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\begin{document}

\title{A Case of Recurring Kikuchi Disease and Autoimmune Hepatitis}



\author[1]{Umaima  Dhamrah}%
\author[1]{Branden  Ireifej}%
\author[2]{Sibghatallah  Ummar}%
\author[1]{Nuzhat  Batool}%
\author[3]{David Song}%
\author[1]{Nirali  Sheth}%
\author[4]{Abhigan  Shrestha}%
\author[5]{Vikash Jaiswal}%
\affil[1]{Icahn School of Medicine at Mount Sinai}%
\affil[2]{Bahria University Medical \& Dental College Karachi, Pakistan}%
\affil[3]{Icahn School of Medicine at Mount Sinai Elmhurst Hospital}%
\affil[4]{M Abdur Rahim Medical Medical College Hospital}%
\affil[5]{AMA School of Medicine}%


\vspace{-1em}



  
  \date{May 24, 2024}


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\begin{abstract}
We present a case of a 47-year-old female with a history of diagnosed KD
and autoimmune hepatitis 13 years ago who presented with recurrent
fevers and a desquamative rash on the lower extremities. Patient has
elevated ASMA titer, and a subsequent liver biopsy confirmed the
diagnosis of autoimmune hepatitis%
\end{abstract}%



\sloppy


\textbf{A Case of Recurring Kikuchi Disease and Autoimmune Hepatitis}

\emph{Authors} :

\begin{enumerate}
\tightlist
\item
  Umaima Dhamrah\textsuperscript{1}(dhamrahumaima@gmail.com),(Department
  of Internal Medicine, NYC Health \& Hospitals/Elmhurst Hospital
  Center, Elmhurst NY, USA
\item
  Branden Ireifej\textsuperscript{1}(\emph{ireifejb@nychhc.org}),
  (Department of Internal Medicine, NYC Health \& Hospitals/Elmhurst
  Hospital Center, Elmhurst NY, USA )
\item
  Sibghatallah
  Ummar\textsuperscript{2}(\emph{ummarsibghatallah@gmail.com}),
  Department of Medicine, Bahria University Medical \& Dental College
  Karachi, Pakistan
\item
  Nuzhat Batool
  (\emph{batooln@nychhc.org})\textsuperscript{1}(Department of Internal
  Medicine, NYC Health \& Hospitals/Elmhurst Hospital Center, Elmhurst
  NY, USA
\item
  David Song DO, (\emph{xdavidsong@gmail.com}), (Department of Internal
  Medicine, NYC Health \& Hospitals/Elmhurst Hospital Center, Elmhurst
  NY, USA)
\item
  Nirali Sheth (\emph{niralisheth221@gmail.com}), (Department of
  Internal Medicine, NYC Health \& Hospitals/Elmhurst Hospital Center,
  Elmhurst NY, USA)
\item
  Abhigan Babu Shrestha MBBS (\emph{abigan17@gmail.com}), (Department of
  Medicine, M Abdur Rahim Medical College, Dinajpur, Bangladesh)
\item
  Vikash Jaiswal (Larkin Community Hospital, South Miami, FL, USA)
\end{enumerate}

\textbf{Corresponding Author:}

Abhigan Babu Shrestha MBBS

Department of Medicine, M Abdur Rahim Medical College, Dinajpur,
Bangladesh

Email Id: \emph{abigan17@gmail.com}

\textbf{Informed Consent:} Written informed consent was obtained rom the
patient to publish this report in accordance with the journal patient
consent policy

\emph{Abstract} :

We present a case of a 47-year-old female with a history of diagnosed KD
and autoimmune hepatitis 13 years ago who presented with recurrent
fevers and a desquamative rash on the lower extremities. CT neck showed
enlarged lymph nodes, and with her daily fevers and skin rashes the
presentation was concerning for recurrence of her KD. Patient was also
found to have an elevated ASMA titer, and subsequent liver biopsy
confirmed the diagnosis of autoimmune hepatitis. She was started on
methylprednisolone with improvement. Our case emphasizes the association
of KD with autoimmune conditions other than SLE. Given the recurrence of
the disease after a decade of quiescence, long-term follow-up of
patients with KD should be implemented.

