Atypical painless vision loss in a patient with granulomatosis with polyangiitis

oc000173 10.3205/oc000173 urn:nbn:de:0183-oc0001733 Case Report Atypical painless vision loss in a patient with granulomatosis with polyangiitis Nelson Nelson Patricia C. PC MD

Ophthalmology, Department of Surgery, Texas Tech University Health Sciences Center El Paso, 2000 B Transmountain Rd, El Paso, TX 79911, United States, Phone: +1 915 215-8521, Fax: +1 915 612-9253Ophthalmology, Department of Surgery, Texas Tech University Health Sciences Center El Paso, United States

patricia.nelson@ttuhsc.edu author Kunam Kunam Vamsi V

Interventional Radiology, Department of Radiology, Texas Tech University Health Sciences Center El Paso, United States

Vamsi.Kunam@ttuhsc.edu author Prospero Ponce Prospero Ponce Claudia C

Ophthalmology, Department of Surgery, Texas Tech University Health Sciences Center El Paso, United States

clprospe@ttuhsc.edu author German Medical Science GMS Publishing House

Düsseldorf

610 20201215 engl This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). 2193-1496 10 GMS Ophthalmology Cases GMS Ophthalmol Cases 46 Vasculitis is a common cause of vision loss, and typically painful. In giant cell arteritis, the most common primary vasculitis in adults, we see elevate</PlainText></TextGroup>d inflammatory markers, granulomatous inflammation, and a<TextGroup><PlainText>ssoci</PlainText></TextGroup>ated 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 an<TextGroup><PlainText>d obtain</PlainText></TextGroup>ing tissue samples are key to diagnosing granulomatosis with polyangiitis to ensure timely treatment of this fatal and blinding disease.</Pgraph></Abstract> <TextBlock linked="yes" name="Introduction"> <MainHeadline>Introduction</MainHeadline><Pgraph>Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis affecting the small- and m<TextGroup><PlainText>ediu</PlainText></TextGroup>m-sized vessels in the orbit, sinus, lungs, and kidneys <TextLink reference="1"></TextLink>, <TextLink reference="2"></TextLink>. It is a rare disease with the annual incidence being 2.4–11 cases per million, equally affecting both genders of individuals in their 40s–70s <TextLink reference="1"></TextLink>, <TextLink reference="3"></TextLink>, <TextLink reference="4"></TextLink>, <TextLink reference="5"></TextLink>, <TextLink reference="6"></TextLink>. Ocular findings are seen in 15–60% of cases and can be some of the earliest findings <TextLink reference="1"></TextLink>, <TextLink reference="2"></TextLink>, <TextLink reference="3"></TextLink>, <TextLink reference="5"></TextLink>, <TextLink reference="7"></TextLink>. Nearly 50% of patients can develop painful scleritis, as well as other ocular signs and symptoms including uveitis, keratitis, and orbital inflammation resulting in proptosis, diplopia, and epiphora. If orbital findings are present, 20–50% of patients can develop severe vision loss <TextLink reference="1"></TextLink>, <TextLink reference="3"></TextLink>, <TextLink reference="8"></TextLink>, <TextLink reference="9"></TextLink>. Painless vision loss without orbital findings has rarely been reported <TextLink reference="1"></TextLink>, <TextLink reference="10"></TextLink>. High clinical suspicion and collaboration with multiple sub-specialties is required to reduce mortality and morbidity.</Pgraph></TextBlock> <TextBlock linked="yes" name="Case description"> <MainHeadline>Case description</MainHeadline><SubHeadline>History</SubHeadline><Pgraph>A 64-year-old Caucasian woman presented to the ER for shortness of breath following a recent sinus surgery for a deviated septum. During her stay, ophthalmology was consulted for right vision loss. The patient was being treated for cavitary pneumonia with suspected invasive aspergillosis. She denied any eye pain, headache, scalp tenderness, or jaw claudication, and could not remember the date of onset of her vision decline, except that it was following recent sinus surgery.</Pgraph><SubHeadline>Examination</SubHeadline><Pgraph>On clinical exam, her vision was no-light-perception (NLP) in the right eye with a relative afferent pupillary defect, and 20/20 vision in her left eye. Anterior and posterior exam was normal in both eyes with no evidence of proptosis, optic nerve edema, hemorrhages, cotton wool spots, or pallor. Initial work-up showed leukocytosis (34.15), elevated erythrocyte sedimentation rate (90), and C-reactive protein (3.51). CT chest showed cavitary lesions, and a bronchio-alveolar lavage showed increased galactom<TextGroup><PlainText>anna</PlainText></TextGroup>n, leading to a diagnosis of suspected aspergillosis. A magnetic resonance imaging (MRI) of the brain and orbits showed no optic nerve abnormalities, enhancement, or orbital inflammation. The patient received amphotericin B and IV steroids with symptomatic improvement except for her vision. The patient declined a lung biopsy. Low-dose oral steroids, 40 mg prednisone, were continued and not increased due to suspected aspergillosis pneumonia.