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Case 2. Tarsal conjunctiva ofthe left eye showed hyperaemia, petechial haemorrhages, induration, and oedema.
Source publication
Eight patients who had limited forms of Wegener's granulomatosis are described, with details of their pathology. Ocular pathological data were available for 2 of them. The condition carries a serious ocular risk; useful vision was lost in 6 out of 16 eyes (37%). An indolent but slowly progressive marginal keratitis and scleritis was a prominent fea...
Similar publications
Scleritis is an ocular inflammatory condition characterized by edema and cellular infiltration of the sclera that causes local redness, pain and loss of visual acuity. It can be the first manifestation of a systemic rheumatic disease such as lupus, rheumatoid arthritis and relapsing polycondritis, although it is more frequently found in a well esta...
Citations
... 1 Ocular and orbital involvement is also common, occurring in 30-50% of patients with GPA, sometimes as the sole presenting feature. 2 Scleritis is a known manifestation of GPA and the most common cause of scleritis associated with systemic vasculitis. 3 Therefore, when patients present with severe necrotizing scleritis, ANCA-related systemic vasculitis should be considered as a possible etiology. ...
Purpose:
To report a patient with bilateral scleritis who was initially treated for infectious scleritis, and subsequently diagnosed as granulomatosis with polyangiitis (GPA).
Method:
Retrospective chart review.
Result:
A 48-year-old female with a known history of diabetes presented with pain, redness, and blurring of vision in the right eye. She was diagnosed as scleritis with retinal detachment and underwent vitrectomy, with silicone oil tamponade and intravitreal injections of antibiotics, and antifungal agents. She presented with active scleritis with additional multiple pus points. Several scleral biopsies failed to yield any microorganism and finally, the painful blind eye was enucleated. The enucleated specimen grew gram-positive bacteria which were identified as Staphylococcus arlettae by polymerase chain reaction (PCR)-based sequencing of the 16S rRNA gene. Nine months after the onset of symptoms in the right eye, the patient developed necrotizing scleritis in the left eye. Laboratory investigation revealed a positive cytoplasmic- anti-neutrophil cytoplasmic autoantibody, which was previously negative during the right eye involvement. She was diagnosed with GPA by a rheumatologist and treated accordingly.
Conclusion:
Retinal detachment may be associated with scleritis, and ANCA testing may not detect GPA in its early stages. Once infection has been excluded, clinicians should not be afraid to use high-dose immunosuppression instead of surgery to treat retinal detachment associated with scleritis.
... In a survey of 701 patients, 30% of patients had ocular involvement [4]. In other surveys and studies, up to 50% of patients had ocular involvement, some having the ocular disease as the only complication of GPA [5][6][7]. This case report presents the ocular involvement of GPA in a 71-year-old female who eventually suffered vision loss. ...
Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis is a small vessel vasculitis with a positive ANCA in the serum. One of three diseases that fall under this category is granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis. This case report presents a patient with an ocular manifestation of GPA, rendering a difficult diagnosis and multi-specialty approach to managing the disease.
... [7,8] While the orbit's proximity to the upper airways contributes to the high rate of ocular involvement in systemic GPA, isolated ocular involvement occurs due to focal necrotizing vasculitis. [9] Many different types of ocular involvement have been described, including conjunctivitis, episcleritis, necrotizing scleritis, corneoscleral ulceration, uveitis, optic neuritis, retinal artery occlusion, and orbital involvement with proptosis from pseudotumor of the orbit or orbital cellulitis. [5,7] Scleritis is the most common ocular manifestation in GPA. ...
Purpose:
To describe the clinical features and management of patients with scleritis associated with granulomatosis with polyangiitis (GPA) at a tertiary eye care center in South India.
