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\begin{document}

\title{A case of IgG4-related disease with unilateral visual field impairment}



\author[1]{Shintaro Kohno}%
\author[1]{Hitoshi Tabuchi}%
\author[1]{Atsuki Fukushima}%
\affil[1]{Tsukazaki Hospital}%


\vspace{-1em}



  
  \date{June 12, 2024}


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\selectlanguage{english}
\begin{abstract}
A 76-year-old man receiving maintenance therapy with oral steroids for
IgG4-related disease presented to our hospital with the chief complaint
of visual disturbance. After detailed history-taking for IgG4-related
disease, an imaging diagnosis of an intracranial lesion was made.
Intensified steroid therapy was performed, which led to symptom
resolution.%
\end{abstract}%



\sloppy


\textbf{Introduction}

IgG4-related disease is an autoimmune disease of unknown etiology in
which IgG4-positive plasma cells infiltrate the body organs and cause
swelling, nodules, and hypertrophic lesions in target organs.
Approximately 20\% and 5\% of patients have vision loss and visual field
defects, respectively\textsuperscript{1)}.

Two major pathological conditions can cause optic neuropathy in this
disease: compression or encasement of the optic nerve by an enlarged
tissue in the orbit and compression of the optic chiasm by the enlarged
pituitary gland. In the former case, compression or encasement of the
optic nerve by the enlarged external ophthalmic muscle or localized or
diffuse tissue proliferation in the orbit has been
reported\textsuperscript{2)}. The latter is a condition that can develop
in any disease that causes pituitary enlargement, including the present
case; however, few studies have reported such
cases\textsuperscript{3)4)} and there are no case reports of visual
impairment alone.

\textbf{Case presentation}

Initial evaluation

A 76-year-old man visited his family physician at the end of April X
(year) due to visual disturbance in the left eye and was referred to our
department on May 27, X. Ophthalmological history included bilateral
cataract surgery, left ptosis (both in X-4 years), intrascleral fixation
of left intraocular lens dislocation (in X-1 year), Irvine Gass
syndrome, and ocular hypertension. Detailed history-taking revealed that
the patient received treatment for IgG4-related disease (retroperitoneal
fibrosis; prednisolone 2.5 mg/day) from his primary care physician.

Current medical history

At the initial visit on June 2 X, there was no conjunctival hyperemia in
both conjunctivae, and anterior chamber cells were mildly present on the
left eye. The left optic nerve papilla was mildly erythematous and
swollen. Visual acuities were 1.2 (1.2 \selectlanguage{ngerman}× S +1.75D) in the right eye and
0.6 (0.7 × S +2.00D) in the left eye, and intraocular pressures were 18
mmHg in the right eye and 16 mmHg in the left eye. Critical flicker
frequency (CFF) was decreased in the left eye (right \selectlanguage{english}|38Hz\selectlanguage{english}|41Hz, left
\selectlanguage{english}|36Hz\selectlanguage{english}|36Hz), and Humphrey visual field test revealed inferior
auriculotemporal one-quarter blindness in the left eye (Figure 1). Brain
magnetic resonance imaging (MRI) showed no abnormal signal in the
infraorbital optic nerve; however, marked pituitary/pituitary
sclerenchyma enlargement with associated optic chiasm compression was
observed (Figure 2).

Intervention description/results

Based on the history and initial evaluation findings, a diagnosis of
left optic neuropathy associated with pituitary/pituitary scrofula
enlargement caused by IgG4-related disease was made. On June 10, visual
acuity and visual field defects improved (Figure 3), and upon
examination on July 7, the pituitary gland and pituitary pattern were
almost normalized (Figure 4), and the CFF decreased (right \selectlanguage{english}|42Hz\selectlanguage{english}|43Hz,
left \selectlanguage{english}|37Hz\selectlanguage{english}|43Hz) (39 Hz). The steroid dose was gradually decreased, and
maintenance therapy with 2.5-mg prednisolone daily was resumed on August
11.

\textbf{Discussion}

In this case, no optic nerve swelling or signal changes were evident on
imaging, but fundus examination revealed mild erythema of the optic
nerve papilla. Although this finding could be attributed to iritis, the
possibility of optic neuritis caused by an autoimmune mechanism in
IgG4-related disease could not be ruled out. However, the visual field
findings were not consistent with those commonly observed in optic
papillitis. Moreover, brain MRI findings suggested optic chiasm
compression due to pituitary enlargement. The patient's visual acuity
and visual field defects improved following pituitary gland enlargement
after steroid treatment, suggesting that pituitary gland enlargement and
pituitary scrofula were the main pathophysiology of vision loss and
visual field defects.

