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Abstract
Background
PAN is a systemic vasculitis with multisystem involvement rarely observed in childhood.
We report two siblings (1 boy, 1 girl) with aggressive PAN who had a persistent sustained
improvement with MMF.
Patients
The boy developed at 8 yrs musculoskeletal pain and painful nodules on the legs, than
vertigo, tinnitus, and diplopia, neurosensorial ipoacusia, left central facial palsy
and hypertension. Angio MRI revealed ischemic alterations on Willis's circle and prompted
to diagnose PAN. Despite aggressive therapy the boy developed ischemic lesions of
3 digits of hands and Iloprost was introduced; over a short time multiple ulcerative
deep cutaneous lesions appeared on the legs. Due to persistent active disease, MMF
(2 g/day) was started and up to now the clinical symptoms are stable and laboratory
work up normalised.
The girl, at 30 months had neurological manifestations (head and eye rotation with
no consciousness, optical bilateral neuritis); over time similar episodes recurred
and periodic ataxia was diagnosed. Cerebral MRI showed areas of hyperintensity on
the thalamus. At 7 yrs maculopapular rash on the face and upper extremities, arthralgia/mialgia
and hypertension were complained. Skin biopsy confirmed necrotizing vasculitis of
medium sided vessels and PAN was diagnosed. Deep cutaneous ulcers appeared on the
legs. After a severe ischemic attack, confirmed by MRI lesions at the pons area, MMF
was started. Since then the girl is stable.
Conclusion
MMF used in the treatment of SLE and primary vasculitis in children, should be considered
as either alternative or adjunctive therapy in intractable severe persistent active
PAN.