Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi Lymphadenitis

Background. Adult onset Still’s disease (AOSD) is a rare inflammatory disorder with a variety of clinical presentations. Even though pneumonitis and pleurisy are known to occur in AOSD, pulmonary onset presentations are exceedingly rare. Case Presentation. We present a 40-year-old male, presenting w...

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Main Authors: G. D. E. Kodithuwakku, C. L. Fonseka, S. Nanayakkara, H. M. M. Herath
Format: Article
Language:English
Published: Wiley 2020-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2020/8327068
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author G. D. E. Kodithuwakku
C. L. Fonseka
S. Nanayakkara
H. M. M. Herath
author_facet G. D. E. Kodithuwakku
C. L. Fonseka
S. Nanayakkara
H. M. M. Herath
author_sort G. D. E. Kodithuwakku
collection DOAJ
description Background. Adult onset Still’s disease (AOSD) is a rare inflammatory disorder with a variety of clinical presentations. Even though pneumonitis and pleurisy are known to occur in AOSD, pulmonary onset presentations are exceedingly rare. Case Presentation. We present a 40-year-old male, presenting with fever and bilateral alveolar shadows with pleural effusions mimicking community-acquired severe pneumonia. He was initially treated as severe pneumonia with poor response to broad spectrum antibiotics. Subsequently, he was managed as AOSD-induced pneumonitis, as he fulfilled Yamaguchi criteria. Few weeks later, he developed macular rash and arthralgia with generalized lymphadenopathy with lymph node histology, showing Kikuchi lymphadenitis. He responded well to steroids and had a complete recovery. Conclusion. Non-infective causes of pneumonitis should be suspected in the setting of poorly resolving pneumonias, especially when microbiological and serological investigations does not support an infective etiology. Presence of systemic symptoms with arthralgia, rash, and disproportionately elevated ferritin level supports the diagnosis of AOSD. Kikuchi lymphadenitis is a reported association with AOSD, and there could be a causal link between the two disorders.
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spelling doaj-art-99797faac3c04223a94b46a6be42b3b92025-02-03T00:59:43ZengWileyCase Reports in Rheumatology2090-68892090-68972020-01-01202010.1155/2020/83270688327068Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi LymphadenitisG. D. E. Kodithuwakku0C. L. Fonseka1S. Nanayakkara2H. M. M. Herath3University Medical Unit, Teaching Hospital Karapitiya, Galle, Sri LankaUniversity Medical Unit, Teaching Hospital Karapitiya, Galle, Sri LankaUniversity Medical Unit, Teaching Hospital Karapitiya, Galle, Sri LankaUniversity Medical Unit, Teaching Hospital Karapitiya, Galle, Sri LankaBackground. Adult onset Still’s disease (AOSD) is a rare inflammatory disorder with a variety of clinical presentations. Even though pneumonitis and pleurisy are known to occur in AOSD, pulmonary onset presentations are exceedingly rare. Case Presentation. We present a 40-year-old male, presenting with fever and bilateral alveolar shadows with pleural effusions mimicking community-acquired severe pneumonia. He was initially treated as severe pneumonia with poor response to broad spectrum antibiotics. Subsequently, he was managed as AOSD-induced pneumonitis, as he fulfilled Yamaguchi criteria. Few weeks later, he developed macular rash and arthralgia with generalized lymphadenopathy with lymph node histology, showing Kikuchi lymphadenitis. He responded well to steroids and had a complete recovery. Conclusion. Non-infective causes of pneumonitis should be suspected in the setting of poorly resolving pneumonias, especially when microbiological and serological investigations does not support an infective etiology. Presence of systemic symptoms with arthralgia, rash, and disproportionately elevated ferritin level supports the diagnosis of AOSD. Kikuchi lymphadenitis is a reported association with AOSD, and there could be a causal link between the two disorders.http://dx.doi.org/10.1155/2020/8327068
spellingShingle G. D. E. Kodithuwakku
C. L. Fonseka
S. Nanayakkara
H. M. M. Herath
Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi Lymphadenitis
Case Reports in Rheumatology
title Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi Lymphadenitis
title_full Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi Lymphadenitis
title_fullStr Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi Lymphadenitis
title_full_unstemmed Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi Lymphadenitis
title_short Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi Lymphadenitis
title_sort pulmonary onset of adult onset still s disease complicated with kikuchi lymphadenitis
url http://dx.doi.org/10.1155/2020/8327068
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AT snanayakkara pulmonaryonsetofadultonsetstillsdiseasecomplicatedwithkikuchilymphadenitis
AT hmmherath pulmonaryonsetofadultonsetstillsdiseasecomplicatedwithkikuchilymphadenitis