\textbf{Key Clinical Message:}

Kikuchi disease (KD) can relapse despite a remission period, with other
recurrent autoimmune diseases closely linked. Our case emphasizes the
association of KD with autoimmune conditions other than SLE. Given the
recurrence of the disease after a decade of quiescence, long-term
follow-up of patients with KD should be implemented.

\textbf{Keywords:} Kikuchi disease, Hepatitis, Pathology

\emph{Background:}

Kikuchi's disease (KD) is a rare, benign disease that is characterized
by fever and localized lymphadenopathy, and its association with
autoimmune diseases has been scarcely reported. We describe a report of
recurring KD, originally in remission for several years, along with a
concomitant flare up of biopsy proven autoimmune hepatitis (AIH).

\emph{Case Presentation:}

A 47-year-old Hispanic female with a history of KD diagnosed 13 years
ago presented with recurrent fevers, desquamative rash on the legs, and
diffuse arthralgias. She reported daily fevers at noon and in the
evening starting one month ago. One week prior to her presentation, she
developed abdominal pain associated with nausea, anorexia, and dark
urine. Vital signs on arrival were unremarkable without evidence of
hypotension, tachycardia, or hypoxia. Physical examination was
significant for a desquamative rash on her thighs bilaterally with no
clinical lymphadenopathy. Infectious work up including chest x-ray,
urinalysis, complete blood count, and blood cultures were unremarkable.
Further laboratory results revealed an elevated AST 241 units/L, ALT 277
units/L, and ALP 118 units/L (previously normal 3 months ago).
Autoimmune work up including ANA, anti-dsDNA, anti-SSA/SSB were negative
but did have a weakly positive ASMA with titers of 1:40. Ultrasound of
the abdomen showed cholelithiasis without cholecystitis and hepatic
steatosis. CT neck showed a 1.8 cm right submandibular, 1.6 cm left
submandibular, subcentimeter submental and supraclavicular lymph nodes.
Her lymphadenopathy with daily fevers, skin rash and joint pain was
concerning for recurrence of her KD. Given her elevated liver function
tests, there was also concern for concomitant acute AIH. Patient then
underwent liver biopsy which confirmed AIH. Patient was subsequently
started on methylprednisolone with improvement in her liver function
tests along with her fevers and arthralgias. She was started on
Azathioprine at discharge and continued to follow up in rheumatology and
liver clinic as outpatient for management of her disease.

The patient had a known history of AIH associated with KD, which was
diagnosed 13 years ago via liver biopsy and axillary lymph node biopsy,
respectively \textbf{(Figure 1)} . Work up at the time of diagnosis was
negative for ANA, anti-dsDNA, anti-Sm, anti-RNP and positive for
elevated liver enzymes. She was started on corticosteroids and
azathioprine, which was self-discontinued after 8 years. Her symptoms
were quiescent for years until now.

\emph{Discussion:}

The extranodal symptoms of KD are uncommon and diverse including skin
rash, night sweats, weight loss, headache, cough, and abdominal pain
{[}1{]}. The disease resolves spontaneously in weeks to months, but in
some cases, it may have systemic involvement or evolve to SLE {[}2-4{]}.
A definite diagnosis of KD is based on characteristic pathologic
findings on biopsy that differentiate this disease from others such as
lymphoma, SLE, and infectious lymphadenopathy. Characteristic
histopathologic findings include necrotic and thrombotic blood vessels.
The karyorrhectic foci are formed by different cellular types,
predominantly histiocytes and plasmacytoid monocytes, but also
immunoblasts and small and large lymphocytes {[}5{]}.

Recurrence of KD is rare. There have been more recent reports of
recurrence rates as high as 13\% in an Asian population in Korea.
{[}6{]} In a study by Jung et al, 54 patients (11.3\%) experienced 1--4
recurrent episodes of KD each. The initial recurrence occurred within a
mean duration of 6 months (range: 1 month to 6 years). Patients with
recurrent KD were more likely to have extranodal symptoms and
lymphopenia. Although the etiology of KD recurrence is unknown, certain
viral infections have been hypothesized to be among the triggers for KD
relapse {[}8{]}.

