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An Interesting Case of Bilateral Hilar Lymphadenopathy Presented with Visual Impairment

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Sarcoidosis is a systemic granulomatous disease of unknown etiology. It primarily affects the lungs and lymphatics; however, the presentation is not always pulmonary. Uveitis is the presenting symptom in 5% of patients, predominantly in females. Cystoid macular edema is the most important sight-threatening sequela of ocular sarcoidosis. Histopathological evidence of noncaseating epithelioid granulomas from a biopsy is the gold standard for the diagnosis of ocular sarcoidosis. Diagnosis of sarcoidosis is made by exclusion of other causes of granulomatous disease, mainly tuberculosis and fungal infection and documentation of involvement of at least one additional organ system. The authors present a case of a patient with sarcoidosis involving lungs and eyes, who had uveitis as an initial presentation.
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An Interesting Case of Bilateral Hilar
Lymphadenopathy Presented with
Visual Impairment
Chandramouli MT1Nandakishore Baikunje1Sunil Kumar Y2Subrahmanya Bhat K3Darshan Bajaj4
Giridhar Belur Hosmane1
1Department of Pulmonary Medicine, K S Hegde Medical Academy,
Mangalore, India
2Department of Pathology, K S Hegde Medical Academy, Mangalore,
India
3Department of Ophthalmology, K S Hegde Medical Academy,
Mangalore, India
4Department of Pulmonary Medicine, King GeorgesMedical
University, Lucknow, India
J Health Allied Sci
NU
2022;12:330332.
Address for correspondence Chandramouli MT, MBBS, MD,
Department of Pulmonary Medicine, K S Hegde Medical Academy,
New Boys Hostel, KSHEMA Campus, Mangalore 575018, India
(e-mail: mouli.aims@gmail.com).
Introduction
Sarcoidosis is a multisystem chronic inammatory disor-
der of unknown etiology, characterized pathologically by
noncaseating granulomas. It usually affects young adults
with variable clinical presentation. The lung is the more
frequently affected organ and accounts for morbidity
and mortality associated with this disease. Other organs
commonly involved include the eyes, skin, and lymph
nodes.
The incidence and prevalence of sarcoidosis vary substan-
tially, depending on the region of the world. It commonly
affects women than men. Diagnosis of sarcoidosis relies on
compatible clinicoradiological ndings and is supported by
histopathological evidence of noncaseating epithelioid gran-
ulomas in involved organs, exclusion of other causes of
granulomatous disease, mainly tuberculosis and fungal in-
fection, and documentation of involvement of at least one
additional organ system.
Corticosteroids are the mainstay treatment for sarcoido-
sis. Additional immunosuppressive agents including metho-
trexate, azathioprine, mycophenolate mofetil, iniximab,
and adalimumab are used in refractory systemic sarcoidosis
and lung transplantation in advanced lung disease.
Keywords
EBUS TBNA
granulomatous lesion
sarcoidosis
uveitis
Abstract Sarcoidosis is a systemic granulomatous disease of unknown etiology. It primarily
affects the lungs and lymphatics; however, the presentation is not always pulmonary.
Uveitis is the presenting symptom in 5% of patients, predominantly in females. Cystoid
macular edema is the most important sight-threatening sequela of ocular sarcoidosis.
Histopathological evidence of noncaseating epithelioid granulomas from a biopsy is
the gold standard for the diagnosis of ocular sarcoidosis. Diagnosis of sarcoidosis is
made by exclusion of other causes of granulomatous disease, mainly tuberculosis and
fungal infection and documentation of involvement of at least one additional organ
system. The authors present a case of a patient with sarcoidosis involving lungs and
eyes, who had uveitis as an initial presentation.
published online
November 5, 2021
DOI https://doi.org/
10.1055/s-0041-1736454.
ISSN 2582-4287.
© 2021. Nitte (Deemed to be University). All rights reserved.
This is an open access article published by Thieme under the terms of the
Creative Commons Attribution-NonDerivative-NonCommercial-License,
permitting copying and reproduction so long as the original work is given
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Case Report
THIEME
330
Article published online: 2021-11-05
Case Report
A 59-year-old female presented with redness and blurring of
vision in both eyes for 20 days. An ophthalmic evaluation
showed panuveitis in both eyes. Physical examination and
laboratory results were unremarkable. A chest X-ray was
taken, which showed bilateral upper and midzone nodular
opacities and hilar lymph nodes. Further contrast-enhanced
computed tomography (CECT) thorax showed bilateral sym-
metrical parenchymal nodular and conuent peribronchovas-
cular opacities, perilymphatic nodules, nodular thickening of
the ssure, septa with upper lobe predominance, and bilateral
hilar and mediastinal enlarged lymph nodes (Figs. 1 and 2).
