| Identifier |
egpa_lee |
| Title |
Eosinophilic granulomatosis with polyangitis (eGPA) |
| Creator |
Andrew G. Lee, MD; Nicole Weber |
| Affiliation |
(AGL) Chairman, Department of Ophthalmology, The Methodist Hospital, Houston, Texas; Professor of Ophthalmology, Weill Cornell Medicine, New York City, New York; (NW) Class of 2022, Baylor College of Medicine, Houston, Texas |
| Subject |
eGPA; Eosinophilic Granulomatosis |
| Description |
Summary: • Eosinophilic granulomatosis with polyangitis (eGPA or Churg-Strauss) o Presentation key features: > Orbital inflammatory syndrome • Redness, pain, lid edema, diplopia, ophthalmoplegia, optic neuropathy > Vasculitis-like presentation • Stroke o Past medical history > asthma o American College of Rheumatology diagnostic criteria include: > Asthma > Eosinophilia (>10% in peripheral blood evident on CBC with differential) > Mononeuritis multiplex or polyneuropathy > Pulmonary infiltrates on chest x-ray (non-specific, patchy) > Paranasal sinusitis > Mononeuritis multiplex or polyneuropathy > Biopsy specimen • Might show histological proof of vasculitis with eosinophils o Summary: the key differentiating feature of eGPA is eosinophilia > Add to differential in patient with history of asthma with neuro-ophthalmic complaint> Work up with a chest x-ray, CBC with differential, ANCA, imaging of head/orbit and biopsy. |
| Transcript |
So today we're going to be talking about eGPA, which stands for "eosinophilic." Which, as you know, is one of the cells in the blood-- the eosinophil. Granulomatosis with polyangitis. And this used to be known as "Churg-Strauss," but as you know we're moving away from the eponyms now, especially the ones with Nazi past, even though Churg-Strauss doesn't have any Nazi past associated with it. So, the key features of eGPA are: they're going to come to neuro-ophthalmology with some complaint. And the neuro-op complaints can either be orbital inflammatory syndrome, and that looks like idiopathic orbital inflammatory psuedotumor-- redness, pain, lid edema, diplopia, ophthalmoplegia, optic neuropathy (the typical orbital presentations). But, you can also have vasculitis-like presentations including stroke. And it's a great mimicker like Wegener's granulomatous, which is the GPA-- that one is the Nazi version, so we call that one granulomatosis with polyangitis without the eosinophilia. So, in the past medical history the main tip-off that we should be thinking about eGPA in this patient is if they have asthma. And so, that is one of the diagnostic criteria that's necessary for the diagnosis of eGPA. So, as soon as you see that PMH "asthma" plus any neuro-ophthalmic complaint, either orbital or neuro-op, (including orbital pain, ophthalmoplegia, diplopia, ptosis) then we should be considering eGPA in the differential diagnosis. The rest of the diagnostic criteria in the American College of Rheumatology criteria, in addition to the asthma and the eosinophils in the blood (usually greater than 10%)-- you should be thinking about it when you have multiple neuropathies that are asynchronous, peripheral neuropathies (motor or sensory), and that we call mononeuritis multiplex, or if they have a polyneuropathy. So, the mononeuritis multiplex is noncontiguous/asynchronous/different locations. And, obviously, we're going to be worried about pulmonary infiltrate and we're going to do a chest x-ray. They're usually very non-specific, patchy infiltrates. There's going to be upper respiratory symptoms just like with GPA (sinusitis) and it could mimic a lot of similar-type benign pathologies in the upper respiratory tree and then the biopsy (of whatever we can biopsy) might show the vasculitis, including temporal artery biopsies, which can show the eosinophilic form of vasculitis, and that eosinophilia is what we're looking for both in the blood (in the CBC differential) and in the biopsy specimen. It's eos' that we're looking for. So the key differentiating feature of eGPA is the "e"-- that's eosinophils. You should be thinking about it in anyone who has a history of asthma, who presents with a neuro-ophthalmic complaint. We're going to do a chest x-ray, a CBC with differential, ANCA, You're going to be looking at their sinuses. If it's orbital, we going to image the orbit. If it's head we're going to image the head. If it's both you're going to do head and orbit. You're going to ask about mononeuritis multiplex and polyneuropathy (motor and sensory). And, ultimately, we're going to look for biopsy looking for those eosinophils. |
| Date |
2021-04 |
| Language |
eng |
| Format |
video/mp4 |
| Type |
Image/MovingImage |
| Collection |
Neuro-Ophthalmology Virtual Education Library: Andrew G. Lee Collection: https://novel.utah.edu/Lee/ |
| Publisher |
North American Neuro-Ophthalmology Society |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890 |
| Rights Management |
Copyright 2019. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6bc9w4c |
| Setname |
ehsl_novel_lee |
| ID |
1680640 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6bc9w4c |