Methods:
The clinical profile and management of patients presenting to a tertiary eye care center in South India with scleritis secondary to GPA from 2003 to 2021 were analyzed retrospectively. Scleritis was classified into anterior diffuse, nodular, and necrotizing scleritis with inflammation according to Watson and Hayreh's classification. Demographic characteristics, clinical features, anti-neutrophil cytoplasmic antibody (ANCA) positivity, treatment response, ocular complications, and status at the last follow-up were analyzed. Statistical analysis of data was performed using Microsoft Excel 2019.
Results:
Nineteen eyes of 17 patients (15 cytoplasmic staining ANCA [c-ANCA], two p-ANCA positive) were included. Fifteen eyes had necrotizing scleritis, two had diffuse anterior scleritis, and two had nodular scleritis. Remission was induced using a combination of steroids and cyclophosphamide or rituximab. Maintenance therapy was instituted using tapering steroids and immunosuppressants like cyclophosphamide, mycophenolate mofetil, methotrexate, or rituximab. Three eyes required a scleral patch graft. Fourteen patients had good anatomical and visual outcomes, and three were lost to follow-up.
Conclusion:
GPA is a rare disease, while it is the most common ANCA-associated vasculitis with scleritis. As scleritis may be the presenting sign of the disease, ophthalmologists must be aware of the various features suggestive of GPA. GPA-associated scleritis can have a good prognosis when diagnosed promptly and managed aggressively in the acute stage, and remission is maintained with adequate systemic immunosuppression.
... GPA is a potentially lethal, multisystemic vasculitis disorder, classically characterized by a triad of necrotizing granuloma of the respiratory system, systemic vasculitis, and necrotizing glomerulonephritis [2]. Ocular and orbital involvement in patients with GPA is not rare, occurring in as high as 30-50% of patients with GPA and may sometimes be the only presenting feature [3][4][5][6]. ...
... With multidisciplinary care, we were able to save vision of all of the four patients. In long standing case or those that are inadequately treated or those with delay in diagnosis, vision loss is a potential event that may be seen in 8-37% of patients [5,6]. Though there is a remarkable association between scleritis and disease activity, timely diagnosis and the effective treatment prevented the vision loss. ...
Introduction
Ocular and orbital involvement in Granulomatosis with polyangiitis (GPA) is common. GPA can lead to life and sight threatening complications due to necrosis and tissue melting.
Cases
We report four cases presenting with ocular pain and redness for varied durations. One had diminution of vision. All of them had deep sectoral/diffuse congestion with one having scleral thinning. All were diagnosed with anterior necrotizing/non-necrotizing scleritis. One had associated penetrating ulcerative keratitis. Topical steroids and systemic non-steroidal anti-inflammatory drugs were started in all cases and rheumatology consultation was taken. Pertinent investigations were sent, and GPA was diagnosed. Intravenous immunosuppressive regimens and oral steroid were started and significant improvements were seen, preventing untoward complications.
Conclusion
Scleritis could be manifesting feature of GPA so cautious history taking and evaluation is important. Management often requires multidisciplinary care and ocular features could be the reference guidelines to adjust dose of systemic medications of GPA.
... Involvement of the upper respiratory tract accounts for 90% of cases and can cause symptoms of rhinitis, sinusitis, deafness, or epistaxis [1]. Ocular symptoms presented in 30-52% of patients with GPA [1,2] and caused vision loss in 8-37% [1,3]. GPA might cause granulomatous inflammation or vasculitis involving almost any eye structure, resulting in tarsal-conjunctival diseases, keratitis, episcleritis, scleritis, and uveitis [4]. ...
... Involvement of the upper respiratory tract accounts for 90% of cases and can cause symptoms of rhinitis, sinusitis, deafness, or epistaxis[1]. Ocular symptoms presented in 30-52% of patients with GPA [1,2] and caused vision loss in 8-37%[1,3] ...
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.
... Although the isolated form of uveitis is not common, intraocular inflammation has been reported in patients with AAV. Uveitis associated with AAV is nonspecific and can be anterior, intermediate, or posterior, with or without vitritis [34,35]. Uveitis, particularly anterior uveitis, typically occurs secondary to keratitis or scleritis rather than to be a primary involvement. ...