Flexible bronchoscopy (FB) followed by endobronchial
ultrasound (EBUS) was planned and performed under mod-
erate sedation, and no endobronchial lesions were visual-
ized. Bronchoalveolar lavage (BAL) was taken and sent for
bacterial and fungal culture. EBUS showed bilateral hilar,
subcarinal, and right paratracheal nodes. EBUS-transbron-
chial needle aspiration (TBNA) and rapid on-site evaluation
(ROSE) were done from the subcarinal lymph node and sent
for cytology and GeneXpert (Fig. 3). Mycobacterium tuber-
culosis was not detected in GeneXpert, and cytological
examination of lymph node aspirate showed noncaseating
granulomatous lymphadenitis (Figs. 4 and 5). The BAL
culture showed no growth for both bacteria and fungus.
The serum angiotensin-converting enzyme (ACE) and calci-
um levels were normal. In the Mantoux test, no signicant
induration was seen. Given the diagnosis of sarcoidosis
Fig. 1 Computed tomographic image of the chest demonstrating
hilar and subcarinal lymph nodes.
Fig. 2 Computed tomographic image of the chest demonstrating
numerous perilymphatic nodules and peribronchovascular opacities.
Fig. 3 Endobronchial ultrasound image demonstrating
transbronchial needle aspiration from subcarinal lymph node.
Fig. 4 Cytology of lymph node aspirate showing noncaseating
granuloma.
Fig. 5 Cytology of sarcoid granuloma showing Langerhans giant cell.
Journal of Health and Allied Sciences
NU
Vol. 12 No. 3/2022 © 2021. Nitte (Deemed to be Universit y). All rights reserved.
Bilateral Hilar Lymphadenopathy and Visual Impairment Chandramouli et al. 331
above, the patient was started on methylprednisolone pulse
therapy, followed by oral corticosteroids.
Discussion
Sarcoidosis is a systemic chronic inammatory granuloma-
tous disease of unknown etiology. It usually affects young
adults with variable clinical presentation. It can affect
multiple organs to a varying extent and degree; lung
involvement is frequent, but the presentation is not usually
pulmonary. In 20 to 50% of patients, uveitis is an early
feature of sarcoidosis.1
The incidence and prevalence of sarcoidosis vary substan-
tially, depending on the region of the world; women are
commonly affected than men. Its incidence and prevalence
are estimated at 15.3 to 21.7/100,000 and 10 to 20/100,000
population, respectively.2In India, sarcoidosis is an under-
diagnosed disease.
Many patients with pulmonary sarcoidosis are asymp-
tomatic with incidental chest radiograph ndings. Common
presenting respiratory symptoms include shortness of
breath, cough, and chest pain. Wheezing may be present in
patients with endobronchial involvement. Symmetrical and
bilateral hilar lymphadenopathy is a classical chest radio-
graph nding, and right hilar adenopathy may be slightly
more prominent.3The lung parenchymal ndings on the
chest radiograph include diffuse reticular or ground-glass
opacities, nodular opacities, and brocystic changes with
mid-to-upper zone predominance. Bronchoscopic ndings
of sarcoidosis include nodules, plaques, erythema, and
cobblestone appearance.4
Ocular sarcoidosis accounts for 10 to 50% of cases, includ-
ing lacrimal gland enlargement.5In A Case-Control Etiologic
Study of Sarcoidosis (ACCESS study), a prospective study,
11.8% of patients had ocular manifestations as presenting
symptoms.6Sarcoidosis is the leading cause of uveitis in
elderly patients.7In retrospective series of histologically
proven sarcoidosis, symptomatic uveitis accounts for 20 to
50%.8Sarcoid uveitis is typically bilateral (8090%), with
anterior uveitis being the most common , accounting for 41 to
75% of sarcoid uveitis, followed by posterior, intermediate
uveitis, and panuveitis.9Panuveitis accounts for 7 to 14% of
sarcoid uveitis and is one of the most important systemic
associations in Japan and Europe.10,11
Typical sarcoid uveitis presents with mutton-fat keratic
precipitates, iris nodules (Koeppe, Busacca nodules), anterior
and posterior synechiae, band keratopathy, and glaucoma.
Posterior involvement includes vitritis, vasculitis, and cho-
roidal lesions. Cystoid macular edema is the most important
and sight-threatening consequence of sarcoid uveitis.
No treatment is required for asymptomatic patients. In
symptomatic patients, corticosteroids are the mainstay of
treatment. Additional immunosuppressive agents including
methotrexate, azathioprine, mycophenolate mofetil, inixi-
mab, and adalimumab are used in refractory systemic sarcoid-
osis and lung transplantation in advanced lung disease.
EBUS-TBNA with ROSE has a good diagnostic yield and
assists in safe, adequate specimen sampling with rapid
results. EBUS-TBNA with or without ROSE is superior to
conventional TBNA (c-TBNA) without ROSE in the diagnosis
of sarcoidosis.12
Conclusion
Uveitis is a common extrapulmonary manifestation of sar-
coidosis, particularly in females; however, due to lack of
investigational modalities, it is underdiagnosed in India.
EBUS-TBNA is exceptionally useful in the evaluation of
mediastinal lymphadenopathy and early diagnosis of dis-
eases like sarcoidosis.
Funding
None.
Conict of Interest
None declared.
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