Abstract
BackgroundNeonatal systemic vasculitis syndromes have been reported in infants born to mothers with systemic lupus erythematosus, Sjögren’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 presentationIn 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.ConclusionThis 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.
Highlights
Neonatal systemic vasculitis syndromes have been reported in infants born to mothers with systemic lupus erythematosus, Sjögren’s syndrome, Behҫet’s disease, cutaneous polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitides
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
We report the first case, to our knowledge, of a new onset of seronegative inflammatory arthritis in a primiparous mother associated with the development of an effectively treated systemic vasculitis syndrome in the neonate
Summary
During pregnancy many changes occur in the immune system to allow tolerance to the fetus. Newborn screen (Additional file 1) was negative and his metabolic work up including ammonia level, plasma amino acids, urine organic acids, total and free carnitine, acylcarnitine profile, methylmalonic acid level were normal. His DNA was screened with targeted sequencing for known hereditary autoinflammatory diseases (17 next-generation sequencing panel including Deficiency of Adenosine Deaminase 2 (DADA2) and STING-associated vasculopathy with infantile onset (SAVI)), we were not able to identify a causative mutation. The mother was seen by an adult rheumatologist postpartum and repeat autoantibody profile remained negative (ANA, RF and anti-CCP) She was weaned off prednisone with a slow taper of approximately 3 months and was treated with hydroxychloroquine for a total of 8 months. She remains in remission off therapy and has had no further pregnancies
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