The ophthalmologic clinical hallmark of IgG4-related ocular disease is
bilateral lacrimal gland enlargement with three features: suborbital
nerve enlargement, exophthalmos, and pressure optic
neuropathy\textsuperscript{2)}. IgG4-related optic neuropathy is
commonly caused by compression of the optic nerve by enlargement of the
lacrimal glands or other intraorbital
tissues\textsuperscript{2)5)6)7)8)}. Few studies have reported on optic
neuropathy caused by compression of the optic chiasm by an enlarged
pituitary gland as in the present case\textsuperscript{3)7)8)}. Although
bilateral hemianopsia is generally observed with pituitary enlargement,
the present case showed mild visual field defects in not only the
auriculolateral lower one-quarter blindness but also the nasal lower
one-quarter region. A previous study\textsuperscript{9)} found visual
field defects in only one eye in two of 10 patients with symmetrical
pituitary enlargement. Because the present case also showed optic chiasm
compression due to pituitary enlargement, it is possible that it caused
an atypical visual field defect and associated visual acuity impairment.

Alternatively, as other studies have shown infiltration of IgG4-positive
plasma cells around the trigeminal nerve in the same
disease\textsuperscript{2)}, a deep infiltrative or inflammatory
mechanism may be involved in this case of optic neuropathy.

In conclusion, it is necessary to consider that the effects of
IgG4-related disease may be involved when patients complain of vision
loss and visual field impairment.

\textbf{Declarations} :

\begin{enumerate}
\tightlist
\item
  State of Ethics: Ethical approval is not required for this study in
  accordance with local or national guidelines. Written informed consent
  was obtained from the patient for publication of the details of their
  medical case and any accompanying images.
\item
  Conflict of Interest: Not applicable.
\item
  Funding Sources- No funding was obtained for this study.
\item
  Author contributions and Data Availability: Shintaro Kohno, Hitoshi
  Tabuchi and Atsuki Fukushima evaluated the findings of patients and
  collected data. Shintaro Kohno and Atsuki Fukushima wrote the
  manuscript. All data generated or analysed during this study are
  included in this article.
\end{enumerate}

\textbf{References}

\begin{enumerate}
\tightlist
\item
  Goto H, Ueda SI, Nemoto R, Ohshima KI, Sogabe Y, Kitagawa K,~\emph{et
  al} . Clinical features and symptoms of IgG4-related ophthalmic
  disease: a multicenter study. Jpn J Ophthalmol. 2021;65:651-656
\item
  Kashii S. IgG4-related disease: a neuro-ophthalmological perspective.
  J Neuroophthalmol. 2014;34:400-407
\item
  Koide H, Shiga A, Komai E, Yamato A, Fujimoto M, Tamura A, \emph{et
  al.} Prednisolone-responsive postpartum IgG4-related hypophysitis.
  Intern Med. 2018;57:367-375
\item
  Osawa S, Ogawa Y, Watanabe M, Tominaga T. Hypophysitis presenting with
  atypical rapid deterioration: with special reference to immunogloblin
  G4-related disease -case report-. Neurol Med Chir. 2009;49:622-625
\item
  Wang X, Sun X, Ao R, Zeng Z, Li D, Li Y. Visual impairment in
  immunoglobulin G4-related disease: a case report. Exp Ther Med.
  2021;21:200
\item
  Lai KKH, Chan RYC, Chin JKY, Li KKW, Young AL, Yuen HKL, \emph{et
  al.}Upper cranial nerve involvement and immunoglobulin G4-related
  optic neuropathy. Opthalmology. 2020;127:699-703
\item
  Takahashi Y, Kitamura A, Kakizaki H. Bilateral optic nerve involvement
  in immunoglobulin G4-related ophthalmic disease. Neuroophthalmol.
  2014;34:16-19
\item
  Zhang W, Luo J, Jiao J\emph{.} Optic nerve involvement in
  immunoglobulin G4-related disease: a case report. Exp Ther Med.
  2016;12:111-114
\item
  Lee IH, Miller NR, Zan E, Tavares F, Blitz AM, Sung H, \emph{et
  al.}Visual defects in patients with pituitary adenomas: the myth of
  bitemporal memianopsia. AJR Am J Roentgenol. 2015;205:W512-518
\end{enumerate}

\textbf{Figure Legends}

Figure 1. HFA visual field test on June 2. A. right eye. No abnormal
findings. B. Left eye. Note inferior auriculotemporal 1/4 blindness in
the left eye.

Figure 2. Brain MRI on June 2. A. Coronal Section. B. Sagittal Section.
Note that marked pituitary/pituitary sclerenchyma enlargement with
associated optic chiasm compression without any abnormal findings in
optic nerve.

Figure 3. HFA visual field test on June 2. A. right eye. B. Left eye.
Note that abnormal findings in the left eye disappeared.

Figure 4. Brain MRI on June 2, X year. R. A. Coronal Section. B.
Sagittal Section. Note that the pituitary gland and pituitary pattern
were almost normalized.

\textbf{Hosted file}

\verb`2022.4.22 Figure .pptx`  available at \url{https://authorea.com/users/478015/articles/566370-a-case-of-igg4-related-disease-with-unilateral-visual-field-impairment}

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