... Several case reports of branch or central retinal vein occlusion (RVO) have also been reported in patients with GPA and EGPA. Possible mechanisms involved in the etiopathogenesis of RVO associated with AAV are focal necrotizing vasculitis of the retinal vessels, subclinical inflammation of the laminar or retrolaminar parts of the optic nerve, and compression of the central retinal vein by extracapillary granulomatous lesions [34,41,42]. The diagnosis is usually confirmed by FFA, which shows delayed or incomplete filling in the distribution of the involved blood vessel or irregular leakage in areas of ischemia. ...
Ocular manifestations in antineutrophil cytoplasmic antibody-associated vasculitis can be associated with the general or limited form of disease and can even occur in the absence of systemic disease. Ocular manifestations of associated vasculitis can be the first symptom of previously not manifested or undiagnosed systemic disease, allowing ophthalmologists to contribute to the diagnosis. Although its ocular findings are variable and nonspecific, the presence of necrotizing changes and peripheral corneal involvement accompanying scleral inflammation are important clues suggesting systemic vasculitis, especially associated vasculitis. The disease may affect all layers of the eye; scleritis and orbital involvement being the most common. Conjunctivitis, episcleritis, peripheral ulcerative keratitis, uveitis and retinal vasculitis are other ocular findings that may be observed during the disease course. Ocular involvement is most commonly seen in granulomatosis with polyangiitis followed by eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. Due to the high morbidity and mortality of associated vasculitis, it is very important to recognize the ocular manifestations of vasculitis as a sign of the underlying systemic disease and an indicator for the disease activity. Treatment varies depending on the location and severity of the ocular involvement. Although localized medical and surgical treatments can help to manage associated ocular disease, systemic immunosuppressive medications are often required to control the underlying disease. With the increasing availability and use of biological agents, prognosis has improved in patients with severe ocular complications. Rituximab appears to be useful in inducing remission and controlling relapses in patients with ocular involvement of associated vasculitis, particularly in cyclophosphamide resistant cases. A multidisciplinary approach in the diagnosis, treatment and follow-up of patients with associated vasculitis is required in order to achieve successful results.
... Although the isolated form of uveitis is not common, intraocular inflammation has been reported in patients with AAV. Uveitis associated with AAV is nonspecific and can be anterior, intermediate, or posterior, with or without vitritis [34,35]. Uveitis, particularly anterior uveitis, typically occurs secondary to keratitis or scleritis rather than to be a primary involvement. ...
... Several case reports of branch or central retinal vein occlusion (RVO) have also been reported in patients with GPA and EGPA. Possible mechanisms involved in the etiopathogenesis of RVO associated with AAV are focal necrotizing vasculitis of the retinal vessels, subclinical inflammation of the laminar or retrolaminar parts of the optic nerve, and compression of the central retinal vein by extracapillary granulomatous lesions[34,41,42]. The diagnosis is usually confirmed by FFA, which shows delayed or incomplete filling in the distribution of the involved blood vessel or irregular leakage in areas of ischemia. ...
Ocular involvement of ANCA associated vasculitis
... Ear, nose and throat (ENT) signs can be present in 70-100%, lung involvement in 50-90%, and ocular involvement in 15-60% of cases [1], [3], [4], [5], [7]. Severe vision loss or total blindness can be seen in 8-37% of cases [3], [11]. Collaboration between subspecialties is critical in these rare conditions -remembering Occam's razor, we must look for a diagnosis that explains all multi-organ symptoms. ...
