Atypical manifestations of Vogt-Koyanagi-Harada syndrome

Abstract Background and Aims Both antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgG4-related disease (IgG4-RD) are multi-system inflammatory disorders. The coexistent of both diseases present the possibility of a new overlap syndrome which leads to different treatment and outcome. In this study, we aim to investigate the possibility and clinicopathologic clues to the diagnosis of this overlap syndrome. Method A case of a 67-year-old man in our hospital who exhibited the clinicopathologic characteristics of both AAV and IgG4-RD was presented. The serial alterations in serum parameters and treatment response of the case were followed up for the next 15 months. Then, a systematic literature review of the overlap syndrome was performed on PUBMED database from 1976 until January 2020. Results Forty-two patients fully met both AAV and IgG4-RD criteria in the literature. The median age was 60 years ranged from 31 to 73 years at diagnosis. Twenty-eight (66.7%) patients were men. Serum IgG4 concentration increased in 41 (97.6%) patients (median:395 mg/dl; range:177-876mg/dl). Forty-one patients (97.6%) tested positive for ANCA with 37 (90.2%) patients showing a specificity for MPO. Kidney histology of 23 (69.7%) patients presented pauci-immune necrotizing or crescentic glomerulonephritis and IgG4-relative tubulointerstitial nephritis. Glucocorticoids combined with cyclophosphamide therapy was commonly prescribed with a high remission rate within 3 months. Four common clinicopathologic features of the overlap syndrome were identified from the case and literature. Conclusion AAV may overlap with IgG4-RD while presenting atypical manifestations. Four common clinicopathologic characteristics could be used as specific clues to the diagnosis of overlap syndrome.

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Atypical Manifestations of Pneumonia in the Elderly

Clinics in Chest Medicine ◽

10.1016/s0272-5231(21)00746-2 ◽

1991 ◽

Vol 12 (2) ◽

pp. 319-336 ◽

Cited By ~ 3

Author(s):

Alan M. Fein ◽

Steven H. Feinsilver ◽

Michael S. Niederman

Keyword(s):

The Elderly ◽

Atypical Manifestations

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Typical and Atypical Manifestations of Gastroesophageal Reflux Disease

Gastrointestinal Motility ◽

10.1007/978-1-4615-4803-4_12 ◽

1999 ◽

pp. 121-129

Author(s):

Donald O. Castell

Keyword(s):

Gastroesophageal Reflux Disease ◽

Gastroesophageal Reflux ◽

Reflux Disease ◽

Atypical Manifestations

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Anti-LGI1 Limbic Encephalitis Presented with Atypical Manifestations

Experimental Neurobiology ◽

10.5607/en.2013.22.4.337 ◽

2013 ◽

Vol 22 (4) ◽

pp. 337-340 ◽

Cited By ~ 8

Author(s):

Jung-Ju Lee ◽

Soon-Tae Lee ◽

Keun-Hwa Jung ◽

Kon Chu ◽

Sang Kun Lee

Keyword(s):

Limbic Encephalitis ◽

Atypical Manifestations

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A Study of Atypical Manifestations of Enteric Fever in Childern

Journal of Nepal Health Research Council ◽

10.3126/jnhrc.v6i1.2436 ◽

1970 ◽

Vol 6 (1) ◽

pp. 1-4

Author(s):

K. N. Shakya ◽

M. R. Baral ◽

R. Shrestha

Keyword(s):

Health Research ◽

Research Council ◽

Enteric Fever ◽

Atypical Features ◽

Atypical Manifestations ◽

Health Research Council

Not uploaded.Key Words: Enteric fever; Atypical features; Susceptibility patternDOI: 10.3126/jnhrc.v6i1.2436Journal of Nepal Health Research Council Vol. 6, No. 1, Issue 12, April, 2008 Page: 1-4

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