Wegener's granulomatosis of the orbit in a 5-year-old child

Ziakas, Nikolaos; Boboridis, Kostas; Gratsonidis, A; Hatzistilianou, Maria; Katriou, D; Georgiadis, Nikolaos

doi:10.1038/sj.eye.6700734

Cited by 9 publications

(4 citation statements)

References 8 publications

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“…5,25 Unlike systemic GPA, there are no definitive diagnostic criteria for the limited forms of GPA, and there are only a handful of case reports describing a clinical picture consistent with limited orbital GPA in children. 26,27 Since the clinical picture and radiographic findings are nonspecific, and ANCA can be negative or borderline in limited disease, biopsy of the orbital inflammatory process and accurate histopathologic interpretations are essential for clinical management.…”

Section: Discussionmentioning

confidence: 99%

Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease in a 12-Year-Old Male

et al. 2018

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Granulomatosis with polyangiitis is rare in children. We report a case of a 12-year-old male who presented with new symptoms of left eyelid swelling and ptosis. Magnetic resonance imaging showed an enhancing orbital mass suspicious for a neoplasm. Excisional biopsy was performed. Microscopic examination revealed fibrovascular tissue with dense collagenous fibrosis and mixed inflammatory infiltrate that included many plasma cells. Many small and medium-sized blood vessels showed granulomatous and necrotizing vasculitis with disruption of the vessel walls and fibrinoid necrosis. Immunostain for IgG highlighted the numerous plasma cells, approximately 50% of which were positive for IgG4 immunostain. A diagnosis of granulomatosis with polyangiitis was suggested, with recommendation of serologic testing for anti-neutrophil cytoplasmic antibodies. Serum anti-neutrophil cytoplasmic antibodies were borderline high with a cytoplasmic staining pattern. The patient improved with steroid and methotrexate therapy. Granulomatosis with polyangiitis can present as an orbital mass in up to 30% of children. It may be misdiagnosed as IgG4-related disease since the inflammatory background in both conditions may be rich in plasma cells with a high proportion of IgG4+ plasma cells, and accompanied by fibrosis and obliterated blood vessels. The differential diagnosis in this location should also include inflammatory pseudotumor and inflammatory myofibroblastic tumor. Knowledge of this unusual manifestation of granulomatosis with polyangiitis and its diagnostic pitfalls can facilitate early diagnosis and treatment.

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Section: Discussionmentioning

confidence: 99%

Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease in a 12-Year-Old Male

et al. 2018

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“…GPA is a rare disease with an annual incidence ranging from 0.5 to 10 cases per million people [6,9]. This form of vasculitis usually manifests in the fourth and fifth decades of life, with a male:female ratio ranging from 1:1 to 1.6:1 [3,[10][11][12][13][14].…”

Section: Discussionmentioning

confidence: 99%

Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) with Hard Palate and Bronchial Perforations Treated with Rituximab—A Case Report

Kosałka

Bazan-Socha²,

Zugaj³

et al. 2014

Advances in Respiratory Medicine

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We present a case of a 57-year-old woman suffering from granulomatosis with polyangiitis (GPA), who in the seventh months of immunosuppressive treatment (cyclophosphamide) progressed with new pulmonary changes and perforations of the hard palate and bronchi. Rituximab was introduced resulting in B-cell depletion and disappearance of anti-PR3 antibody. Palatal holes have substantially diminished and all bronchial perforations disappeared, covered by fibrous tissue. In the fourth month after rituximab administration, large scarring obstruction of the right main bronchus with upper and middle lobes atelectasis emerged. Because of increasing dyspnoea, stenotic bronchus was re-opened by bronchoscopy. Intervention was complicated by bilateral pneumothorax and later, on the seventh day, by fatal pulmonary bleeding. To our knowledge, this is the first report of GPA refractory to cyclophosphamide complicated by palatal and bronchial perforations.

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“…Despite treatment with corticosteroids and immunosuppressive agents, three eyes required enucleation [12]. A recent literature review only revealed four pediatric cases with orbital masses due to WG [11,31,32]. In Belostostky et al [11], two of nine children with eye involvement presented with proptosis and an orbital mass.…”

Section: Wegener's Granulomatosis and Sarcoidosismentioning

confidence: 99%

Ocular complications of childhood rheumatic diseases: Nonuveitic inflammatory eye diseases

Reiff

2009

Curr Rheumatol Rep

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Ocular involvement is common in pediatric rheumatologic diseases, supporting the concept that these conditions do not manifest in isolation but are components of a multisystem inflammatory process. It remains unclear why the eye and its adjacent tissues become a target during paninflammatory disease. Pediatric rheumatologists must recognize ocular disorders, as these conditions significantly concern the treatment team managing serious cases of inflammatory eye disease. Close collaboration between the treating rheumatologist and ophthalmologist is required to prevent potentially devastating outcomes. Therapeutic interventions, such as topical steroids, systemic immunosuppressants, and biologics, must balance the necessity of controlling ocular inflammation and treatment-related adverse effects. This article-the second in a series on ocular complications of childhood rheumatic diseases-reviews the presentation and management of the more common nonuveitic inflammatory ocular manifestations of childhood rheumatologic disease.

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Wegener's granulomatosis of the orbit in a 5-year-old child

Cited by 9 publications

References 8 publications

Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease in a 12-Year-Old Male

Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease in a 12-Year-Old Male

Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) with Hard Palate and Bronchial Perforations Treated with Rituximab—A Case Report

Ocular complications of childhood rheumatic diseases: Nonuveitic inflammatory eye diseases

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