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. 2023 Jul 2;12(13):4448.
doi: 10.3390/jcm12134448.

Ocular Involvement of Granulomatosis with Polyangiitis

Anna Byszewska  1 , Izabela Skrzypiec  1 , Aleksandra Rymarz  2 , Stanisław Niemczyk  2 , Marek Rękas  1
Affiliations

Ocular Involvement of Granulomatosis with Polyangiitis

Anna Byszewska et al. J Clin Med. .

Abstract

Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener's disease, is a form of ANCA-associated vasculitis. It manifests mainly in the kidneys and the upper respiratory tract, but ocular involvement is not uncommon. In this article, four cases with ocular manifestations are presented with comprehensive photographic documentation. We describe the way to proper diagnosis, which may be long, the possible treatment, and the final outcomes. Our patients had the following ocular manifestations of GPA: retinal vasculitis, anterior necrotizing scleritis, medial orbital wall and orbital floor erosion with middle face deformation, compressive optic neuropathy due to retrobulbar inflammatory mass, and the abscess of the eyelids, inflammatory intraorbital mass causing exophthalmos and diplopia. This manuscript includes the description of severe forms of GPA, the initial signs and symptoms, relapses, and difficulties in achieving remission. The extraocular involvement is described with diagnostic modalities and laboratory findings. One of the reported cases was diagnosed by an ophthalmologist on the basis of ocular symptoms in the early stages of the disease. Our outcomes are compared with those discussed in the literature.

Keywords: ANCA; GPA; GPA ocular symptoms; Wegener’s disease; vasculitis.

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Conflict of interest statement

No conflict of interest to declare.

Figures

Figure 1
Figure 1
Left lower limb skin ulcer before the introduction of treatment.
Figure 2
Figure 2
A photo of the patient’s right eye at the initial presentation. Conjunctival redness and chemosis. Adjacent to the area of scleritis, corneal peripheral keratitis can be distinguished (the asterisk).
Figure 3
Figure 3
External photo at the initial presentation.
Figure 4
Figure 4
A photo of the fundus with vasculitis in the retinal vessels. Arteries and veins are involved. Cotton wool spots and hemorrhages are present. A color photo on the left and a red-free photo on the right.
Figure 5
Figure 5
Anterior necrotizing scleritis. The upper image is slit lamp photo, and the lower image is a photo with fluorescein and a cobalt filter. (A1) Initial presentation, instead of the sclera, a whitish discharge overlies the uvea. (A2) Fluorescein staining in the whole area of scleritis. (B1) New vessels are seen around the margins of scleritis. (B2) No staining in the areas of new vessels. (C1) More new vessels around the area. (C2) A significantly smaller area of staining. The area without staining is covered with new tissue. (D1) After recovery, the white tissue with a net of small vessels covers the uvea. (D2) No staining.
Figure 6
Figure 6
The collapsed nasal cavity with the involvement of the left orbit. Necrotic nasal structures. The position of the lower eyelid is lower than in the right eye. Lack of soft tissues in the median palpebral angle.
Figure 7
Figure 7
A CT scan of the head. Loss of bony and soft tissue in the middle face and left orbital walls.
Figure 8
Figure 8
Loss of skin tissue in the median palpebral angle, loss of conjunctiva. Necrotizing tissue covered with whitish discharge. Lagophthalmos of the left eye.
Figure 9
Figure 9
Structures of the orbit could be assessed through the collapsed nasal cavity. The following was noted in the patient: necrotic tissue at the medial wall and floor of the orbit, visible medial loss of conjunctival tissue on the orbital wall, and bare medial rectus muscle.
Figure 10
Figure 10
Patient after the initial step of the planned reconstructive surgery. Skin transplant partially covers the nasal cavity. The left eyeball is significantly lower with the enophthalmos of 4 mm. There is a relatively high risk of displacement of the affected eye into the orbit.
Figure 11
Figure 11
OCT RNFL image. Bilateral optic disc edema due to central venous thrombosis, more pronounced in the RE.
Figure 12
Figure 12
MRI of axial plane—a compressive mass in the left retrobulbar space causing the compression of the left optic nerve.
Figure 13
Figure 13
MRI of coronal plane with a massive lesion in the right ethmoid sinus cavity.
Figure 14
Figure 14
MRI of the head and axial plane revealed frontal sinus involvement, more severe on the right side.
Figure 15
Figure 15
MRI of the head (sagittal plane) revealed frontal and ethmoid sinus involvement.
Figure 16
Figure 16
Fundus OCT and RNFL image. Optic disc pallor following compressive neuropathy in the LE. Difficulty with fixation due to light perception in the LE.
Figure 17
Figure 17
Typical nasal discharge in a GPA patient.
Figure 18
Figure 18
(A) Skin lesions on the on the face (behind and in front of the right ear) and (B) on the chest.
Figure 19
Figure 19
(A) Right eyelid edema due to an abscess in the lower eyelid. (B) The right lower eyelid after surgical evacuation of the abscess. (C) Improvement of the edema.
Figure 20
Figure 20
CT of the head and orbit. The image revealed a retrobulbar massive lesion on the right side. Proptosis of the right eye and RE motility restriction.
Figure 21
Figure 21
OCT RNFL. Optic disc edema in the right eye caused by retrobulbar granulomatosis.
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