</Pgraph><SubHeadline>Clinical course</SubHeadline><Pgraph>Three weeks later, oral prednisone had been discontinued and the patient began developing new ocular symptoms in her left eye as well as a right 3<Superscript>rd</Superscript> nerve palsy. C-ANCA positivity was confirmed. Despite high-dose steroids, the patient progressed to NLP vision in the contralateral left eye, and repeated MRI demonstrated interval development of acute left optic nerve ischemia and bilateral intra-orbital inflammation (Figure 1 <ImgLink imgNo="1" imgType="figure"/>). Repeat ENT biopsy was obtained and demonstrated focal arteritis with some giant cells and no invasive fungal elements, ruling out invasive aspergillosis and confirming the suspicion of GPA. The patient developed a nasal deformity with a saddle nose, cotton wool spots in the left retina, and acute renal failure requiring dialysis. Reinstitution of high-dose steroids and adjunctive cyclophosphamide reversed the patient’s acute renal failure and the 3<Superscript>rd</Superscript> nerve palsy, but did not reverse her vision loss.</Pgraph></TextBlock> <TextBlock linked="yes" name="Discussion"> <MainHeadline>Discussion</MainHeadline><Pgraph>Granulomatosis with polyangiitis is a rare disease and often affects multiple organ systems. 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 <TextLink reference="1"></TextLink>, <TextLink reference="3"></TextLink>, <TextLink reference="4"></TextLink>, <TextLink reference="5"></TextLink>, <TextLink reference="7"></TextLink>. Severe vision loss or total blindness can be seen in 8–37% of cases <TextLink reference="3"></TextLink>, <TextLink reference="11"></TextLink>. 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. Nasal tissue biopsy ruled out aspergillosis and suggested changes of vasculitis with diffuse necrosis. Pathologic tissue confirmation is highly recommended in patients when imaging and other laboratory studies are insufficient.</Pgraph></TextBlock> <TextBlock linked="yes" name="Conclusions"> <MainHeadline>Conclusions</MainHeadline><Pgraph><OrderedList><ListItem level="1" levelPosition="1" numString="1.">Granulomatosis with polyangiitis can present with painless vision loss without other orbital or ocular findings.</ListItem><ListItem level="1" levelPosition="2" numString="2.">A multidisciplinary approach is essential for timely diagnosis and management of granulomatosis with polyangiitis.</ListItem><ListItem level="1" levelPosition="3" numString="3.">Tissue specimen is essential to diagnose GPA and rule out other confounding etiologies.</ListItem></OrderedList></Pgraph></TextBlock> <TextBlock linked="yes" name="Notes"> <MainHeadline>Notes</MainHeadline><SubHeadline>Competing interests</SubHeadline><Pgraph>The authors declare that they have no competing interests.</Pgraph></TextBlock> <References linked="yes"> <Reference refNo="1"> <RefAuthor>Sfiniadaki E</RefAuthor> <RefAuthor>Tsiara I</RefAuthor> <RefAuthor>Theodossiadis P</RefAuthor> <RefAuthor>Chatziralli I</RefAuthor> <RefTitle>Ocular Manifestations of Granulomatosis with Polyangiitis: A Review of the Literature</RefTitle> <RefYear>2019</RefYear> <RefJournal>Ophthalmol Ther</RefJournal> <RefPage>227-34</RefPage> <RefTotal>Sfiniadaki E, Tsiara I, Theodossiadis P, Chatziralli I. 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DOI: 10.1136/bjo.65.8.553</RefTotal> <RefLink>https://doi.org/10.1136/bjo.65.8.553</RefLink> </Reference> </References> <Media> <Tables> <NoOfTables>0</NoOfTables> </Tables> <Figures> <Figure format="png" height="520" width="731"> <MediaNo>1</MediaNo> <MediaID>1</MediaID> <Caption><Pgraph><Mark1>Figure 1: Post-Gd axial T1-weighted MR image with fat saturation shows enhancement of bilateral posterior sclera extending to the adjacent optic nerve sheath (arrows); findings are consistent with scleritis. Also noted is mild ill-defined enhancement in retro-conal fat (arrowheads), consistent with mild post-septal cellulitis.</Mark1></Pgraph></Caption> </Figure> <NoOfPictures>1</NoOfPictures> </Figures> <InlineFigures> <NoOfPictures>0</NoOfPictures> </InlineFigures> <Attachments> <NoOfAttachments>0</NoOfAttachments> </Attachments> </Media> </OrigData> </GmsArticle>