Childhood polyarteritis nodosa presenting as central nervous system vasculitis

Childhood polyarteritis nodosa (c-PAN) is one of the rare causes of childhood central nervous system (CNS) vasculitis. Nervous system involvement is less common, with peripheral nervous system frequently involved than the CNS. To the best of our knowledge, a very few cases of c-PAN presenting as CNS...

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Main Authors: Sham Santhanam, Ravikumar Thambithurai, Nataraj Palaniappan, Mukul Vij, Srinivasan Kalyanasundaram
Format: Article
Language:English
Published: SAGE Publishing 2019-01-01
Series:Indian Journal of Rheumatology
Subjects:
Online Access:http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2019;volume=14;issue=1;spage=65;epage=68;aulast=Santhanam
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author Sham Santhanam
Ravikumar Thambithurai
Nataraj Palaniappan
Mukul Vij
Srinivasan Kalyanasundaram
author_facet Sham Santhanam
Ravikumar Thambithurai
Nataraj Palaniappan
Mukul Vij
Srinivasan Kalyanasundaram
author_sort Sham Santhanam
collection DOAJ
description Childhood polyarteritis nodosa (c-PAN) is one of the rare causes of childhood central nervous system (CNS) vasculitis. Nervous system involvement is less common, with peripheral nervous system frequently involved than the CNS. To the best of our knowledge, a very few cases of c-PAN presenting as CNS vasculitis involving both the anterior and posterior circulation have been reported in the literature. Hence, we report a 14-year-old female presenting with extensive CNS vasculitis with severe neurological deficits. She also had systemic symptoms, myalgia, leg ulcers, weight loss, axonal neuropathy, and proteinuria. She was treated with pulse methylprednisolone, monthly intravenous pulse cyclophosphamide, and intravenous immunoglobulin. At the end of 3 months, her proteinuria came down significantly with no major improvement in her neurological status. Hence, c-PAN has to be considered in the differential diagnosis of childhood CNS vasculitis and if not picked up early can lead to severe neurological deficits.
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institution Kabale University
issn 0973-3698
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publishDate 2019-01-01
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record_format Article
series Indian Journal of Rheumatology
spelling doaj-art-28f76095c10e4099af923b0dc7b25ccf2025-01-03T01:45:19ZengSAGE PublishingIndian Journal of Rheumatology0973-36980973-37012019-01-01141656810.4103/injr.injr_129_18Childhood polyarteritis nodosa presenting as central nervous system vasculitisSham SanthanamRavikumar ThambithuraiNataraj PalaniappanMukul VijSrinivasan KalyanasundaramChildhood polyarteritis nodosa (c-PAN) is one of the rare causes of childhood central nervous system (CNS) vasculitis. Nervous system involvement is less common, with peripheral nervous system frequently involved than the CNS. To the best of our knowledge, a very few cases of c-PAN presenting as CNS vasculitis involving both the anterior and posterior circulation have been reported in the literature. Hence, we report a 14-year-old female presenting with extensive CNS vasculitis with severe neurological deficits. She also had systemic symptoms, myalgia, leg ulcers, weight loss, axonal neuropathy, and proteinuria. She was treated with pulse methylprednisolone, monthly intravenous pulse cyclophosphamide, and intravenous immunoglobulin. At the end of 3 months, her proteinuria came down significantly with no major improvement in her neurological status. Hence, c-PAN has to be considered in the differential diagnosis of childhood CNS vasculitis and if not picked up early can lead to severe neurological deficits.http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2019;volume=14;issue=1;spage=65;epage=68;aulast=SanthanamCentral nervous systemchildhoodpolyarteritis nodosavasculitis
spellingShingle Sham Santhanam
Ravikumar Thambithurai
Nataraj Palaniappan
Mukul Vij
Srinivasan Kalyanasundaram
Childhood polyarteritis nodosa presenting as central nervous system vasculitis
Indian Journal of Rheumatology
Central nervous system
childhood
polyarteritis nodosa
vasculitis
title Childhood polyarteritis nodosa presenting as central nervous system vasculitis
title_full Childhood polyarteritis nodosa presenting as central nervous system vasculitis
title_fullStr Childhood polyarteritis nodosa presenting as central nervous system vasculitis
title_full_unstemmed Childhood polyarteritis nodosa presenting as central nervous system vasculitis
title_short Childhood polyarteritis nodosa presenting as central nervous system vasculitis
title_sort childhood polyarteritis nodosa presenting as central nervous system vasculitis
topic Central nervous system
childhood
polyarteritis nodosa
vasculitis
url http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2019;volume=14;issue=1;spage=65;epage=68;aulast=Santhanam
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AT ravikumarthambithurai childhoodpolyarteritisnodosapresentingascentralnervoussystemvasculitis
AT natarajpalaniappan childhoodpolyarteritisnodosapresentingascentralnervoussystemvasculitis
AT mukulvij childhoodpolyarteritisnodosapresentingascentralnervoussystemvasculitis
AT srinivasankalyanasundaram childhoodpolyarteritisnodosapresentingascentralnervoussystemvasculitis