and summarize the clinical features, presentations, diagnostic modalities, and management of ophthalmic manifestations of eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome). Methods: A systematic PubMed search of all English articles on EGPA with ophthalmic involvement was performed. Emphasis was placed on English-language articles, but any article with an abstract translated into English was also included. Only those cases that satisfied the American Rheumatology criteria (1990) for diagnosis were included. Data examined included epidemiology, pathogenesis, presentations, diagnostic modalities, and management. Results: There was a wide range in ophthalmic manifestations of EGPA. In order of most frequent presentation to least frequent, these include central retinal artery or vein occlusion, ischemic optic neuropathy, conjunctival nodules, orbital myositis, proptosis, dacryoadenitis, retinal vasculitis/infarcts/edema, cranial nerve palsy, and amaurosis. The 46 qualifying cases were divided into the categories of ischemic vasculitis versus idiopathic orbital inflammation due to prognostic significance. Ischemic vasculitis cases tended to be older patients (p = 0.03), unilateral (p = 0.006), require immunosuppressive therapy beyond steroids (p = 0.015), and were less likely to show improvement on therapy (p = 0.0003). Conclusions: Prompt diagnosis of EGPA by the ophthalmologist can decrease patient morbidity and mortality. This requires knowledge of likely ophthalmic EGPA presentations, as well as recommended workups and treatment.
Macula Retinal Vasculitis and Choroiditis Associated with Granulomatosis with Polyangiitis
Retinal Cases & Brief Reports, 2016
Purpose: To report a case series of two cases of granulomatosis with polyangiitis, previously known as Wegener granulomatosis, which developed macular necrosis, not previously associated with granulomatosis with polyangiitis, healed with fibrosis, despite aggressive immune-modulating therapy and good control of systemic disease. Methods: Case series of two cases with observation of treatment progress. 2 Results: The results reported the progress of response to treatment in the two cases, which resulted in the final outcome of fibrosis in the macula region, despite being on aggressive immune-modulating therapy and good systemic control. Conclusion: Granulomatosis with polyangiitis can be associated with macular necrosis leading to fibrosis, unresponsive to immune-modulating therapy. A significant proportion of granulomatosis with polyangiitis (GPA), previously known as Wegener granulomatosis, cases are associated with ocular complications, mainly orbital inflammation, peripheral keratitis, and scleritis. Less common ocular complications include retinitis, uveitis, retinal vascular occlusions, and exudative retinal detachment. We report a case series of two cases of GPA, which developed macular necrosis, not previously associated with GPA, healed with fibrosis, despite aggressive immune-modulating therapy (IMT) and good control of systemic disease.
Bilateral isolated retrobulbar optic neuropathy in limited Wegener's granulomatosis
Journal of clinical neuro-ophthalmology, 1993
Wegener's granulomatosis causes a variety of ophthalmologic disorders, some of which occasionally constitute the initial presentation of the disease. We describe a patient who presented with bilateral, isolated, consecutive, posterior optic neuropathy with light perception and no light perception vision. The patient had no other symptoms or signs of orbital disease. Investigations revealed cavitary lung lesions, positive antineutrophilic cytoplasmic antibody (ANCA) titers, subtle focal enhancement of the intracanalicular optic nerves on magnetic resonance imaging, and a confirmatory bronchial biopsy. This exceedingly rare ocular presentation of Wegener's granulomatosis may pose a diagnostic quandary.