Patients with KD showing progression to autoimmune diseases were more
likely to have fever, common extranodal symptoms, a higher recurrence
rate, and a higher ANA positivity rate at KD diagnosis {[}1{]}.
Hepatosplenomegaly was present in 18\% of patients of KD {[}7{]} with
few cases having clinically diagnosed AIH. Our case had biopsy proven
AIH without clinical and laboratory findings of SLE. In addition, our
case had recurrence of symptoms of KD after 9 years which improved after
starting corticosteroids.

\textbf{Conclusion:}

KD is a rare condition, and it should be considered in differential
diagnoses of tender lymphadenopathy, especially lymphadenopathy
localized to the cervical region and recurrent fever with AIH. Although
the disease takes a self-limiting clinical course in most cases, we
reported a case of KD with a prolonged relapse of ten years. Our case
emphasizes the association of KD with autoimmune conditions other than
SLE. Full recovery with a good response to corticosteroid regimen was
achieved after the recurrence; therefore, considering the recurrence of
KD, long-term follow-up of patients with KD should be implemented.

\textbf{Informed Consent:} Written informed consent was obtained rom the
patient to publish this report in accordance with the journal patient
consent policy

\textbf{Conflicts of interest:} The authors have no conflicts of
interest to disclose.

\textbf{Funding:} The authors have no financial support to disclose.

\textbf{Ethical statement:} Institutional Review Board (IRB) approval is
not required for case reports at our institution.

\textbf{Availability of data and materials}

The data used in the case report are available on reasonable request.

\textbf{Acknowledgments}

Not applicable.

\textbf{Authors' contributions}

UD, BI , NU, and DS were involved in patient care (diagnosis, treatment,
and follow-up). UD, BI, NU, NS, VJ, AS and SU contributed to the
collection of case information, writing of the manuscript, and
manuscript revision. VJ and DS were involved in revising the manuscript
critically for important intellectual content. All authors approved the
final version.

\emph{References}

1) Jung HJ, Lee IJ, Yoon SH. Risk Assessment of Recurrence and
Autoimmune Disorders in Kikuchi Disease. \emph{Risk Manag Healthc
Policy} . 2020;13:1687-1693. Published 2020 Sep 22.
doi:10.2147/RMHP.S271283

2) Wong CY, Law GT, Shum TT, Wong KY, Li YK. Pulmonary haemorrhage in a
patient with Kikuchi disease. \emph{Monaldi Arch Chest
Dis}2001;56:118--20.

3) Lin SH, Ko WS, Lee HS, Hwang WS. Kikuchi's disease associated with
lupus-like syndrome---a fatal case. \emph{J Rheumatol} 1992;19: 1995--6.

4) Chen YH, Lan JL. Kikuchi disease in systemic lupus erythemato- sus:
clinical features and literature review. \emph{J Microbiol Immunol
Infect} 1998;31:187--92.

5) Chien-Yu Cheng, Wang-Huei Sheng, Yi-Chun Lo, Chen-Shuan Chung,
Yee-Chun Chen, Shan-Chwen Chang,Clinical Presentations, Laboratory
Results and Outcomes of Patients with Kikuchi's Disease: Emphasis on the
Association Between Recurrent Kikuchi's Disease and Autoimmune Diseases,
Journal of Microbiology, Immunology and Infection, Volume 43, Issue 5,
2010,Pages 366-371,ISSN 1684-1182,

6) Song JY, et al. Clincial outcome and predictive factors or recurrence
among patiens with kikuchi's disease. \emph{Int J Infect Dis} 2009; 13:
322--6

7) Tanasescu C, Nitescu D, Staniceanu F, Ardeleanu C, Atanasiu C.
Kikuchi's disease associated with systemic lupus erythematosus and
autoimmune-like hepatitis. Rom J Intern Med 2003; 41:299-305

8) Lee HY, Huang YC, Lin TY, Huang JL, Yang CP, Hsueh T, et al. Primary
Epstein-Barr virus infection associated with Kikuchi's disease and
hemophagocytic lymphohistiocytosis: a case report and review of the
literature. \emph{J Microbiol Immunol Infect.} 2010;43(3):253--7

\textbf{Hosted file}

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