Vasculitis is a common cause of vision loss, and typically painful. In giant cell arteritis, the most common primary vasculitis in adults, we see elevated inflammatory markers, granulomatous inflammation, and associated headache or scalp tenderness. Vision loss caused by granulomatous with polyangiitis (GPA) is rare and typically associated with pain and orbital findings. Our patient presented for shortness of breath and painless vision loss without orbital inflammation or neural enhancement and a normal fundus exam, suggesting posterior ischemic optic neuropathy. Collaboration amongst sub-specialties and obtaining tissue samples are key to diagnosing granulomatosis with polyangiitis to ensure timely treatment of this fatal and blinding disease.
... The ocular manifestations can be categorized according to the structures involved. The orbit compromise is usually attributed to contiguous spread from the adjacent sinuses [14], and some of the most commonly encountered manifestations are proptosis, diplopia, and decreased vision, secondary to mass compression with nerve involvement (ischemic optic neuropathy) and entrapment of extraocular muscle [4,15]. Eyelid changes in GPA may include edema, entropion, trichiasis, and xanthelasma [16]. ...
... The first, based on the histopathological findings, expose an immune-mediated occlusive necrotizing vasculitis of the anterior ciliary arteries that supply the anterior segment of the eye, leading to ulceration of the peripheral cornea and often accompanied by scleritis (usually necrotizing) [13]. The second refers to the absence of other early signs of anterior segment ischemia, and the early presence of marginal infiltrates that may be an indication of the immunological deficit suffered by patients with GPA, as well as their failure to overcome a bacterial infection that may be an initiating or stimulatory factor for the vasculitis and subsequent tissue necrosis [14]. In both cases, reference is made to the peripheral corneal involvement, which is why the case of our patient is considered unusual, given the very well demarcated paracentral involvement of the cornea with healthy epithelium around it on his OS. ...
... In a case series of 8 patients with GPA and ocular compromise, reported by Spalton et al. in 1981, 3 of the 8 presented PUK of approximately 4 months evolution prior to diagnosis. In all 8 cases, after the initiation of immunosuppressive treatment with corticosteroids or in combination therapy with azathioprine, the patients demonstrated a quiescence of the disease in follow-up periods ranging from 1 to 8 years [14]. In comparison, our patient presented a severe disease phenotype that began with ocular symptomatology and suffered a rapid progression of the disease that led to death in a period of 2 months following the onset of symptoms despite immunosuppressive management. ...
Purpose: To report a case of Granulomatosis with Polyangiitis (GPA) with a fulminant phenotype characterized by diverse ocular and systemic manifestations.
Methods: Case report and literature comparison.
Results: We report the case of a 58 years old male patient with diagnosis of GPA, who debuted with atypical ocular manifestations concomitant with systemic compromise that led to a severe and fulminant progression of the disease.
Conclusion: Early diagnosis and an appropriate interdisciplinary approach are required in patients with atypícal manifestations of GPA since it could be a life-threatening disease.
... of an asymptomatic patient 2 . There may be involvement of other tissues such as the skin and eye 6,8,9 Chest X-ray in this condition shows pulmonary lesions predominantly in the lower lobes and the most common form is a discrete lesion greater than 1cm. in diameter and in two thirds of cases the lesions are multiple and bilateral ; and in one third cases cavitation occurs. 2 CECT Scan of the thorax can show nodular and cavitating lesions which are not obvious on chest radiography. 11 Pulmonary lesions in Limited Wegener`s.Granulomatosis are similar to that of Classical form. ...
Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis involving small and medium-sized blood vessels and granulomatous inflammation of upper and lower respiratory systems and/or renal system. In the limited form of GPA, there is no systemic involvement of disease with sparing of kidneys. The respiratory system is the commonly involved organ in limited GPA. Herein, we report the case of a 40-year-old male who was initially diagnosed as sarcoidosis clinically. Lung biopsy revealed necrotizing granulomatous angiitis. Diagnosis of GPA was made which was substantiated by antineutrophil cytoplasmic antibody (ANCA) positivity. This was a case of limited GPA with isolated lung involvement. The early diagnosis and initiation of treatment are critical for improved survival of patients with GPA. Tissue biopsy is necessary for the diagnosis of GPA.
