Association between Tubulointerstitial Nephritis and Uveitis Syndrome and Small-Vessel CNS Vasculitis

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Association between Tubulointerstitial Nephritis and Uveitis Syndrome and Small-Vessel CNS Vasculitis. / Grinstein, Lev; Hecher, Laura; Weiss, Deike; Johannsen, Jessika; Denecke, Jonas.

in: NEUROPEDIATRICS, Jahrgang 55, Nr. 2, 04.2024, S. 117-123.

Publikationen: SCORING: Beitrag in Fachzeitschrift/ZeitungSCORING: ZeitschriftenaufsatzForschungBegutachtung

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@article{e21166b5cdcf41dcb763abece38cafd3,
title = "Association between Tubulointerstitial Nephritis and Uveitis Syndrome and Small-Vessel CNS Vasculitis",
abstract = "INTRODUCTION:  We report a case study of two male pediatric patients presenting with anterior uveitis and elevated renal function parameters. Both were diagnosed with tubulointerstitial nephritis and uveitis syndrome and subsequently developed diffuse cerebral symptoms such as headache, fatigue, and diziness.METHODS:  Magnetic resonance images (MRIs) of the brain showed T2-hyperintense lesions with and without gadolinium enhancement leading to brain biopsy and diagnosis of small-vessel central nervous system (CNS) vasculitis in both cases. Both patients were treated according to BrainWorks small-vessel vasculitis protocol and symptoms vanished over the course of treatment. Follow-up MRIs up to 12 months after initiation of therapy showed no signs of recurrence indicating a monophasic disease.CONCLUSION:  Small-vessel CNS vasculitis can occur simultaneously to other autoimmune diseases (ADs) in the scope of polyautoimmunity. As clinical findings of CNS vasculitis are often unspecific, neurological symptoms in nonneurological ADs should be adressed thoroughly. Under suspicion of small-vessel CNS vasculitis brain biopsy is still the gold standard and only secure way of definitive diagnosis.",
keywords = "Child, Contrast Media/therapeutic use, Gadolinium/therapeutic use, Humans, Male, Nephritis, Interstitial, Uveitis/complications, Vasculitis, Central Nervous System/complications",
author = "Lev Grinstein and Laura Hecher and Deike Weiss and Jessika Johannsen and Jonas Denecke",
note = "Thieme. All rights reserved.",
year = "2024",
month = apr,
doi = "10.1055/a-2239-1678",
language = "English",
volume = "55",
pages = "117--123",
journal = "NEUROPEDIATRICS",
issn = "0174-304X",
publisher = "Hippokrates Verlag GmbH",
number = "2",

}

RIS

TY - JOUR

T1 - Association between Tubulointerstitial Nephritis and Uveitis Syndrome and Small-Vessel CNS Vasculitis

AU - Grinstein, Lev

AU - Hecher, Laura

AU - Weiss, Deike

AU - Johannsen, Jessika

AU - Denecke, Jonas

N1 - Thieme. All rights reserved.

PY - 2024/4

Y1 - 2024/4

N2 - INTRODUCTION:  We report a case study of two male pediatric patients presenting with anterior uveitis and elevated renal function parameters. Both were diagnosed with tubulointerstitial nephritis and uveitis syndrome and subsequently developed diffuse cerebral symptoms such as headache, fatigue, and diziness.METHODS:  Magnetic resonance images (MRIs) of the brain showed T2-hyperintense lesions with and without gadolinium enhancement leading to brain biopsy and diagnosis of small-vessel central nervous system (CNS) vasculitis in both cases. Both patients were treated according to BrainWorks small-vessel vasculitis protocol and symptoms vanished over the course of treatment. Follow-up MRIs up to 12 months after initiation of therapy showed no signs of recurrence indicating a monophasic disease.CONCLUSION:  Small-vessel CNS vasculitis can occur simultaneously to other autoimmune diseases (ADs) in the scope of polyautoimmunity. As clinical findings of CNS vasculitis are often unspecific, neurological symptoms in nonneurological ADs should be adressed thoroughly. Under suspicion of small-vessel CNS vasculitis brain biopsy is still the gold standard and only secure way of definitive diagnosis.

AB - INTRODUCTION:  We report a case study of two male pediatric patients presenting with anterior uveitis and elevated renal function parameters. Both were diagnosed with tubulointerstitial nephritis and uveitis syndrome and subsequently developed diffuse cerebral symptoms such as headache, fatigue, and diziness.METHODS:  Magnetic resonance images (MRIs) of the brain showed T2-hyperintense lesions with and without gadolinium enhancement leading to brain biopsy and diagnosis of small-vessel central nervous system (CNS) vasculitis in both cases. Both patients were treated according to BrainWorks small-vessel vasculitis protocol and symptoms vanished over the course of treatment. Follow-up MRIs up to 12 months after initiation of therapy showed no signs of recurrence indicating a monophasic disease.CONCLUSION:  Small-vessel CNS vasculitis can occur simultaneously to other autoimmune diseases (ADs) in the scope of polyautoimmunity. As clinical findings of CNS vasculitis are often unspecific, neurological symptoms in nonneurological ADs should be adressed thoroughly. Under suspicion of small-vessel CNS vasculitis brain biopsy is still the gold standard and only secure way of definitive diagnosis.

KW - Child

KW - Contrast Media/therapeutic use

KW - Gadolinium/therapeutic use

KW - Humans

KW - Male

KW - Nephritis, Interstitial

KW - Uveitis/complications

KW - Vasculitis, Central Nervous System/complications

U2 - 10.1055/a-2239-1678

DO - 10.1055/a-2239-1678

M3 - SCORING: Journal article

C2 - 38181817

VL - 55

SP - 117

EP - 123

JO - NEUROPEDIATRICS

JF - NEUROPEDIATRICS

SN - 0174-304X

IS - 2

ER -