Giant coronary artery aneurysms in juvenile polyarteritis nodosa

A case report

Therese L. Canares, Dawn M. Wahezi, Kanwal M. Farooqi, Robert H. Pass, Norman Todd Ilowite

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with fever, arthritis and two giant coronary artery aneurysms, initially diagnosed as atypical Kawasaki disease and treated with IVIG and methylprednisolone. Her persistent fevers, arthritis, myalgias were refractory to treatment, and onset of a vasculitic rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia, hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous cyclophosphamide and high dose corticosteroids for presumed PAN related coronary vasculitis. Maintenance therapy was continued with azathioprine and the patient currently remains without evidence of active vasculitis. She remains on anticoagulation for persistence of the aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery aneurysms in the setting of juvenile PAN.

Original languageEnglish (US)
Article number1
JournalPediatric Rheumatology
Volume10
DOIs
StatePublished - Jan 5 2012

Fingerprint

Coronary Aneurysm
Polyarteritis Nodosa
Coronary Vessels
Vasculitis
Arthritis
Fever
Mucocutaneous Lymph Node Syndrome
Intravenous Immunoglobulins
Myalgia
Methylprednisolone
Azathioprine
Exanthema
Cyclophosphamide
Aneurysm
Adrenal Cortex Hormones
Arteries
Hypertension
Skin
Therapeutics

Keywords

  • Coronary artery aneurysm
  • Kawasaki disease
  • Polyarteritis nodosa
  • Vasculitis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Pediatrics, Perinatology, and Child Health

Cite this

Giant coronary artery aneurysms in juvenile polyarteritis nodosa : A case report. / Canares, Therese L.; Wahezi, Dawn M.; Farooqi, Kanwal M.; Pass, Robert H.; Ilowite, Norman Todd.

In: Pediatric Rheumatology, Vol. 10, 1, 05.01.2012.

Research output: Contribution to journalArticle

@article{d89ffe3693f347e683ebbd3a2201213c,
title = "Giant coronary artery aneurysms in juvenile polyarteritis nodosa: A case report",
abstract = "Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with fever, arthritis and two giant coronary artery aneurysms, initially diagnosed as atypical Kawasaki disease and treated with IVIG and methylprednisolone. Her persistent fevers, arthritis, myalgias were refractory to treatment, and onset of a vasculitic rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia, hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous cyclophosphamide and high dose corticosteroids for presumed PAN related coronary vasculitis. Maintenance therapy was continued with azathioprine and the patient currently remains without evidence of active vasculitis. She remains on anticoagulation for persistence of the aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery aneurysms in the setting of juvenile PAN.",
keywords = "Coronary artery aneurysm, Kawasaki disease, Polyarteritis nodosa, Vasculitis",
author = "Canares, {Therese L.} and Wahezi, {Dawn M.} and Farooqi, {Kanwal M.} and Pass, {Robert H.} and Ilowite, {Norman Todd}",
year = "2012",
month = "1",
day = "5",
doi = "10.1186/1546-0096-10-1",
language = "English (US)",
volume = "10",
journal = "Pediatric Rheumatology",
issn = "1546-0096",
publisher = "BioMed Central",

}

TY - JOUR

T1 - Giant coronary artery aneurysms in juvenile polyarteritis nodosa

T2 - A case report

AU - Canares, Therese L.

AU - Wahezi, Dawn M.

AU - Farooqi, Kanwal M.

AU - Pass, Robert H.

AU - Ilowite, Norman Todd

PY - 2012/1/5

Y1 - 2012/1/5

N2 - Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with fever, arthritis and two giant coronary artery aneurysms, initially diagnosed as atypical Kawasaki disease and treated with IVIG and methylprednisolone. Her persistent fevers, arthritis, myalgias were refractory to treatment, and onset of a vasculitic rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia, hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous cyclophosphamide and high dose corticosteroids for presumed PAN related coronary vasculitis. Maintenance therapy was continued with azathioprine and the patient currently remains without evidence of active vasculitis. She remains on anticoagulation for persistence of the aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery aneurysms in the setting of juvenile PAN.

AB - Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with fever, arthritis and two giant coronary artery aneurysms, initially diagnosed as atypical Kawasaki disease and treated with IVIG and methylprednisolone. Her persistent fevers, arthritis, myalgias were refractory to treatment, and onset of a vasculitic rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia, hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous cyclophosphamide and high dose corticosteroids for presumed PAN related coronary vasculitis. Maintenance therapy was continued with azathioprine and the patient currently remains without evidence of active vasculitis. She remains on anticoagulation for persistence of the aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery aneurysms in the setting of juvenile PAN.

KW - Coronary artery aneurysm

KW - Kawasaki disease

KW - Polyarteritis nodosa

KW - Vasculitis

UR - http://www.scopus.com/inward/record.url?scp=84855393793&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84855393793&partnerID=8YFLogxK

U2 - 10.1186/1546-0096-10-1

DO - 10.1186/1546-0096-10-1

M3 - Article

VL - 10

JO - Pediatric Rheumatology

JF - Pediatric Rheumatology

SN - 1546-0096

M1 - 1

ER -