PMID- 34717662
OWN - NLM
STAT- MEDLINE
DCOM- 20220314
LR  - 20220314
IS  - 1546-0096 (Electronic)
IS  - 1546-0096 (Linking)
VI  - 19
IP  - 1
DP  - 2021 Oct 30
TI  - A case report of a severe neonatal systemic vasculitis on the first day of life.
PG  - 154
LID - 10.1186/s12969-021-00618-x [doi]
LID - 154
AB  - BACKGROUND: Neonatal systemic vasculitis syndromes have been reported in infants 
      born to mothers with systemic lupus erythematosus, Sjogren's syndrome, Behҫet's 
      disease, cutaneous polyarteritis nodosa and anti-neutrophil cytoplasmic 
      antibody-associated vasculitides. Here we report a novel association of a case of 
      new-onset maternal seronegative inflammatory arthritis associated with a 
      transient systemic vasculitis in a neonate. CASE PRESENTATION: In the first 24 h 
      of life, a preterm Caucasian baby boy was noted to have blue discoloration to all 
      four extremities. Despite antibiotics, fresh frozen plasma and anticoagulation, 
      the discoloration remained, particularly in the left index finger. This was 
      associated with fever and a maximum C-reactive protein (CRP) of 148 mg/L. 
      Intravenous immunoglobulin (IVIG) was given with short-term improvement. Initial 
      echocardiogram showed enlarged coronary arteries with normalization on repeat 
      1 week later. Clinical signs and symptoms responded to high dose oral steroid 
      administration. MRI angiography (MRA) of the body and heart showed tortuosity of 
      arteries in the upper and lower extremities with gadolinium uptake, suggestive of 
      vasculitis. Autoantibody profile negative. Genetic panel for hereditary 
      autoinflammatory diseases was negative as was whole exome sequencing performed on 
      the trio. The baby was weaned off steroids by 5 months of age. A small distal 
      autoamputation of the left index finger occurred. He was born to a 28-year-old 
      woman who developed new onset severe symmetrical polyarthritis at 8 weeks 
      gestation. This was presumed a reactive arthritis secondary to a dental 
      infection. Infectious work up and autoantibodies were negative. She was treated 
      with high dose prednisone for the remainder of her pregnancy. The mother was 
      weaned off prednisone, treated with hydroxychloroquine for 8 months post-partum 
      and remains in remission. A repeat MRA done at 1 year old showed mild residual 
      tortuosities of the arteries in the forearms. The remainder of the medium and 
      large vessels were within normal limits with no gadolinium enhancement to suggest 
      active disease. The child is now 4 years old with normal growth and development. 
      CONCLUSION: This is a unique case of new-onset seronegative presumed reactive 
      arthritis in a mother with the rare development of a successfully treated medium 
      vessel vasculitis in an infant.
CI  - (c) 2021. The Author(s).
FAU - Wong, Stephanie
AU  - Wong S
AUID- ORCID: 0000-0002-3237-1725
AD  - British Columbia Children's Hospital, 4480 Oak Street Ambulatory care building, 
      Vancouver, British Columbia, V6H 3V4, Canada. Stephanie.wong2@phsa.ca.
AD  - University of British Columbia, Vancouver, Canada. Stephanie.wong2@phsa.ca.
FAU - Demirkaya, Erkan
AU  - Demirkaya E
AD  - Children's Hospital, London Health Sciences Centre, London, Ontario, Canada.
AD  - Western University, London, Canada.
FAU - Berard, Roberta
AU  - Berard R
AD  - Children's Hospital, London Health Sciences Centre, London, Ontario, Canada.
AD  - Western University, London, Canada.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20211030
PL  - England
TA  - Pediatr Rheumatol Online J
JT  - Pediatric rheumatology online journal
JID - 101248897
SB  - IM
MH  - Adult
MH  - Arthritis/*complications
MH  - Female
MH  - Humans
MH  - Infant, Newborn
MH  - Magnetic Resonance Angiography/methods
MH  - Male
MH  - Pregnancy
MH  - Pregnancy Complications/*diagnosis
MH  - Prenatal Exposure Delayed Effects/*diagnosis
MH  - Systemic Vasculitis/*diagnosis/etiology
PMC - PMC8556936
OTO - NOTNLM
OT  - Neonate
OT  - Polyarthritis
OT  - Pregnancy
OT  - Vasculitis
COIS- The authors declare that they have no competing interests.
EDAT- 2021/11/01 06:00
MHDA- 2022/03/15 06:00
PMCR- 2021/10/30
CRDT- 2021/10/31 20:35
PHST- 2021/02/26 00:00 [received]
PHST- 2021/06/16 00:00 [accepted]
PHST- 2021/10/31 20:35 [entrez]
PHST- 2021/11/01 06:00 [pubmed]
PHST- 2022/03/15 06:00 [medline]
PHST- 2021/10/30 00:00 [pmc-release]
AID - 10.1186/s12969-021-00618-x [pii]
AID - 618 [pii]
AID - 10.1186/s12969-021-00618-x [doi]
PST - epublish
SO  - Pediatr Rheumatol Online J. 2021 Oct 30;19(1):154. doi: 
      10.1186/s12969-021-00618-x.