Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings

Karra Muller; Jonathan H Lin

doi:10.5858/arpa.2013-0006-RS

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Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings

Journal article

Open access

Peer reviewed

Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings

Karra Muller and Jonathan H Lin

Archives of pathology & laboratory medicine (1976), Vol.138(8), pp.1110-1114

08/2014

DOI: 10.5858/arpa.2013-0006-RS

PMCID: PMC4140401

PMID: 25076302

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Abstract

The pathology of granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis, typically features a granulomatous and sometimes necrotizing vasculitis targeting the respiratory tract and kidneys. However, orbital involvement occurs in up to 60% of patients and is frequently the first or only clinical presentation in patients with systemic or limited forms of GPA. Orbital GPA can cause significant morbidity and potentially lead to complete loss of vision and permanent facial deformity. Fortunately, GPA is highly responsive to medical treatment with corticosteroids combined with cyclophosphamide or, more recently, rituximab. Therefore, it is imperative for this disease to be accurately diagnosed on orbital biopsy and distinguished from other histologically similar orbital lesions. Herein, we review the clinical and pathologic findings of orbital GPA, focusing on the differentiation of this disease from other inflammatory orbital lesions.

Granulomatosis with Polyangiitis - diagnosis

Prognosis

Orbit - immunology

Humans

Immunosuppressive Agents - therapeutic use

Orbit - blood supply

Microvessels - pathology

Adrenal Cortex Hormones - therapeutic use

Microvessels - immunology

Cyclophosphamide - therapeutic use

Orbital Diseases - diagnosis

Granulomatosis with Polyangiitis - immunology

Orbital Diseases - drug therapy

Drug Therapy, Combination

Granulomatosis with Polyangiitis - pathology

Orbit - pathology

Diagnosis, Differential

Microvessels - drug effects

Rituximab

Orbit - drug effects

Orbital Diseases - immunology

Biopsy

Orbital Diseases - pathology

Granulomatosis with Polyangiitis - drug therapy

Early Diagnosis

Antibodies, Monoclonal, Murine-Derived - therapeutic use

Details

Title: Subtitle: Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings
Creators: Karra Muller - From the Department of Pathology, UC San Diego, San Diego (Dr Muller), and the Department of Pathology, UC San Diego, La Jolla (Dr Lin), California
Jonathan H Lin
Resource Type: Journal article
Publication Details: Archives of pathology & laboratory medicine (1976), Vol.138(8), pp.1110-1114
DOI: 10.5858/arpa.2013-0006-RS
PMID: 25076302
PMCID: PMC4140401
NLM abbreviation: Arch Pathol Lab Med
ISSN: 0003-9985
eISSN: 1543-2165
Publisher: United States
Grant note: R01 EY020846 / NEI NIH HHS
Language: English
Date published: 08/2014
Academic Unit: Pathology
Record Identifier: 9984047767502771

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